miércoles, 8 de mayo de 2019

Childhood Soft Tissue Sarcoma Treatment (PDQ®) 6/7 —Health Professional Version - National Cancer Institute

Childhood Soft Tissue Sarcoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute

National Cancer Institute



Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version

Tumors of Uncertain Differentiation

Tumors of uncertain differentiation include the following subtypes:

Synovial sarcoma NOS

Synovial sarcoma accounts for 9% of soft tissue sarcomas in patients younger than 20 years (refer to Table 1).
Synovial sarcoma is one of the most common nonrhabdomyosarcomatous soft tissue sarcomas in children and adolescents. In a 1973 to 2005 SEER review, 1,268 patients with synovial sarcoma were identified. Approximately 17% of these patients were children and adolescents, and the median age at diagnosis was 34 years.[170]
Histologic classification
Synovial sarcoma can be subclassified as the following types:
  • Synovial sarcoma NOS.
  • Synovial sarcoma, spindle cell.
  • Synovial sarcoma, biphasic.
Clinical presentation
The most common tumor location is the extremities, followed by trunk and head and neck.[170] Rarely, a synovial sarcoma may arise in the heart or pericardium.[171]
The most common site of metastasis is the lung.[172,173] The risk of metastases is highly influenced by tumor size; it is estimated that patients with tumors that are larger than 5 cm have a 32-fold risk of developing metastases when compared with other patients.
Diagnostic evaluation and molecular features
The diagnosis of synovial sarcoma is made by immunohistochemical analysis, ultrastructural findings, and demonstration of the specific chromosomal translocation t(x;18)(p11.2;q11.2). This abnormality is specific for synovial sarcoma and is found in all morphologic subtypes. Synovial sarcoma results in rearrangement of the SYT gene on chromosome 18 with one of the subtypes (1, 2, or 4) of the SSX gene on chromosome X.[174,175] It is thought that the SYT/SSX18 transcript promotes epigenetic silencing of key tumor suppressor genes.[176]
In one report, reduced INI1 nuclear reactivity on immunohistochemical staining was seen in 49 cases of synovial sarcoma, suggesting that this pattern may help distinguish synovial sarcoma from other histologies.[177]
Prognosis
Patients younger than 10 years have more favorable outcomes and clinical features—including extremity primaries, smaller tumors, and localized disease—than do older patients.[170,178] A meta-analysis also suggested that response to chemotherapy was correlated with improved survival.[179]
The following studies have reported multiple factors associated with unfavorable outcomes:
  • In a retrospective analysis of synovial sarcoma in children and adolescents who were treated in Germany and Italy, tumor size (>5 cm or ≤5 cm in greatest dimension) was an important predictor of EFS.[180] In this analysis, local invasiveness conferred an inferior probability of EFS, but surgical margins were not associated with clinical outcome.
  • In a single-institution retrospective analysis of 111 patients with synovial sarcoma who were younger than 22 years at diagnosis, larger tumor size, greater depth in tissue, greater local invasiveness, and more proximal tumor location were associated with poorer OS.[181][Level of evidence: 3iiA]
  • A multicenter analysis of 219 children from various treating centers, including Germany, SJCRH, Instituto Tumori, and MD Anderson Cancer Center, reported an estimated 5-year OS of 80% and EFS rate of 72%.[179] In this analysis, an interaction between tumor size and invasiveness was observed; in multivariate analysis, patients with large or invasive tumors or with Intergroup Rhabdomyosarcoma Study (IRS) group III disease (localized, incompletely resected or with biopsy only) and group IV disease (metastases at diagnosis) had decreased OS. Treatment with radiation therapy was related to improved OS (hazard ratio, 0.4; 95% CI, 0.2–0.7). In IRS group III patients, objective response to chemotherapy (18 of 30 [60%]) correlated with improved survival. In adults, factors such as International Union Against Cancer/American Joint Committee on Cancer stage III and stage IVA, tumor necrosis, truncal location, elevated mitotic rate, age, and histologic grade have been associated with a worse prognosis.[182-184]
  • Expression and genomic index prognostic signatures have been studied in synovial sarcoma. Complex genomic profiles, with greater rearrangement of the genome, are more common in adults than in younger patients with synovial sarcoma and are associated with a higher risk of metastasis.[185]
  • A review of 84 patients with localized synovial sarcoma who had information on fusion status (SYT-SSX) and histologic grading found no difference in OS according to these criteria. However, for tumor size at diagnosis, the study showed that patients with tumors between 5 cm and 10 cm had a worse prognosis than those with smaller tumors (P = .02), and patients with tumors larger than 10 cm had even worse OS (P = .0003).[186][Level of evidence: 3iiiA]
  • The German CWS group reviewed 27 evaluable patients younger than 21 years with pulmonary metastases among 296 patients with synovial sarcoma. Metastases involved the lungs in all patients. The 5-year EFS rate was 26%, and the OS rate was 30%. The most important prognostic factor at presentation was that the metastases were limited to one lesion in one lung or one lesion in both lungs (a group they termed oligometastatic). Treatment elements associated with superior survival were adequate local therapy of the primary tumor and, if feasible, for the metastases. The use of whole-lung irradiation did not correlate with better outcomes.[187][Level of evidence: 3iiA]
Treatment
Treatment options for synovial sarcoma include the following:
  1. Surgery. Radiation therapy and/or chemotherapy may be given before or after surgery.[9,10]
  2. Chemotherapy.
The COG and the European Pediatric Soft Tissue Sarcoma Study Group reported a combined analysis of 60 patients younger than 21 years with localized synovial sarcoma prospectively assigned to surgery without adjuvant radiation therapy or chemotherapy.[188] Enrollment was limited to patients with initial complete resection with histologically free margins, with a grade 2 tumor of any size or a grade 3 tumor 5 cm or smaller. The 3-year EFS was 90% (median follow-up, 5.2 years; range, 1.9–9.1). All eight events were local tumor recurrence; no metastatic recurrences were seen. All patients with recurrent disease were effectively treated with second-line therapy, resulting in 100% OS.
Synovial sarcoma appears to be more sensitive to chemotherapy than many other soft tissue sarcomas, and children with synovial sarcoma seem to have a better prognosis than do adults with synovial sarcoma.[15,173,184,189-193] The most commonly used regimens for the treatment of synovial sarcoma incorporate ifosfamide and doxorubicin.[179,192,194] Response rates to the ifosfamide and doxorubicin regimen are higher than in other nonrhabdomyosarcomatous soft tissue sarcomas.[195]
Studies have reported the following chemotherapy-associated treatment findings:
  • Several treatment centers advocate chemotherapy after resection and radiation therapy of synovial sarcoma in children and young adults.[179,180,196-198]
  • The International Society of Pediatric Oncology-Malignant Mesenchymal Tumors studies showed that select patients (young age, <5 cm resected tumors) with nonmetastatic synovial sarcoma can have excellent outcomes in the absence of radiation, but it is still unclear whether that approach obviates an advantage of radiation for local or regional control.[197]
  • A German trial suggested a benefit for postoperative chemotherapy in children with synovial sarcoma.[198]
  • A meta-analysis also suggested that chemotherapy may provide benefit.[179]
  • In the COG ARST0332 (NCT00346164) study, 129 patients with synovial sarcoma were prospectively treated using a risk-based therapy program (as detailed in the prognosis section), of which 43 were categorized as low risk, 66 as intermediate risk, and 20 as high risk. At a median follow-up of 2.6 years, 3-year EFS for low-, intermediate-, and high-risk groups were 83%, 79%, and 16%, respectively. The use of risk factor–directed therapy accurately predicted outcomes.[199]
  • The European Pediatric Soft Tissue Sarcoma Study Group performed a prospective study of patients younger than 21 years with synovial sarcoma (CCLG-EPSSG-NRSTS-2005 [NCT00334854]).[200][Level of evidence: 3iiA] Patients were stratified into the following three risk groups and nonrandomly assigned to treatment by risk group:
    • Low-risk patients had IRS group I tumors less than 5 cm in size and nonaxial primary tumors.
    • Intermediate-risk patients had no axial primary tumors and IRS group I tumors greater than 5 cm or IRS group II tumors.
    • High-risk patients included all patients with axial primary sites (head and neck, lung and pleura, trunk, retroperitoneal), IRS group III tumors, or N1 tumors.
    Outcomes for patients treated on the CCLG-EPSSG-NRSTS-2005 trial are described in Table 9.
    Table 9. Event-Free Survival (EFS) and Overall Survival (OS) in Patients With Low-, Intermediate-, and High-Risk Synovial Sarcoma Treated on the CCLG-EPSSG-NRSTS-2005 Trial
    Risk GroupTreatment3-Year EFS (%)3-Year OS (%)
    IRS = Intergroup Rhabdomyosarcoma Study; RT = radiation therapy.
    aChemotherapy was ifosfamide/doxorubicin, with doxorubicin omitted during radiation therapy.
    b59.4 Gy in cases without the option of secondary resection; 50.4 Gy as preoperative radiation therapy; 50.4, 54, and 59.4 Gy as postoperative radiation therapy, in the case of R0, R1, and R2 resections, respectively (no additional radiation therapy in the case of secondary complete resections with free margins, in children younger than 6 years).
    LowSurgery alone92100
    IntermediateSurgery, 3–6 cycles chemotherapya, ± RTb91100
    High (IRS group III)3 cycles of chemotherapya, surgery, 3 additional cycles of chemotherapy, ± RTb7794
    High (axial primary sites)Surgery, 6 cycles of chemotherapya, RTb78100
Recurrent synovial sarcoma NOS
Survival after relapse is poor (30%–40% at 5 years). Factors associated with outcome after relapse include duration of first remission (> or ≤ 18 months) and lack of a second remission.[201,202] In the German experience, surgical resection of metastatic deposits was the most common way to achieve a second complete remission.[202] Maintenance chemotherapy with oral trofosfamide, idarubicin, and etoposide or oral cyclophosphamide and intravenous vinblastine was administered on an individual basis.
Treatment options under clinical evaluation
Information about NCI-supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, refer to the ClinicalTrials.gov website.
The following is an example of a national and/or institutional clinical trial that is currently being conducted:
  • ADP 04511 (NCT01343043) (A Pilot Study of Genetically Engineered NY-ESO-1 Specific [c259] T Cells in HLA-A2+ Patients With Synovial Sarcoma): Patients with unresectable, metastatic, or recurrent synovial sarcoma undergo apheresis. Cells are shipped to a central laboratory where they undergo NY-ESO-1 transduction, expansion, and cryopreservation. Patients undergo lymphodepletion with fludarabine and cyclophosphamide, followed by an infusion of autologous transfected cells. Eligibility is restricted to patients with HLA type A2+, age older than 4 years, and weight greater than 18 kg.
  • NCT02683148 (Prasterone in Treating Patients With Synovial Sarcoma That Is Metastatic or Cannot Be Removed by Surgery): Prasterone (dehydroepiandrosterone [DHEA]) is a natural allosteric inhibitor of glucose-6-phosphate dehydrogenase (G6PD). G6PD is a key regulatory enzyme needed for the survival of synovial sarcoma. The investigators postulate that treatment with DHEA can inhibit the production of NADPH (reduced form of nicotinamide adenine dinucleotide phosphate) in synovial sarcoma and cause cell death.

Epithelioid sarcoma

Epithelioid sarcoma is a rare mesenchymal tumor of uncertain histogenesis that displays multilineage differentiation.[203]
Clinical presentation
Epithelioid sarcoma commonly presents as a slowly growing firm nodule based in the deep soft tissue; the proximal type predominantly affects adults and involves the axial skeleton and proximal sites. The tumor is highly aggressive and has a propensity for lymph node metastases.
Molecular features
Epithelioid sarcoma is characterized by inactivation of the SMARCB1 gene, which is present in both conventional and proximal types of epithelioid sarcoma.[204] This abnormality leads to increased dependence on EZH2 and tumor formation.[205]
Treatment
Treatment options for epithelioid sarcoma include the following:
  1. Chemotherapy.
  2. Surgery.
  3. Surgery preceded or followed by radiation therapy.
Patients should be carefully evaluated for the presence of involved lymph nodes; suspicious lymph nodes are biopsied. Surgical removal of primary and recurrent tumor(s) is the most effective treatment.[206][Level of evidence: 3iiiA] Because of the propensity of this disease to have occult metastasis to the lymph nodes, sentinel lymph node biopsy is recommended for epithelioid sarcoma of the extremities or buttocks in the absence of clinically (by imaging or physical examination) enlarged lymph nodes.[207]
In a review of 30 pediatric patients with epithelioid sarcoma (median age at presentation, 12 years), responses to chemotherapy were reported in 40% of patients using sarcoma-based regimens, and 60% of patients were alive at 5 years after initial diagnosis.[208] A single-institution retrospective review of 20 patients, which included children and adults (median age, 27.3 years), found no difference in the probability of recurrence between patients who received chemotherapy and those who did not receive chemotherapy and suggested that radiation therapy may be useful.[206]
In a phase I trial of the EZH2 inhibitor tazemetostat, two patients with INI1-negative epithelioid sarcoma had prolonged stable disease for more than 20 months after starting therapy.[209]
Treatment options under clinical evaluation
Information about NCI-supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, refer to the ClinicalTrials.gov website.
The following is an example of a national and/or institutional clinical trial that is currently being conducted:
  • NCT02601937 (A Phase I Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma): Patients with INI1-negative tumors are eligible for targeted treatment with an EZH2 inhibitor. This is a phase I, open-label, dose-escalation, and dose-expansion study with a twice-daily oral dose of tazemetostat.

Alveolar soft part sarcoma

Alveolar soft part sarcomas account for 1.4% of soft tissue sarcomas in patients younger than 20 years (refer to Table 1).
Clinical presentation
The median age at presentation is 25 years, and alveolar soft part sarcoma most commonly arises in the extremities but can occur in the oral and maxillofacial region.[210-212] Alveolar soft part sarcoma in children can present with evidence of metastatic disease.[213] Delayed metastases to the brain and lung are uncommon.[210]
In a series of 61 patients with alveolar soft part sarcoma who were treated in four consecutive CWS trials and the SoTiSaR registry, 46 patients presented with localized disease and 15 patients had evidence of metastasis at diagnosis.[214] Of the nine children with alveolar soft part sarcoma younger than 30 years who were treated between 1980 and 2014 at four major institutions, the median age at diagnosis was 17 years, and 64% of patients were female. The most common site of disease was the lower extremity, and 26 patients had an ASSPL-TFE3 translocation. The distribution by Intergroup Rhabdomyosarcoma Study (IRS) group was as follows: 19 patients with IRS I disease, 7 patients with IRS II disease, 5 patients with IRS III disease, and 38 patients with IRS IV disease.[215]
Molecular features
This tumor of uncertain histogenesis is characterized by a consistent chromosomal translocation t(X;17)(p11.2;q25) that fuses the ASPSCR1 gene with the TFE3 gene.[216,217]
Prognosis
Alveolar soft part sarcoma in children may have an indolent course.[213] Patients with alveolar soft part sarcoma may relapse several years after a prolonged period of apparent remission.[214,218] Because these tumors are rare, all children with alveolar soft part sarcoma should be considered for enrollment in prospective clinical trials. Information about ongoing clinical trials is available from the NCI website.
In a series of 19 treated patients, one group reported a 5-year OS rate of 80%, a 91% OS rate for patients with localized disease, a 100% OS rate for patients with tumors 5 cm or smaller, and a 31% OS rate for patients with tumors larger than 5 cm.[219] In another series of 33 patients, OS was 68% at 5 years from diagnosis and 53% at 10 years from diagnosis. Survival was better for smaller tumors (≤5 cm) and completely resected tumors.[220][Level of evidence: 3iiA]
A retrospective review of children and young adults younger than 30 years (median age, 17 years; range, 1.5–30 years) from four institutions identified 69 patients treated primarily with surgery between 1980 and 2014.[215][Level of evidence: 3iiA] The ASPL-TFE3translocation was present in all 26 patients tested. There were 19 patients with IRS postsurgical staging group I tumors (28%), 7 patients with IRS group II tumors (10%), 5 patients with IRS group III tumors (7%), and 38 patients with IRS group IV tumors (55%). The 5-year EFS was 80% and the OS was 87% for the 31 patients with localized tumors (IRS postsurgical groups I, II, and III). The 5-year EFS was 7% and the OS was 61% for the 38 patients with metastatic tumors (IRS postsurgical group IV).
In patients with alveolar soft part sarcoma, presentation with metastases is common and often has a prolonged indolent course. In a series of patients treated on consecutive studies from Germany, 15 of 61 patients (25%) presented with metastases, often miliary in nature. Despite lack of response to chemotherapy, the 5-year OS was 61%, with an EFS of 20%.[214]
Treatment
Treatment options for alveolar soft part sarcoma include the following:
  1. Surgery.
  2. Surgery preceded or followed by radiation therapy.[9,10]
  3. Targeted therapy (tyrosine kinase inhibitor).
The standard approach is complete resection of the primary lesion.[219] If complete excision is not feasible, radiation therapy is administered. A study from China reported on 18 patients with alveolar soft part sarcoma of the oral and maxillofacial region; 15 patients were younger than 30 years.[212][Level of evidence: 3iiDii] Surgical removal with negative margins was the primary treatment. All patients survived, and only one patient had metastatic disease recurrence.
A series of 51 pediatric patients aged 0 to 21 years with alveolar soft part sarcoma found an OS rate at 10 years of 78% and an EFS rate of about 63%. Patients with localized disease (n = 37) had a 10-year OS of 87%, and the 14 patients with metastases at diagnosis had a 10-year OS of 44%, partly resulting from surgical removal of primary tumor and lung metastases in some patients. Only 3 of 18 patients (17%) with measurable disease had a response to conventional antisarcoma chemotherapy, but two of four patients treated with sunitinib had a partial response.[210][Level of evidence: 3iiiA]
In a series of patients treated on consecutive studies from Germany, PFS for patients without metastases on presentation appeared to improve with complete resection of the primary tumor; the 5-year EFS was 100% for patients with completely resected tumors, compared with 50% for patients with microscopic or gross residual disease.[214]
There have been sporadic reports of objective responses to interferon-alpha and bevacizumab.[210,221,222]
Studies of tyrosine kinase inhibitors have observed the following:
  • A small retrospective study of nine adult patients with metastatic alveolar soft part sarcoma treated with sunitinib reported partial responses in five patients and stable disease in two patients.[223][Level of evidence: 3iiiDiv]
  • In another study, 15 patients with alveolar soft part sarcoma were treated with sunitinib, and six patients experienced partial responses. The median PFS was 19 months, and the median OS was 56 months. The 5-year OS rate was 49%.[224][Level of evidence: 3iiA] Five patients were treated with sunitinib for longer than 2 years.
  • In a phase II trial of cediranib, an inhibitor of all three known vascular epidermal growth factor receptors, 15 of 43 adult patients (35%) with metastatic alveolar soft part sarcoma had partial responses.[225][Level of evidence: 3iiDiv]
  • In an open-label trial that evaluated the efficacy of pazopanib in six adult patients, one patient achieved a partial response and five patients had stable disease.[226] In another trial of 30 adult patients treated with pazopanib, one patient experienced a complete response, seven patients experienced partial responses, and 17 patients had stable disease. The median PFS was 13.6 months.[227]
Treatment options under clinical evaluation for alveolar soft part sarcoma
Information about NCI-supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, refer to the ClinicalTrials.gov website.
The following is an example of a national and/or institutional clinical trial that is currently being conducted:
  • NCT01391962 (Sunitinib or Cediranib for Alveolar Soft Part Sarcoma): A phase II trial in which patients with metastatic alveolar soft part sarcoma are randomly assigned to either sunitinib or cediranib monotherapy, with crossover at disease progression. Patients aged 16 years and older are eligible. This study is being conducted at the Clinical Center of the National Institutes of Health.

Clear cell sarcoma of soft tissue

Clear cell sarcoma (formerly and inappropriately called malignant melanoma of soft parts) is a rare soft tissue sarcoma that typically involves the deep soft tissues of the extremities. It is also called clear cell sarcoma of tendons and aponeuroses. The tumor often affects adolescents and young adults.
Patients who have small, localized tumors with low mitotic rate and intermediate histologic grade fare best.[228]
Clinical presentation
The tumor most commonly affects the lower extremity, particularly the foot, heel, and ankle.[229,230] It has a high propensity for nodal dissemination, especially metastases to regional lymph nodes (12%–43%).[230,231] The tumor typically has an indolent clinical course.
Molecular features
Clear cell sarcoma of soft tissue is characterized by an EWSR1-ATF1 or EWSR1-CREB1 fusion.[232,233]
Treatment
Treatment options for clear cell sarcoma of soft tissue include the following:
  1. Surgery.
  2. Surgery preceded or followed by radiation therapy.[9,10]
In a series of 28 pediatric patients reported by the Italian and German Soft Tissue Cooperative Studies, the median age at diagnosis was 14 years and the lower extremity was the most common primary site (50%). Surgery with or without radiation therapy is the treatment of choice and offers the best chance for cure. In this series, 12 of 13 patients with completely resected tumors were cured. For patients with more advanced disease, the outcome is poor and chemotherapy is rarely effective.[234]; [235][Level of evidence: 3iiDii] In a study by the European Organization for Research and Treatment of Cancer, 26 patients with clear cell sarcoma who had metastatic disease and documented EWSR1rearrangements were treated with crizotinib.[236] One patient achieved a partial response, and 17 patients had stable disease.

Extraskeletal myxoid chondrosarcoma

Extraskeletal myxoid chondrosarcoma is relatively rare among soft tissue sarcomas, representing only 2.3% of all soft tissue sarcoma.[237] It has been reported in children and adolescents.[238]
Molecular features
Extraskeletal myxoid chondrosarcoma is a multinodular neoplasm. The rounded cells are arranged in cords and strands in a chondroitin sulfate myxoid background. Several cytogenetic abnormalities have been identified (refer to Table 2), with the most frequent being the translocation t(9;22)(q22;q12), involving the EWSR1-NR4A3 genes.[239]
Prognosis
The tumor has traditionally been considered of low-grade malignant potential.[240] However, recent reports from large institutions showed that extraskeletal myxoid chondrosarcoma has significant malignant potential, especially if patients are monitored for a long time.[241,242] Patients tend to have slow protracted courses. Nodal involvement has been well described. Local recurrence (57%) and metastatic spread to lungs (26%) have been reported.[242]
Treatment
Treatment options for extraskeletal myxoid chondrosarcoma include the following:
  1. Surgery.
  2. Radiation therapy.
Aggressive local control and resection of metastases led to OS rates of 87% at 5 years and 63% at 10 years. Tumors were relatively resistant to radiation therapy.[241] The therapeutic benefit of chemotherapy has not been established.
There may be potential genetic targets for small molecules, but these should be studied as part of a clinical trial. In an adult study, six of ten patients who received sunitinib achieved partial responses.[243]
Treatment options under clinical evaluation
Information about NCI-supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, refer to the ClinicalTrials.gov website.
The following is an example of a national and/or institutional clinical trial that is currently being conducted:
  • NCT02601937 (A Phase I Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma): Patients with INI1-negative tumors are eligible for targeted treatment with an EZH2 inhibitor. This is a phase I, open-label, dose-escalation, and dose-expansion study with a twice-daily oral dose of tazemetostat.

Extraskeletal Ewing sarcoma

(Refer to the PDQ summary on Ewing Sarcoma Treatment for more information.)

Desmoplastic small round cell tumor

Desmoplastic small round cell tumor is a rare primitive sarcoma.
Clinical presentation
Desmoplastic small round cell tumor most frequently involves the peritoneum in the abdomen, pelvis, and/or peritoneum into the scrotal sac, but it may occur in the kidney or other solid organs.[244-248] Dozens to hundreds of intraperitoneal implants are often found. The tumor occurs in males (85%) and may spread to the lungs and elsewhere.[248,249]
A large single-institution series of 65 patients compared computed tomography (CT) scans in most patients (n = 54) with positron emission tomography (PET)-CT scans (n = 11). PET-CT scans had very few false-negative results and detected metastatic sites missed on conventional CT scans.[249]
Molecular features
Cytogenetic studies of these tumors have demonstrated the recurrent translocation t(11;22)(p13;q12), which has been characterized as a fusion of the WT1 and EWSR1 genes.[247,250] The WT1-EWSR1 fusion confirms the diagnosis of desmoplastic small round cell tumor.
Prognosis
The overall prognosis for desmoplastic small round cell tumor remains extremely poor, with reported rates of death at 90%. Greater than 90% tumor resection either at presentation or after preoperative chemotherapy may be a favorable prognostic factor for OS.[251,252]; [253][Level of evidence: 3iiiA] Response to neoadjuvant chemotherapy and complete resection (near 100%) is associated with improved outcome.[248,254]
Treatment
There is no standard approach to the treatment of desmoplastic small round cell tumor.
Treatment options for desmoplastic small round cell tumor include the following:
  1. Surgery.
  2. Chemotherapy followed by surgery.
  3. Radiation therapy.
Complete surgical resections are rare, but critical for any improved survival. Treatment may include chemotherapy, surgery, and radiation therapy. Multiagent chemotherapy analogous to that used for sarcomas has been used, as well as total abdominal radiation therapy.[244,245,251,255-258]
The Center for International Blood and Marrow Transplant Research analyzed patients with desmoplastic small round cell tumor in their registry who received consolidation with high-dose chemotherapy and autologous stem cell reconstitution.[259] While this retrospective registry analysis suggested some benefit to this approach, other investigators have abandoned the approach because of excessive toxicity and lack of efficacy.[251]
A single-institution study reported that five of five patients with recurrent desmoplastic small round cell tumor had partial responses to treatment with the combination of vinorelbine, cyclophosphamide, and temsirolimus.[260]

Extra-renal (extracranial) rhabdoid tumor

Malignant rhabdoid tumors were first described in children with renal tumors in 1981 (refer to the Rhabdoid Tumors of the Kidney section in the PDQ summary on Wilms Tumor and Other Childhood Kidney Tumors Treatment for more information) and were later found in a variety of extra-renal sites. These tumors are uncommon and highly malignant, especially in children younger than 2 years.
Extra-renal (extracranial) rhabdoid tumors account for 2% of soft tissue sarcoma in patients younger than 20 years (refer to Table 1).
Molecular features
The first sizeable series of 26 children with extra-renal extracranial malignant rhabdoid tumor of soft tissues came from patients enrolled on the Intergroup Rhabdomyosarcoma Studies I through III during a review of pathology material. Only five patients (19%) were alive without disease.[261] Later, investigation of children with atypical teratoid/rhabdoid tumors of the brain, as well as those with renal and extra-renal malignant rhabdoid tumors, found germline and acquired mutations of the SMARCB1 gene in all 29 tumors tested.[262] Rhabdoid tumors may be associated with germline mutations of the SMARCB1gene and may be inherited from an apparently unaffected parent.[263] This observation was extended to 32 malignant rhabdoid tumors at all sites in patients whose mean age at diagnosis was 12 months.[264]
Prognosis
In a SEER study of 229 patients with renal, central nervous system (CNS), and extra-renal malignant rhabdoid tumor, patients aged 2 to 18 years, limited extent of tumor, and delivery of radiation therapy were shown to affect the outcome favorably compared with other patients (P < .002 for each comparison). Site of the primary tumor was not prognostically significant. OS at 5 years was 33%.[265]
Treatment
Treatment options for extra-renal (extracranial) rhabdoid tumor include the following:[266][Level of evidence: 3iA]; [267,268][Level of evidence: 3iiiB]
  1. Surgical removal when possible.
  2. Chemotherapy as used for soft tissue sarcomas (but no single regimen is currently accepted as best).
  3. Radiation therapy.
Responses to alisertib have been documented in four patients with CNS atypical teratoid/rhabdoid tumors.[269] (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment summary for more information about CNS atypical teratoid/rhabdoid tumors.)
Treatment options under clinical evaluation
Information about NCI-supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, refer to the ClinicalTrials.gov website.
The following are examples of national and/or institutional clinical trials that are currently being conducted:
  • NCT02601937 (A Phase I Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma): Patients with INI1-negative tumors are eligible for targeted treatment with an EZH2 inhibitor. This is a phase I, open-label, dose-escalation, and dose-expansion study with a twice-daily oral dose of tazemetostat.
  • APEC1621 (NCT03155620) (Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients with Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders): NCI-COG Pediatric Molecular Analysis for Therapeutic Choice (MATCH), referred to as Pediatric MATCH, will match targeted agents with specific molecular changes identified using a next-generation sequencing targeted assay of more than 3,000 different mutations across more than 160 genes in refractory and recurrent solid tumors. Children and adolescents aged 1 to 21 years are eligible for the trial.
    Tumor tissue from progressive or recurrent disease must be available for molecular characterization. Patients with tumors that have molecular variants addressed by treatment arms included in the trial will be offered treatment on Pediatric MATCH. Additional information can be obtained on the ClinicalTrials.gov website for APEC1621 (NCT03155620).

Neoplasms with perivascular epithelioid cell differentiation (PEComas)

Risk factors and molecular features
Benign PEComas are common in tuberous sclerosis, an autosomal dominant syndrome that also predisposes to renal cell cancer and brain tumors. Tuberous sclerosis is caused by germline inactivation of either TSC1 (9q34) or TSC2 (16p13.3), and the same tumor suppressor genes are inactivated somatically in sporadic PEComas.[270] Inactivation of either gene results in stimulation of the mTOR pathway, providing the basis for the treatment of nonsurgically curable PEComas with mTOR inhibitors.[271,272] A small proportion of PEComas have TFE3 rearrangements with fusions involving various genes, including SFPQ/PSF and RAD51B.[273]
Clinical presentation
PEComas occur in various rare gastrointestinal, pulmonary, gynecologic, and genitourinary sites. Soft tissue, visceral, and gynecologic PEComas are more commonly seen in middle-aged female patients and are usually not associated with the tuberous sclerosis complex.[274] The disease course may be indolent.
Prognosis
Most PEComas have a benign clinical course, but malignant behavior has been reported and can be predicted based on the size of the tumor, mitotic rate, and presence of necrosis.[275]
Treatment
Treatment options have not been defined. Treatment may include surgery or observation followed by surgery when the tumor is large.[276]
Clinical activity with mTOR inhibitors, such as sirolimus, in tumors with evidence of mTORC1activation and TSC loss has been well documented.[277]

Undifferentiated/Unclassified Sarcoma

From 1972 to 2006, patients with undifferentiated soft tissue sarcoma were eligible for participation in rhabdomyosarcoma trials coordinated by the Intergroup Rhabdomyosarcoma Study Group and the COG. The rationale was the observation that patients with undifferentiated soft tissue sarcoma had sites of disease and outcomes that were similar to those in patients with alveolar rhabdomyosarcoma. Therapeutic trials for adults with soft tissue sarcoma include patients with undifferentiated soft tissue sarcoma and other histologies, which are treated similarly, using ifosfamide and doxorubicin, and sometimes with other chemotherapy agents, surgery, and radiation therapy.
In the COG ARST0332 (NCT00346164) trial, patients with high-grade undifferentiated sarcoma were treated with an ifosfamide and doxorubicin-based regimen and were treated with rhabdomyosarcoma-directed therapies in previous Intergroup Rhabdomyosarcoma Study Group studies, with a 5-year survival estimate for nonmetastatic patients of 72%.[278][Level of evidence: 3iiA]
In a report of 32 patients with undifferentiated soft tissue sarcomas who were enrolled on the ARST0332 (NCT00346164) trial, the median age at enrollment was 13.6 years, and two-thirds of the patients were male. The most common primary sites were the paraspinal region and extremities. Five patients presented with metastatic disease.[279]
  • The 5-year EFS rate was 71%, and the OS rate was 83%.
  • Of the nine children with low-risk disease (localized low-grade resected disease or localized high-grade disease <5 cm resected with negative margins) who were treated with surgery or radiation therapy only, the 5-year EFS rate was 65% and the OS rate was 100%, suggesting that patients with recurrent disease can be salvaged with additional therapy.
  • The remaining 23 patients had either intermediate-risk disease (resected high-grade tumor >5 cm, unresected high-grade tumor >5 cm) or high-risk disease (metastasis to lymph nodes or distant sites) and were treated with chemoradiation therapy and delayed surgery when feasible. The 5-year EFS rate was 73%, and the OS estimate was 77%.
  • Copy number aberrations were common, most frequently involving loss of 1p (25%), gain of 1q (25%), gain of chromosome 8 (25%), and gain of chromosome 2 (16%). These alterations were more commonly seen in patients with intermediate-risk or high-risk tumors, and there was a strong association between loss of chromosome 1p or gain of chromosome 1q and inferior clinical outcomes. Co-occurrence of 1q gain and 1p loss was associated with a particularly poor clinical outcome (5-year EFS and OS of 20%). Next-generation sequencing identified oncogenic fusions in eight of ten samples, which included BCOR and CIC rearrangements, as well as COL1A1-PDGFBKIAA1549-BRAF, and SAMD-SASH1 fusions.

Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma (high-grade)

At one time, malignant fibrous histiocytoma was the single most common histotype among adults with soft tissue sarcomas. Since it was first recognized in the early 1960s, malignant fibrous histiocytoma has been plagued by controversy in terms of both its histogenesis and its validity as a clinicopathologic entity. The latest WHO classification no longer includes malignant fibrous histiocytoma as a distinct diagnostic category but rather as a subtype of an undifferentiated pleomorphic sarcoma.[4,280]
This entity accounts for 2% to 6% of all childhood soft tissue sarcomas.[281]
Molecular features
Undifferentiated pleomorphic sarcoma was most often called malignant fibrous histiocytoma in the past. Historically, this entity has been difficult to evaluate because of the shifting diagnostic criteria. Analysis of 70 cases diagnosed as malignant fibrous histiocytosis of no specific type, storiform or pleomorphic malignant fibrous histiocytoma, pleomorphic sarcoma, or undifferentiated pleomorphic sarcoma showed a highly complex karyotype with no specific recurrent aberrations.[282]
Undifferentiated sarcomas with 12q13–15 amplification, including MDM2 and CDK4, are best classified as dedifferentiated liposarcomas;[282] the relationship between this tumor and the family of undifferentiated/unclassified tumors with spindle cell morphology remains relatively undefined.
Risk factors
These tumors can arise in previously irradiated sites or as a second malignancy in patients with retinoblastoma.
Clinical presentation and treatment
These tumors occur mainly in the second decade of life. In a series of ten patients, the median age was 10 years and the tumor was most commonly located in the extremities. In this series, all tumors were localized and five of nine (for whom follow-up was available) were alive and in first remission.[281] In another series of 17 pediatric patients with malignant fibrous histiocytoma, the median age at diagnosis was 5 years and the extremities were involved in eight cases.[283] All patients with metastatic disease died and two patients experienced a clinical response to a doxorubicin-based regimen.
(Refer to the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about the treatment of malignant fibrous histiocytoma of bone.)

Undifferentiated round cell sarcomas with BCOR-CCNB3 rearrangements

Molecular features
Undifferentiated round cell sarcomas are characterized by paracentric inversion of chromosome X and a BCOR-CCNB3 rearrangement; alternative BCOR partners, including MAML3 and ZC3H7B, have also been reported.[284] Despite clinical pathologic similarities to Ewing sarcoma, these tumors are biologically different by expression profiling and single-nucleotide polymorphism array analysis.
Clinical presentation
Undifferentiated round cell sarcoma accounts for about 5% of all EWSR1-negative rearranged sarcomas and more commonly affects males. Over 70% of cases occur in patients younger than 18 years (median age at diagnosis, 13–15 years).[285,286][Level of evidence: 3iiA] These tumors more commonly arise in the bones of the pelvis and extremities, and metastases are present in about 30% of cases.
Treatment
When treated with Ewing sarcoma–like therapies, 75% of patients show significant treatment-associated pathologic responses. In one series of 36 cases, the 3-year and 5-year survival rates were 93% and 72%, respectively.[285][Level of evidence: 3iiA] In another series of 26 patients, the 5-year OS was 76.5%, and survival was better for patients who received induction therapy using a Ewing sarcoma–type regimen.[287][Level of evidence: 3iiA] Most of the tumors in these series arose in the bone.
(Refer to the PDQ summary on Ewing Sarcoma Treatment for more information.)

Undifferentiated round cell sarcomas with CIC-DUX rearrangements

Molecular features
These tumors are characterized by a CIC-DUX fusion resulting from a recurrent t(4;19) or t(10;19) and are the most common EWSR1-FUS fusion–negative undifferentiated round cell sarcomas.[288]
Clinical presentation
These tumors most commonly affect young adults, with 50% of cases occurring between the ages of 21 and 40 years. In a series of 115 cases, the median age at diagnosis was 32 years, and 22% of cases occurred in patients younger than 18 years.[286,288] This entity more commonly affects males and usually originates from the soft tissues of the trunk and extremities.
Treatment
In a series of 115 cases of CIC-rearranged round cell sarcomas, 57 patients had adequate follow-up information.[288] Nine patients presented with metastases, and 53% of patients with localized disease experienced a recurrence commonly involving the lung. Patients treated with neoadjuvant chemotherapy had an inferior survival compared with patients who were treated with up-front surgical resection; however, this difference might have been related to a larger tumor size at presentation in the former group. The 2-year and 5-year survival rates were 53% and 43%, respectively. These survival rates are significantly lower than the survival rates observed in patients with Ewing sarcoma. Further study is required to identify optimal treatments for this disease.

Vascular Tumors

Vascular tumors vary from hemangiomas, which are always considered benign, to angiosarcomas, which are highly malignant.[289] Malignant vascular tumors include the following subtypes:

Epithelioid hemangioendothelioma

Incidence and outcome
This tumor was first described in soft tissue by Weiss and Enzinger in 1982. Epithelioid hemangioendotheliomas can occur at younger ages, but the peak incidence is in the fourth and fifth decades of life. The tumors can have an indolent or very aggressive course, with overall survival of 73% at 5 years. There are case reports of patients with untreated multiple lesions who have a very benign course compared with other patients who have a very aggressive course. Some pathologists have tried to stratify patients to evaluate risks and adjust treatment, but more research is needed.[290-296]
The presence of effusions, tumor size larger than 3 cm, and a high mitotic index (>3 mitoses/50 high-power fields) have been associated with unfavorable outcomes.[292]
Histopathology and molecular features
WWTR1-CAMTA1 gene fusion has been found in a large percentage of patients; less commonly, a YAP1-TFE3 gene fusion has been reported.[290] These fusions are not directly targetable with current medicines. Monoclonality has been described in multiple liver lesions, suggesting a metastatic process.
Histologically, these lesions are characterized as epithelioid lesions arranged in nests, strands, and trabecular patterns, with infrequent vascular spaces. Features that may be associated with aggressive clinical behavior include cellular atypia, one or more mitoses per 10 high-power fields, an increased proportion of spindled cells, focal necrosis, and metaplastic bone formation.[292]
The number of pediatric patients reported in the literature is limited.
Clinical presentation and diagnostic evaluation
Common sites of involvement are liver alone (21%), liver plus lung (18%), lung alone (12%), and bone alone (14%).[292,297,298] Clinical presentation depends on site of involvement, as follows:
  • Liver: Hepatic nodules have central vascularity on ultrasound, contrast-enhancing lesions by computed tomography, and low T1 signal and moderate T2 signal on magnetic resonance imaging.
  • Lung: Pulmonary epithelioid hemangioendothelioma may be an asymptomatic finding on chest x-ray or be associated with pleuritic pain, hemoptysis, anemia, and fibrosis.
  • Bone: Bone metastasis may be associated with pathologic fracture. On x-rays, they are well-defined osteolytic lesions and can be multiple or solitary.
  • Soft tissue: Thirty percent of soft tissue cases are associated with metastases, and when present, can have a very aggressive course, with limited response to chemotherapy.
  • Skin: Cutaneous lesions can be raised and nodular or can be warm red-brown plaques.
Treatment of epithelioid hemangioendothelioma
Treatment options for epithelioid hemangioendothelioma include the following:
  1. Observation.
  2. Surgery.
  3. Immunotherapy.
  4. Targeted therapy.
  5. Chemotherapy.
For indolent cases, observation is warranted. For more aggressive cases, multiple medications have been used, including interferon, thalidomide, sorafenib, pazopanib, and sirolimus.[299] The most aggressive cases are treated with angiosarcoma-type chemotherapy. Surgery is used when possible. Liver transplantation has been used with aggressive liver lesions, both with and without metastases.[292,300-303]
Patients or families who desire additional disease-directed therapy should consider entering trials of novel therapeutic approaches because no standard agents have demonstrated clinically significant activity.
Regardless of whether a decision is made to pursue disease-directed therapy at the time of progression, palliative care remains a central focus of management. This ensures that quality of life is maximized while attempting to reduce symptoms and stress related to the terminal illness.
Treatment options under clinical evaluation for epithelioid hemangioendothelioma
Information about National Cancer Institute (NCI)–supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, refer to the ClinicalTrials.gov website.
The following are examples of national and/or institutional clinical trials that are currently being conducted:
  1. NCT03148275 (Trametinib in Treating Patients with Epithelioid Hemangioendothelioma That Is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery): This is a phase II trial assessing the efficacy of trametinib, with patient-reported outcomes as secondary aims.
  2. NCT01532687 (Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma): This randomized phase II trial studies how well gemcitabine hydrochloride works with or without pazopanib hydrochloride in treating patients with refractory soft tissue sarcoma.
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Angiosarcoma of the soft tissue

Incidence
Angiosarcoma is a rare (accounting for 2% of sarcomas), aggressive, vascular tumor that can arise in any part of the body, but is more common in the soft tissue. Angiosarcoma has an estimated incidence of 2 cases per 1 million people; in the United States, it annually affects approximately 600 people who are typically aged 60 to 70 years.[304]
Angiosarcomas are extremely rare in children and it is unclear if the pathophysiology of this tumor is different in the pediatric population. Cases have been reported in neonates and toddlers, with presentation of multiple cutaneous lesions and liver lesions, some of which are GLUT1 positive.[305-308] Most angiosarcomas involve the skin and superficial soft tissue, although the liver, spleen, and lung can be affected; bone is rarely affected.
Risk factors
Established risk factors include the following:[309]
  • Vinyl chloride exposure.
  • Radiation exposure.
  • Chronic lymphedema from any cause, including Stewart-Treves syndrome.
Histopathology and molecular features
Angiosarcomas are largely aneuploid tumors. The rare cases of angiosarcoma that arise from benign lesions such as hemangiomas have a distinct pathway that needs to be investigated. MYC amplification is seen in radiation-induced angiosarcoma. KDR-VEGFR2mutations and FLT4-VEGFR3 amplifications have been seen with a frequency of less than 50%.[309]
Histopathologic diagnosis can be very difficult because there can be areas of varied atypia. The common feature is an irregular network of channels in a dissective pattern along dermal collagen bundles. There is varied cellular shape, size, mitosis, endothelial multilayering, and papillary formation. Epithelioid cells can also be present. Necrosis and hemorrhage are common. Tumors stain for factor VIII, CD31, and CD34. Some liver lesions can mimic infantile hemangiomas and have focal GLUT1 positivity. Nomenclature of these liver lesions has been difficult and confusing with use of terminology from 1971 (e.g., type I hemangioendothelioma: infantile hemangioma; type II hemangioendothelioma: low-grade angiosarcoma; type III hemangioendothelioma: high-grade angiosarcoma).[306]
Treatment of angiosarcoma of the soft tissue
Treatment options for angiosarcoma of the soft tissue include the following:
  1. Surgery (localized disease).
  2. Radiation therapy (localized cutaneous disease in adults).
  3. Surgery, chemotherapy, and radiation therapy (metastatic disease).
Localized disease is cured by aggressive surgery. Complete surgical excision appears to be crucial for angiosarcomas and lymphangiosarcomas despite evidence of tumor shrinkage in some patients who were treated with local or systemic therapy.[307,310-312] A review of 222 patients (median age, 62 years; range, age 15–90 years) showed an overall disease-specific survival (DSS) rate of 38% at 5 years. Five-year DSS was 44% in 138 patients with localized, resected tumors but only 16% in 43 patients with metastases at diagnosis.[312] Data on liver transplantation for localized angiosarcoma are limited.[313][Level of evidence: 3iiA]
Localized disease, especially cutaneous angiosarcoma, can be treated with radiation therapy. Most of these reported cases are in adults.[314]
Multimodal treatment with surgery, systemic chemotherapy, and radiation therapy is used for metastatic disease, although it is rarely curative.[315] Disease control is the objective in metastatic angiosarcoma, with published progression-free survival rates between 3 months and 7 months [316] and a median overall survival (OS) rate of 14 months to 18 months.[317] In both adults and children, 5-year OS rates between 20% and 35% are reported.[307,308,318]
In a child diagnosed with angiosarcoma secondary to malignant transformation from infantile hemangioma, response to treatment with bevacizumab, a monoclonal antibody against vascular endothelial growth factor, combined with systemic chemotherapy, has been reported.[305,315] A report of eight cases of liver angiosarcoma in children highlighted the misuse of the term hemangioendothelioma and the importance of early diagnosis and treatment of these tumors.[319]
Biologic agents that inhibit angiogenesis have shown activity in adults with angiosarcoma.[306,318]
Patients or families who desire additional disease-directed therapy should consider entering trials of novel therapeutic approaches because no standard agents have demonstrated clinically significant activity.
Regardless of whether a decision is made to pursue disease-directed therapy at the time of progression, palliative care remains a central focus of management. This ensures that quality of life is maximized while attempting to reduce symptoms and stress related to the terminal illness.
Treatment options under clinical evaluation for angiosarcoma of the soft tissue
Information about National Cancer Institute (NCI)–supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, refer to the ClinicalTrials.gov website.
The following is an example of a national and/or institutional clinical trial that is currently being conducted:
  1. NCT01532687 (Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma): This randomized phase II trial studies how well gemcitabine hydrochloride works with or without pazopanib hydrochloride in treating patients with refractory soft tissue sarcoma.
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References
  1. Ferrari A, Casanova M, Collini P, et al.: Adult-type soft tissue sarcomas in pediatric-age patients: experience at the Istituto Nazionale Tumori in Milan. J Clin Oncol 23 (18): 4021-30, 2005. [PUBMED Abstract]
  2. Stanelle EJ, Christison-Lagay ER, Sidebotham EL, et al.: Prognostic factors and survival in pediatric and adolescent liposarcoma. Sarcoma 2012: 870910, 2012. [PUBMED Abstract]
  3. Alaggio R, Coffin CM, Weiss SW, et al.: Liposarcomas in young patients: a study of 82 cases occurring in patients younger than 22 years of age. Am J Surg Pathol 33 (5): 645-58, 2009. [PUBMED Abstract]
  4. Fletcher CDM, Bridge JA, Hogendoorn P, et al., eds.: WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. Lyon, France: IARC Press, 2013.
  5. Sreekantaiah C, Karakousis CP, Leong SP, et al.: Cytogenetic findings in liposarcoma correlate with histopathologic subtypes. Cancer 69 (10): 2484-95, 1992. [PUBMED Abstract]
  6. Sugiura H, Takahashi M, Katagiri H, et al.: Additional wide resection of malignant soft tissue tumors. Clin Orthop (394): 201-10, 2002. [PUBMED Abstract]
  7. Cecchetto G, Guglielmi M, Inserra A, et al.: Primary re-excision: the Italian experience in patients with localized soft-tissue sarcomas. Pediatr Surg Int 17 (7): 532-4, 2001. [PUBMED Abstract]
  8. Chui CH, Spunt SL, Liu T, et al.: Is reexcision in pediatric nonrhabdomyosarcoma soft tissue sarcoma necessary after an initial unplanned resection? J Pediatr Surg 37 (10): 1424-9, 2002. [PUBMED Abstract]
  9. Bahig H, Roberge D, Bosch W, et al.: Agreement among RTOG sarcoma radiation oncologists in contouring suspicious peritumoral edema for preoperative radiation therapy of soft tissue sarcoma of the extremity. Int J Radiat Oncol Biol Phys 86 (2): 298-303, 2013. [PUBMED Abstract]
  10. Baldini EH, Wang D, Haas RL, et al.: Treatment Guidelines for Preoperative Radiation Therapy for Retroperitoneal Sarcoma: Preliminary Consensus of an International Expert Panel. Int J Radiat Oncol Biol Phys 92 (3): 602-12, 2015. [PUBMED Abstract]
  11. La Quaglia MP, Spiro SA, Ghavimi F, et al.: Liposarcoma in patients younger than or equal to 22 years of age. Cancer 72 (10): 3114-9, 1993. [PUBMED Abstract]
  12. Lee ATJ, Thway K, Huang PH, et al.: Clinical and Molecular Spectrum of Liposarcoma. J Clin Oncol 36 (2): 151-159, 2018. [PUBMED Abstract]
  13. Beane JD, Yang JC, White D, et al.: Efficacy of adjuvant radiation therapy in the treatment of soft tissue sarcoma of the extremity: 20-year follow-up of a randomized prospective trial. Ann Surg Oncol 21 (8): 2484-9, 2014. [PUBMED Abstract]
  14. Ferrari A, Casanova M, Spreafico F, et al.: Childhood liposarcoma: a single-institutional twenty-year experience. Pediatr Hematol Oncol 16 (5): 415-21, 1999 Sep-Oct. [PUBMED Abstract]
  15. Cecchetto G, Alaggio R, Dall'Igna P, et al.: Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies. Cancer 104 (9): 2006-12, 2005. [PUBMED Abstract]
  16. Huh WW, Yuen C, Munsell M, et al.: Liposarcoma in children and young adults: a multi-institutional experience. Pediatr Blood Cancer 57 (7): 1142-6, 2011. [PUBMED Abstract]
  17. Gronchi A, Bui BN, Bonvalot S, et al.: Phase II clinical trial of neoadjuvant trabectedin in patients with advanced localized myxoid liposarcoma. Ann Oncol 23 (3): 771-6, 2012. [PUBMED Abstract]
  18. Demetri GD, von Mehren M, Jones RL, et al.: Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial. J Clin Oncol 34 (8): 786-93, 2016. [PUBMED Abstract]
  19. Baruchel S, Pappo A, Krailo M, et al.: A phase 2 trial of trabectedin in children with recurrent rhabdomyosarcoma, Ewing sarcoma and non-rhabdomyosarcoma soft tissue sarcomas: a report from the Children's Oncology Group. Eur J Cancer 48 (4): 579-85, 2012. [PUBMED Abstract]
  20. Demetri GD, Schöffski P, Grignani G, et al.: Activity of Eribulin in Patients With Advanced Liposarcoma Demonstrated in a Subgroup Analysis From a Randomized Phase III Study of Eribulin Versus Dacarbazine. J Clin Oncol 35 (30): 3433-3439, 2017. [PUBMED Abstract]
  21. Schafer ES, Rau RE, Berg S, et al.: A phase 1 study of eribulin mesylate (E7389), a novel microtubule-targeting chemotherapeutic agent, in children with refractory or recurrent solid tumors: A Children's Oncology Group Phase 1 Consortium study (ADVL1314). Pediatr Blood Cancer 65 (8): e27066, 2018. [PUBMED Abstract]
  22. Wang L, Motoi T, Khanin R, et al.: Identification of a novel, recurrent HEY1-NCOA2 fusion in mesenchymal chondrosarcoma based on a genome-wide screen of exon-level expression data. Genes Chromosomes Cancer 51 (2): 127-39, 2012. [PUBMED Abstract]
  23. Nyquist KB, Panagopoulos I, Thorsen J, et al.: Whole-transcriptome sequencing identifies novel IRF2BP2-CDX1 fusion gene brought about by translocation t(1;5)(q42;q32) in mesenchymal chondrosarcoma. PLoS One 7 (11): e49705, 2012. [PUBMED Abstract]
  24. Frezza AM, Cesari M, Baumhoer D, et al.: Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study. Eur J Cancer 51 (3): 374-81, 2015. [PUBMED Abstract]
  25. Schneiderman BA, Kliethermes SA, Nystrom LM: Survival in Mesenchymal Chondrosarcoma Varies Based on Age and Tumor Location: A Survival Analysis of the SEER Database. Clin Orthop Relat Res 475 (3): 799-805, 2017. [PUBMED Abstract]
  26. Kawaguchi S, Weiss I, Lin PP, et al.: Radiation therapy is associated with fewer recurrences in mesenchymal chondrosarcoma. Clin Orthop Relat Res 472 (3): 856-64, 2014. [PUBMED Abstract]
  27. Dantonello TM, Int-Veen C, Leuschner I, et al.: Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults: experiences of the CWS and COSS study groups. Cancer 112 (11): 2424-31, 2008. [PUBMED Abstract]
  28. Bishop MW, Somerville JM, Bahrami A, et al.: Mesenchymal Chondrosarcoma in Children and Young Adults: A Single Institution Retrospective Review. Sarcoma 2015: 608279, 2015. [PUBMED Abstract]
  29. Morioka H, Takahashi S, Araki N, et al.: Results of sub-analysis of a phase 2 study on trabectedin treatment for extraskeletal myxoid chondrosarcoma and mesenchymal chondrosarcoma. BMC Cancer 16: 479, 2016. [PUBMED Abstract]
  30. Thampi S, Matthay KK, Boscardin WJ, et al.: Clinical Features and Outcomes Differ between Skeletal and Extraskeletal Osteosarcoma. Sarcoma 2014: 902620, 2014. [PUBMED Abstract]
  31. Jour G, Wang L, Middha S, et al.: The molecular landscape of extraskeletal osteosarcoma: A clinicopathological and molecular biomarker study. J Pathol Clin Res 2 (1): 9-20, 2016. [PUBMED Abstract]
  32. Sordillo PP, Hajdu SI, Magill GB, et al.: Extraosseous osteogenic sarcoma. A review of 48 patients. Cancer 51 (4): 727-34, 1983. [PUBMED Abstract]
  33. Paludo J, Fritchie K, Haddox CL, et al.: Extraskeletal Osteosarcoma: Outcomes and the Role of Chemotherapy. Am J Clin Oncol 41 (9): 832-837, 2018. [PUBMED Abstract]
  34. Longhi A, Bielack SS, Grimer R, et al.: Extraskeletal osteosarcoma: A European Musculoskeletal Oncology Society study on 266 patients. Eur J Cancer 74: 9-16, 2017. [PUBMED Abstract]
  35. Nieuwenhuis MH, Casparie M, Mathus-Vliegen LM, et al.: A nation-wide study comparing sporadic and familial adenomatous polyposis-related desmoid-type fibromatoses. Int J Cancer 129 (1): 256-61, 2011. [PUBMED Abstract]
  36. Rossato M, Rigotti M, Grazia M, et al.: Congenital hypertrophy of the retinal pigment epithelium (CHRPE) and familial adenomatous polyposis (FAP). Acta Ophthalmol Scand 74 (4): 338-42, 1996. [PUBMED Abstract]
  37. Baker RH, Heinemann MH, Miller HH, et al.: Hyperpigmented lesions of the retinal pigment epithelium in familial adenomatous polyposis. Am J Med Genet 31 (2): 427-35, 1988. [PUBMED Abstract]
  38. Kattentidt Mouravieva AA, Geurts-Giele IR, de Krijger RR, et al.: Identification of Familial Adenomatous Polyposis carriers among children with desmoid tumours. Eur J Cancer 48 (12): 1867-74, 2012. [PUBMED Abstract]
  39. Wang WL, Nero C, Pappo A, et al.: CTNNB1 genotyping and APC screening in pediatric desmoid tumors: a proposed algorithm. Pediatr Dev Pathol 15 (5): 361-7, 2012 Sep-Oct. [PUBMED Abstract]
  40. Lewis JJ, Boland PJ, Leung DH, et al.: The enigma of desmoid tumors. Ann Surg 229 (6): 866-72; discussion 872-3, 1999. [PUBMED Abstract]
  41. Lazar AJ, Tuvin D, Hajibashi S, et al.: Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol 173 (5): 1518-27, 2008. [PUBMED Abstract]
  42. Faulkner LB, Hajdu SI, Kher U, et al.: Pediatric desmoid tumor: retrospective analysis of 63 cases. J Clin Oncol 13 (11): 2813-8, 1995. [PUBMED Abstract]
  43. Gounder MM, Mahoney MR, Van Tine BA, et al.: Sorafenib for Advanced and Refractory Desmoid Tumors. N Engl J Med 379 (25): 2417-2428, 2018. [PUBMED Abstract]
  44. Merchant NB, Lewis JJ, Woodruff JM, et al.: Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer 86 (10): 2045-52, 1999. [PUBMED Abstract]
  45. Honeyman JN, Theilen TM, Knowles MA, et al.: Desmoid fibromatosis in children and adolescents: a conservative approach to management. J Pediatr Surg 48 (1): 62-6, 2013. [PUBMED Abstract]
  46. Bonvalot S, Ternès N, Fiore M, et al.: Spontaneous regression of primary abdominal wall desmoid tumors: more common than previously thought. Ann Surg Oncol 20 (13): 4096-102, 2013. [PUBMED Abstract]
  47. Bonvalot S, Eldweny H, Haddad V, et al.: Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol 34 (4): 462-8, 2008. [PUBMED Abstract]
  48. Merchant TE, Nguyen D, Walter AW, et al.: Long-term results with radiation therapy for pediatric desmoid tumors. Int J Radiat Oncol Biol Phys 47 (5): 1267-71, 2000. [PUBMED Abstract]
  49. Zelefsky MJ, Harrison LB, Shiu MH, et al.: Combined surgical resection and iridium 192 implantation for locally advanced and recurrent desmoid tumors. Cancer 67 (2): 380-4, 1991. [PUBMED Abstract]
  50. Weiss AJ, Lackman RD: Low-dose chemotherapy of desmoid tumors. Cancer 64 (6): 1192-4, 1989. [PUBMED Abstract]
  51. Klein WA, Miller HH, Anderson M, et al.: The use of indomethacin, sulindac, and tamoxifen for the treatment of desmoid tumors associated with familial polyposis. Cancer 60 (12): 2863-8, 1987. [PUBMED Abstract]
  52. Soto-Miranda MA, Sandoval JA, Rao B, et al.: Surgical treatment of pediatric desmoid tumors. A 12-year, single-center experience. Ann Surg Oncol 20 (11): 3384-90, 2013. [PUBMED Abstract]
  53. Ferrari A, Orbach D, Affinita MC, et al.: Evidence of hydroxyurea activity in children with pretreated desmoid-type fibromatosis: A new option in the armamentarium of systemic therapies. Pediatr Blood Cancer : e27472, 2018. [PUBMED Abstract]
  54. O'Dea FJ, Wunder J, Bell RS, et al.: Preoperative radiotherapy is effective in the treatment of fibromatosis. Clin Orthop Relat Res (415): 19-24, 2003. [PUBMED Abstract]
  55. Skapek SX, Ferguson WS, Granowetter L, et al.: Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol 25 (5): 501-6, 2007. [PUBMED Abstract]
  56. Gandhi MM, Nathan PC, Weitzman S, et al.: Successful treatment of life-threatening generalized infantile myofibromatosis using low-dose chemotherapy. J Pediatr Hematol Oncol 25 (9): 750-4, 2003. [PUBMED Abstract]
  57. Gega M, Yanagi H, Yoshikawa R, et al.: Successful chemotherapeutic modality of doxorubicin plus dacarbazine for the treatment of desmoid tumors in association with familial adenomatous polyposis. J Clin Oncol 24 (1): 102-5, 2006. [PUBMED Abstract]
  58. Constantinidou A, Jones RL, Scurr M, et al.: Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis. Eur J Cancer 45 (17): 2930-4, 2009. [PUBMED Abstract]
  59. Ananth P, Werger A, Voss S, et al.: Liposomal doxorubicin: Effective treatment for pediatric desmoid fibromatosis. Pediatr Blood Cancer 64 (7): , 2017. [PUBMED Abstract]
  60. Bisogno G, Tagarelli A, Stramare R, et al.: Hydroxyurea treatment can avoid the need for aggressive surgery in pediatric fibromatosis. J Pediatr Hematol Oncol 35 (4): e171-3, 2013. [PUBMED Abstract]
  61. Meazza C, Casanova M, Trecate G, et al.: Objective response to hydroxyurea in a patient with heavily pre-treated aggressive fibromatosis. Pediatr Blood Cancer 55 (3): 587-8, 2010. [PUBMED Abstract]
  62. Balamuth NJ, Womer RB: Successful treatment of fibromatosis with hydroxyurea: Analysis of 20 pediatric cases. [Abstract] The Connective Tissue Oncology Society (CTOS) 14th Annual Meeting, 13–15 November 2008, London, United Kingdom A-34852, 2008. Also available online. Last accessed April 18, 2019.
  63. Gounder MM, Mahoney MR, Van Tine BA, et al.: Phase III, randomized, double blind, placebo-controlled trial of sorafenib in desmoid tumors (Alliance A091105). [Abstract] J Clin Oncol 36 (Suppl 18): A-11500, 2018. Also available online. Last accessed April 18, 2019.
  64. Agresta L, Kim H, Turpin BK, et al.: Pazopanib therapy for desmoid tumors in adolescent and young adult patients. Pediatr Blood Cancer 65 (6): e26968, 2018. [PUBMED Abstract]
  65. Shang H, Braggio D, Lee YJ, et al.: Targeting the Notch pathway: A potential therapeutic approach for desmoid tumors. Cancer 121 (22): 4088-96, 2015. [PUBMED Abstract]
  66. Messersmith WA, Shapiro GI, Cleary JM, et al.: A Phase I, dose-finding study in patients with advanced solid malignancies of the oral γ-secretase inhibitor PF-03084014. Clin Cancer Res 21 (1): 60-7, 2015. [PUBMED Abstract]
  67. Meazza C, Bisogno G, Gronchi A, et al.: Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer 116 (1): 233-40, 2010. [PUBMED Abstract]
  68. Hansmann A, Adolph C, Vogel T, et al.: High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer 100 (3): 612-20, 2004. [PUBMED Abstract]
  69. Skapek SX, Anderson JR, Hill DA, et al.: Safety and efficacy of high-dose tamoxifen and sulindac for desmoid tumor in children: results of a Children's Oncology Group (COG) phase II study. Pediatr Blood Cancer 60 (7): 1108-12, 2013. [PUBMED Abstract]
  70. Rutenberg MS, Indelicato DJ, Knapik JA, et al.: External-beam radiotherapy for pediatric and young adult desmoid tumors. Pediatr Blood Cancer 57 (3): 435-42, 2011. [PUBMED Abstract]
  71. Buckley PG, Mantripragada KK, Benetkiewicz M, et al.: A full-coverage, high-resolution human chromosome 22 genomic microarray for clinical and research applications. Hum Mol Genet 11 (25): 3221-9, 2002. [PUBMED Abstract]
  72. Valdivielso-Ramos M, Torrelo A, Campos M, et al.: Pediatric dermatofibrosarcoma protuberans in Madrid, Spain: multi-institutional outcomes. Pediatr Dermatol 31 (6): 676-82, 2014 Nov-Dec. [PUBMED Abstract]
  73. Gooskens SL, Oranje AP, van Adrichem LN, et al.: Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP). Pediatr Blood Cancer 55 (2): 369-73, 2010. [PUBMED Abstract]
  74. Rubio GA, Alvarado A, Gerth DJ, et al.: Incidence and Outcomes of Dermatofibrosarcoma Protuberans in the US Pediatric Population. J Craniofac Surg 28 (1): 182-184, 2017. [PUBMED Abstract]
  75. Meguerditchian AN, Wang J, Lema B, et al.: Wide excision or Mohs micrographic surgery for the treatment of primary dermatofibrosarcoma protuberans. Am J Clin Oncol 33 (3): 300-3, 2010. [PUBMED Abstract]
  76. Dagan R, Morris CG, Zlotecki RA, et al.: Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol 28 (6): 537-9, 2005. [PUBMED Abstract]
  77. Sun LM, Wang CJ, Huang CC, et al.: Dermatofibrosarcoma protuberans: treatment results of 35 cases. Radiother Oncol 57 (2): 175-81, 2000. [PUBMED Abstract]
  78. Price VE, Fletcher JA, Zielenska M, et al.: Imatinib mesylate: an attractive alternative in young children with large, surgically challenging dermatofibrosarcoma protuberans. Pediatr Blood Cancer 44 (5): 511-5, 2005. [PUBMED Abstract]
  79. McArthur GA, Demetri GD, van Oosterom A, et al.: Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol 23 (4): 866-73, 2005. [PUBMED Abstract]
  80. Rutkowski P, Van Glabbeke M, Rankin CJ, et al.: Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol 28 (10): 1772-9, 2010. [PUBMED Abstract]
  81. Miller SJ, Alam M, Andersen JS, et al.: Dermatofibrosarcoma protuberans. J Natl Compr Canc Netw 10 (3): 312-8, 2012. [PUBMED Abstract]
  82. Kovach SJ, Fischer AC, Katzman PJ, et al.: Inflammatory myofibroblastic tumors. J Surg Oncol 94 (5): 385-91, 2006. [PUBMED Abstract]
  83. Brodlie M, Barwick SC, Wood KM, et al.: Inflammatory myofibroblastic tumours of the respiratory tract: paediatric case series with varying clinical presentations. J Laryngol Otol 125 (8): 865-8, 2011. [PUBMED Abstract]
  84. Xiao Y, Zhou S, Ma C, et al.: Radiological and histopathological features of hepatic inflammatory myofibroblastic tumour: analysis of 10 cases. Clin Radiol 68 (11): 1114-20, 2013. [PUBMED Abstract]
  85. Karnak I, Senocak ME, Ciftci AO, et al.: Inflammatory myofibroblastic tumor in children: diagnosis and treatment. J Pediatr Surg 36 (6): 908-12, 2001. [PUBMED Abstract]
  86. Collin M, Charles A, Barker A, et al.: Inflammatory myofibroblastic tumour of the bladder in children: a review. J Pediatr Urol 11 (5): 239-45, 2015. [PUBMED Abstract]
  87. Coffin CM, Hornick JL, Fletcher CD: Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol 31 (4): 509-20, 2007. [PUBMED Abstract]
  88. Lovly CM, Gupta A, Lipson D, et al.: Inflammatory myofibroblastic tumors harbor multiple potentially actionable kinase fusions. Cancer Discov 4 (8): 889-95, 2014. [PUBMED Abstract]
  89. Devaney KO, Lafeir DJ, Triantafyllou A, et al.: Inflammatory myofibroblastic tumors of the head and neck: evaluation of clinicopathologic and prognostic features. Eur Arch Otorhinolaryngol 269 (12): 2461-5, 2012. [PUBMED Abstract]
  90. Mehta B, Mascarenhas L, Zhou S, et al.: Inflammatory myofibroblastic tumors in childhood. Pediatr Hematol Oncol 30 (7): 640-5, 2013. [PUBMED Abstract]
  91. Favini F, Resti AG, Collini P, et al.: Inflammatory myofibroblastic tumor of the conjunctiva: response to chemotherapy with low-dose methotrexate and vinorelbine. Pediatr Blood Cancer 54 (3): 483-5, 2010. [PUBMED Abstract]
  92. Doski JJ, Priebe CJ Jr, Driessnack M, et al.: Corticosteroids in the management of unresected plasma cell granuloma (inflammatory pseudotumor) of the lung. J Pediatr Surg 26 (9): 1064-6, 1991. [PUBMED Abstract]
  93. Diop B, Konate I, Ka S, et al.: Mesenteric myofibroblastic tumor: NSAID therapy after incomplete resection. J Visc Surg 148 (4): e311-4, 2011. [PUBMED Abstract]
  94. Dalton BG, Thomas PG, Sharp NE, et al.: Inflammatory myofibroblastic tumors in children. J Pediatr Surg 51 (4): 541-4, 2016. [PUBMED Abstract]
  95. Butrynski JE, D'Adamo DR, Hornick JL, et al.: Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor. N Engl J Med 363 (18): 1727-33, 2010. [PUBMED Abstract]
  96. Mossé YP, Lim MS, Voss SD, et al.: Safety and activity of crizotinib for paediatric patients with refractory solid tumours or anaplastic large-cell lymphoma: a Children's Oncology Group phase 1 consortium study. Lancet Oncol 14 (6): 472-80, 2013. [PUBMED Abstract]
  97. Gaudichon J, Jeanne-Pasquier C, Deparis M, et al.: Complete and Repeated Response of a Metastatic ALK-rearranged Inflammatory Myofibroblastic Tumor to Crizotinib in a Teenage Girl. J Pediatr Hematol Oncol 38 (4): 308-11, 2016. [PUBMED Abstract]
  98. Mossé YP, Voss SD, Lim MS, et al.: Targeting ALK With Crizotinib in Pediatric Anaplastic Large Cell Lymphoma and Inflammatory Myofibroblastic Tumor: A Children's Oncology Group Study. J Clin Oncol 35 (28): 3215-3221, 2017. [PUBMED Abstract]
  99. Nishio M, Murakami H, Horiike A, et al.: Phase I Study of Ceritinib (LDK378) in Japanese Patients with Advanced, Anaplastic Lymphoma Kinase-Rearranged Non-Small-Cell Lung Cancer or Other Tumors. J Thorac Oncol 10 (7): 1058-66, 2015. [PUBMED Abstract]
  100. Sulkowski JP, Raval MV, Browne M: Margin status and multimodal therapy in infantile fibrosarcoma. Pediatr Surg Int 29 (8): 771-6, 2013. [PUBMED Abstract]
  101. Hirschfeld R, Welch JJG, Harrison DJ, et al.: Two cases of humoral hypercalcemia of malignancy complicating infantile fibrosarcoma. Pediatr Blood Cancer 64 (10): , 2017. [PUBMED Abstract]
  102. Kao YC, Fletcher CDM, Alaggio R, et al.: Recurrent BRAF Gene Fusions in a Subset of Pediatric Spindle Cell Sarcomas: Expanding the Genetic Spectrum of Tumors With Overlapping Features With Infantile Fibrosarcoma. Am J Surg Pathol 42 (1): 28-38, 2018. [PUBMED Abstract]
  103. Wegert J, Vokuhl C, Collord G, et al.: Recurrent intragenic rearrangements of EGFR and BRAF in soft tissue tumors of infants. Nat Commun 9 (1): 2378, 2018. [PUBMED Abstract]
  104. Orbach D, Rey A, Cecchetto G, et al.: Infantile fibrosarcoma: management based on the European experience. J Clin Oncol 28 (2): 318-23, 2010. [PUBMED Abstract]
  105. Orbach D, Brennan B, De Paoli A, et al.: Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience. Eur J Cancer 57: 1-9, 2016. [PUBMED Abstract]
  106. Spunt SL, Million L, Coffin C: The nonrhabdomyosarcoma soft tissue sarcoma. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams and Wilkins, 2015, pp 827-54.
  107. Loh ML, Ahn P, Perez-Atayde AR, et al.: Treatment of infantile fibrosarcoma with chemotherapy and surgery: results from the Dana-Farber Cancer Institute and Children's Hospital, Boston. J Pediatr Hematol Oncol 24 (9): 722-6, 2002. [PUBMED Abstract]
  108. Akyüz C, Küpeli S, Varan A, et al.: Infantile fibrosarcoma: retrospective analysis of eleven patients. Tumori 97 (2): 166-9, 2011 Mar-Apr. [PUBMED Abstract]
  109. Gallego S, Pericas N, Barber I, et al.: Infantile fibrosarcoma of the retroperitoneum: a site of unfavorable prognosis? Pediatr Hematol Oncol 28 (5): 451-3, 2011. [PUBMED Abstract]
  110. Parida L, Fernandez-Pineda I, Uffman JK, et al.: Clinical management of infantile fibrosarcoma: a retrospective single-institution review. Pediatr Surg Int 29 (7): 703-8, 2013. [PUBMED Abstract]
  111. Mody RJ, Wu YM, Lonigro RJ, et al.: Integrative Clinical Sequencing in the Management of Refractory or Relapsed Cancer in Youth. JAMA 314 (9): 913-25, 2015. [PUBMED Abstract]
  112. Wong V, Pavlick D, Brennan T, et al.: Evaluation of a Congenital Infantile Fibrosarcoma by Comprehensive Genomic Profiling Reveals an LMNA-NTRK1 Gene Fusion Responsive to Crizotinib. J Natl Cancer Inst 108 (1): , 2016. [PUBMED Abstract]
  113. Kummar S, Lassen UN: TRK Inhibition: A New Tumor-Agnostic Treatment Strategy. Target Oncol 13 (5): 545-556, 2018. [PUBMED Abstract]
  114. Drilon A, Nagasubramanian R, Blake JF, et al.: A Next-Generation TRK Kinase Inhibitor Overcomes Acquired Resistance to Prior TRK Kinase Inhibition in Patients with TRK Fusion-Positive Solid Tumors. Cancer Discov 7 (9): 963-972, 2017. [PUBMED Abstract]
  115. Yanagisawa R, Noguchi M, Fujita K, et al.: Preoperative Treatment With Pazopanib in a Case of Chemotherapy-Resistant Infantile Fibrosarcoma. Pediatr Blood Cancer 63 (2): 348-51, 2016. [PUBMED Abstract]
  116. Madden NP, Spicer RD, Allibone EB, et al.: Spontaneous regression of neonatal fibrosarcoma. Br J Cancer Suppl 18: S72-5, 1992. [PUBMED Abstract]
  117. Evans HL: Low-grade fibromyxoid sarcoma: a clinicopathologic study of 33 cases with long-term follow-up. Am J Surg Pathol 35 (10): 1450-62, 2011. [PUBMED Abstract]
  118. Guillou L, Benhattar J, Gengler C, et al.: Translocation-positive low-grade fibromyxoid sarcoma: clinicopathologic and molecular analysis of a series expanding the morphologic spectrum and suggesting potential relationship to sclerosing epithelioid fibrosarcoma: a study from the French Sarcoma Group. Am J Surg Pathol 31 (9): 1387-402, 2007. [PUBMED Abstract]
  119. O'Sullivan MJ, Sirgi KE, Dehner LP: Low-grade fibrosarcoma (hyalinizing spindle cell tumor with giant rosettes) with pulmonary metastases at presentation: case report and review of the literature. Int J Surg Pathol 10 (3): 211-6, 2002. [PUBMED Abstract]
  120. Folpe AL, Lane KL, Paull G, et al.: Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas. Am J Surg Pathol 24 (10): 1353-60, 2000. [PUBMED Abstract]
  121. Sargar K, Kao SC, Spunt SL, et al.: MRI and CT of Low-Grade Fibromyxoid Sarcoma in Children: A Report From Children's Oncology Group Study ARST0332. AJR Am J Roentgenol 205 (2): 414-20, 2015. [PUBMED Abstract]
  122. Maretty-Nielsen K, Baerentzen S, Keller J, et al.: Low-Grade Fibromyxoid Sarcoma: Incidence, Treatment Strategy of Metastases, and Clinical Significance of the FUS Gene. Sarcoma 2013: 256280, 2013. [PUBMED Abstract]
  123. Prieto-Granada C, Zhang L, Chen HW, et al.: A genetic dichotomy between pure sclerosing epithelioid fibrosarcoma (SEF) and hybrid SEF/low-grade fibromyxoid sarcoma: a pathologic and molecular study of 18 cases. Genes Chromosomes Cancer 54 (1): 28-38, 2015. [PUBMED Abstract]
  124. Chew W, Benson C, Thway K, et al.: Clinical Characteristics and efficacy of chemotherapy in sclerosing epithelioid fibrosarcoma. Med Oncol 35 (11): 138, 2018. [PUBMED Abstract]
  125. Arbajian E, Puls F, Antonescu CR, et al.: In-depth Genetic Analysis of Sclerosing Epithelioid Fibrosarcoma Reveals Recurrent Genomic Alterations and Potential Treatment Targets. Clin Cancer Res 23 (23): 7426-7434, 2017. [PUBMED Abstract]
  126. Pollock BH, Jenson HB, Leach CT, et al.: Risk factors for pediatric human immunodeficiency virus-related malignancy. JAMA 289 (18): 2393-9, 2003. [PUBMED Abstract]
  127. Kleinerman RA, Tucker MA, Abramson DH, et al.: Risk of soft tissue sarcomas by individual subtype in survivors of hereditary retinoblastoma. J Natl Cancer Inst 99 (1): 24-31, 2007. [PUBMED Abstract]
  128. Samuels BL, Chawla S, Patel S, et al.: Clinical outcomes and safety with trabectedin therapy in patients with advanced soft tissue sarcomas following failure of prior chemotherapy: results of a worldwide expanded access program study. Ann Oncol 24 (6): 1703-9, 2013. [PUBMED Abstract]
  129. Enzinger FM, Zhang RY: Plexiform fibrohistiocytic tumor presenting in children and young adults. An analysis of 65 cases. Am J Surg Pathol 12 (11): 818-26, 1988. [PUBMED Abstract]
  130. Black J, Coffin CM, Dehner LP: Fibrohistiocytic tumors and related neoplasms in children and adolescents. Pediatr Dev Pathol 15 (1 Suppl): 181-210, 2012. [PUBMED Abstract]
  131. Moosavi C, Jha P, Fanburg-Smith JC: An update on plexiform fibrohistiocytic tumor and addition of 66 new cases from the Armed Forces Institute of Pathology, in honor of Franz M. Enzinger, MD. Ann Diagn Pathol 11 (5): 313-9, 2007. [PUBMED Abstract]
  132. Billings SD, Folpe AL: Cutaneous and subcutaneous fibrohistiocytic tumors of intermediate malignancy: an update. Am J Dermatopathol 26 (2): 141-55, 2004. [PUBMED Abstract]
  133. Remstein ED, Arndt CA, Nascimento AG: Plexiform fibrohistiocytic tumor: clinicopathologic analysis of 22 cases. Am J Surg Pathol 23 (6): 662-70, 1999. [PUBMED Abstract]
  134. Salomao DR, Nascimento AG: Plexiform fibrohistiocytic tumor with systemic metastases: a case report. Am J Surg Pathol 21 (4): 469-76, 1997. [PUBMED Abstract]
  135. Redlich GC, Montgomery KD, Allgood GA, et al.: Plexiform fibrohistiocytic tumor with a clonal cytogenetic anomaly. Cancer Genet Cytogenet 108 (2): 141-3, 1999. [PUBMED Abstract]
  136. Luzar B, Calonje E: Cutaneous fibrohistiocytic tumours - an update. Histopathology 56 (1): 148-65, 2010. [PUBMED Abstract]
  137. Carli M, Ferrari A, Mattke A, et al.: Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol 23 (33): 8422-30, 2005. [PUBMED Abstract]
  138. Malbari F, Spira M, B Knight P, et al.: Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis: Impact of Family History. J Pediatr Hematol Oncol 40 (6): e359-e363, 2018. [PUBMED Abstract]
  139. Zhang M, Wang Y, Jones S, et al.: Somatic mutations of SUZ12 in malignant peripheral nerve sheath tumors. Nat Genet 46 (11): 1170-2, 2014. [PUBMED Abstract]
  140. Röhrich M, Koelsche C, Schrimpf D, et al.: Methylation-based classification of benign and malignant peripheral nerve sheath tumors. Acta Neuropathol 131 (6): 877-87, 2016. [PUBMED Abstract]
  141. Kaplan HG, Rostad S, Ross JS, et al.: Genomic Profiling in Patients With Malignant Peripheral Nerve Sheath Tumors Reveals Multiple Pathways With Targetable Mutations. J Natl Compr Canc Netw 16 (8): 967-974, 2018. [PUBMED Abstract]
  142. Hagel C, Zils U, Peiper M, et al.: Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors. J Neurooncol 82 (2): 187-92, 2007. [PUBMED Abstract]
  143. Zou C, Smith KD, Liu J, et al.: Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg 249 (6): 1014-22, 2009. [PUBMED Abstract]
  144. Okada K, Hasegawa T, Tajino T, et al.: Clinical relevance of pathological grades of malignant peripheral nerve sheath tumor: a multi-institution TMTS study of 56 cases in Northern Japan. Ann Surg Oncol 14 (2): 597-604, 2007. [PUBMED Abstract]
  145. Amirian ES, Goodman JC, New P, et al.: Pediatric and adult malignant peripheral nerve sheath tumors: an analysis of data from the surveillance, epidemiology, and end results program. J Neurooncol 116 (3): 609-16, 2014. [PUBMED Abstract]
  146. Valentin T, Le Cesne A, Ray-Coquard I, et al.: Management and prognosis of malignant peripheral nerve sheath tumors: The experience of the French Sarcoma Group (GSF-GETO). Eur J Cancer 56: 77-84, 2016. [PUBMED Abstract]
  147. Høland M, Kolberg M, Danielsen SA, et al.: Inferior survival for patients with malignant peripheral nerve sheath tumors defined by aberrant TP53. Mod Pathol : , 2018. [PUBMED Abstract]
  148. Bergamaschi L, Bisogno G, Manzitti C, et al.: Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors. Pediatr Blood Cancer 65 (2): , 2018. [PUBMED Abstract]
  149. Ferrari A, Bisogno G, Macaluso A, et al.: Soft-tissue sarcomas in children and adolescents with neurofibromatosis type 1. Cancer 109 (7): 1406-12, 2007. [PUBMED Abstract]
  150. Okur FV, Oguz A, Karadeniz C, et al.: Malignant triton tumor of the pelvis in a 2-year-old boy. J Pediatr Hematol Oncol 28 (3): 173-6, 2006. [PUBMED Abstract]
  151. Griffin BB, Chou PM, George D, et al.: Malignant Ectomesenchymoma: Series Analysis of a Histologically and Genetically Heterogeneous Tumor. Int J Surg Pathol 26 (3): 200-212, 2018. [PUBMED Abstract]
  152. Huang SC, Alaggio R, Sung YS, et al.: Frequent HRAS Mutations in Malignant Ectomesenchymoma: Overlapping Genetic Abnormalities With Embryonal Rhabdomyosarcoma. Am J Surg Pathol 40 (7): 876-85, 2016. [PUBMED Abstract]
  153. Dantonello TM, Leuschner I, Vokuhl C, et al.: Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS). Pediatr Blood Cancer 60 (2): 224-9, 2013. [PUBMED Abstract]
  154. Rodriguez-Galindo C, Ramsey K, Jenkins JJ, et al.: Hemangiopericytoma in children and infants. Cancer 88 (1): 198-204, 2000. [PUBMED Abstract]
  155. Ferrari A, Casanova M, Bisogno G, et al.: Hemangiopericytoma in pediatric ages: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Cancer 92 (10): 2692-8, 2001. [PUBMED Abstract]
  156. Bien E, Stachowicz-Stencel T, Godzinski J, et al.: Retrospective multi-institutional study on hemangiopericytoma in Polish children. Pediatr Int 51 (1): 19-24, 2009. [PUBMED Abstract]
  157. Weiss SW, Goldblum JR: Enzinger and Weiss's Soft Tissue Tumors. 5th ed. St. Louis, Mo: Mosby, 2008.
  158. Fernandez-Pineda I, Parida L, Jenkins JJ, et al.: Childhood hemangiopericytoma: review of St Jude Children's Research Hospital. J Pediatr Hematol Oncol 33 (5): 356-9, 2011. [PUBMED Abstract]
  159. Haller F, Knopf J, Ackermann A, et al.: Paediatric and adult soft tissue sarcomas with NTRK1 gene fusions: a subset of spindle cell sarcomas unified by a prominent myopericytic/haemangiopericytic pattern. J Pathol 238 (5): 700-10, 2016. [PUBMED Abstract]
  160. Doebele RC, Davis LE, Vaishnavi A, et al.: An Oncogenic NTRK Fusion in a Patient with Soft-Tissue Sarcoma with Response to the Tropomyosin-Related Kinase Inhibitor LOXO-101. Cancer Discov 5 (10): 1049-57, 2015. [PUBMED Abstract]
  161. Wiswell TE, Davis J, Cunningham BE, et al.: Infantile myofibromatosis: the most common fibrous tumor of infancy. J Pediatr Surg 23 (4): 315-8, 1988. [PUBMED Abstract]
  162. Chung EB, Enzinger FM: Infantile myofibromatosis. Cancer 48 (8): 1807-18, 1981. [PUBMED Abstract]
  163. Modi N: Congenital generalised fibromatosis. Arch Dis Child 57 (11): 881-2, 1982. [PUBMED Abstract]
  164. Levine E, Fréneaux P, Schleiermacher G, et al.: Risk-adapted therapy for infantile myofibromatosis in children. Pediatr Blood Cancer 59 (1): 115-20, 2012. [PUBMED Abstract]
  165. Larralde M, Hoffner MV, Boggio P, et al.: Infantile myofibromatosis: report of nine patients. Pediatr Dermatol 27 (1): 29-33, 2010 Jan-Feb. [PUBMED Abstract]
  166. Cheung YH, Gayden T, Campeau PM, et al.: A recurrent PDGFRB mutation causes familial infantile myofibromatosis. Am J Hum Genet 92 (6): 996-1000, 2013. [PUBMED Abstract]
  167. Agaimy A, Bieg M, Michal M, et al.: Recurrent Somatic PDGFRB Mutations in Sporadic Infantile/Solitary Adult Myofibromas But Not in Angioleiomyomas and Myopericytomas. Am J Surg Pathol 41 (2): 195-203, 2017. [PUBMED Abstract]
  168. Gopal M, Chahal G, Al-Rifai Z, et al.: Infantile myofibromatosis. Pediatr Surg Int 24 (3): 287-91, 2008. [PUBMED Abstract]
  169. Weaver MS, Navid F, Huppmann A, et al.: Vincristine and Dactinomycin in Infantile Myofibromatosis With a Review of Treatment Options. J Pediatr Hematol Oncol 37 (3): 237-41, 2015. [PUBMED Abstract]
  170. Sultan I, Rodriguez-Galindo C, Saab R, et al.: Comparing children and adults with synovial sarcoma in the Surveillance, Epidemiology, and End Results program, 1983 to 2005: an analysis of 1268 patients. Cancer 115 (15): 3537-47, 2009. [PUBMED Abstract]
  171. Wang JG, Li NN: Primary cardiac synovial sarcoma. Ann Thorac Surg 95 (6): 2202-9, 2013. [PUBMED Abstract]
  172. Pappo AS, Fontanesi J, Luo X, et al.: Synovial sarcoma in children and adolescents: the St Jude Children's Research Hospital experience. J Clin Oncol 12 (11): 2360-6, 1994. [PUBMED Abstract]
  173. Ferrari A, De Salvo GL, Oberlin O, et al.: Synovial sarcoma in children and adolescents: a critical reappraisal of staging investigations in relation to the rate of metastatic involvement at diagnosis. Eur J Cancer 48 (9): 1370-5, 2012. [PUBMED Abstract]
  174. van de Rijn M, Barr FG, Collins MH, et al.: Absence of SYT-SSX fusion products in soft tissue tumors other than synovial sarcoma. Am J Clin Pathol 112 (1): 43-9, 1999. [PUBMED Abstract]
  175. Krsková L, Sumerauer D, Stejskalová E, et al.: A novel variant of SYT-SSX1 fusion gene in a case of spindle cell synovial sarcoma. Diagn Mol Pathol 16 (3): 179-83, 2007. [PUBMED Abstract]
  176. Su L, Sampaio AV, Jones KB, et al.: Deconstruction of the SS18-SSX fusion oncoprotein complex: insights into disease etiology and therapeutics. Cancer Cell 21 (3): 333-47, 2012. [PUBMED Abstract]
  177. Arnold MA, Arnold CA, Li G, et al.: A unique pattern of INI1 immunohistochemistry distinguishes synovial sarcoma from its histologic mimics. Hum Pathol 44 (5): 881-7, 2013. [PUBMED Abstract]
  178. Vlenterie M, Ho VK, Kaal SE, et al.: Age as an independent prognostic factor for survival of localised synovial sarcoma patients. Br J Cancer 113 (11): 1602-6, 2015. [PUBMED Abstract]
  179. Okcu MF, Munsell M, Treuner J, et al.: Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol 21 (8): 1602-11, 2003. [PUBMED Abstract]
  180. Brecht IB, Ferrari A, Int-Veen C, et al.: Grossly-resected synovial sarcoma treated by the German and Italian Pediatric Soft Tissue Sarcoma Cooperative Groups: discussion on the role of adjuvant therapies. Pediatr Blood Cancer 46 (1): 11-7, 2006. [PUBMED Abstract]
  181. Stanelle EJ, Christison-Lagay ER, Healey JH, et al.: Pediatric and adolescent synovial sarcoma: multivariate analysis of prognostic factors and survival outcomes. Ann Surg Oncol 20 (1): 73-9, 2013. [PUBMED Abstract]
  182. Trassard M, Le Doussal V, Hacène K, et al.: Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients. J Clin Oncol 19 (2): 525-34, 2001. [PUBMED Abstract]
  183. Guillou L, Benhattar J, Bonichon F, et al.: Histologic grade, but not SYT-SSX fusion type, is an important prognostic factor in patients with synovial sarcoma: a multicenter, retrospective analysis. J Clin Oncol 22 (20): 4040-50, 2004. [PUBMED Abstract]
  184. Ferrari A, Gronchi A, Casanova M, et al.: Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution. Cancer 101 (3): 627-34, 2004. [PUBMED Abstract]
  185. Lagarde P, Przybyl J, Brulard C, et al.: Chromosome instability accounts for reverse metastatic outcomes of pediatric and adult synovial sarcomas. J Clin Oncol 31 (5): 608-15, 2013. [PUBMED Abstract]
  186. Stegmaier S, Leuschner I, Poremba C, et al.: The prognostic impact of SYT-SSX fusion type and histological grade in pediatric patients with synovial sarcoma treated according to the CWS (Cooperative Weichteilsarkom Studie) trials. Pediatr Blood Cancer 64 (1): 89-95, 2017. [PUBMED Abstract]
  187. Scheer M, Dantonello T, Hallmen E, et al.: Primary Metastatic Synovial Sarcoma: Experience of the CWS Study Group. Pediatr Blood Cancer 63 (7): 1198-206, 2016. [PUBMED Abstract]
  188. Ferrari A, Chi YY, De Salvo GL, et al.: Surgery alone is sufficient therapy for children and adolescents with low-risk synovial sarcoma: A joint analysis from the European paediatric soft tissue sarcoma Study Group and the Children's Oncology Group. Eur J Cancer 78: 1-6, 2017. [PUBMED Abstract]
  189. McGrory JE, Pritchard DJ, Arndt CA, et al.: Nonrhabdomyosarcoma soft tissue sarcomas in children. The Mayo Clinic experience. Clin Orthop (374): 247-58, 2000. [PUBMED Abstract]
  190. Van Glabbeke M, van Oosterom AT, Oosterhuis JW, et al.: Prognostic factors for the outcome of chemotherapy in advanced soft tissue sarcoma: an analysis of 2,185 patients treated with anthracycline-containing first-line regimens--a European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group Study. J Clin Oncol 17 (1): 150-7, 1999. [PUBMED Abstract]
  191. Koscielniak E, Harms D, Henze G, et al.: Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86. J Clin Oncol 17 (12): 3706-19, 1999. [PUBMED Abstract]
  192. Pappo AS, Devidas M, Jenkins J, et al.: Phase II trial of neoadjuvant vincristine, ifosfamide, and doxorubicin with granulocyte colony-stimulating factor support in children and adolescents with advanced-stage nonrhabdomyosarcomatous soft tissue sarcomas: a Pediatric Oncology Group Study. J Clin Oncol 23 (18): 4031-8, 2005. [PUBMED Abstract]
  193. Pappo AS, Rao BN, Jenkins JJ, et al.: Metastatic nonrhabdomyosarcomatous soft-tissue sarcomas in children and adolescents: the St. Jude Children's Research Hospital experience. Med Pediatr Oncol 33 (2): 76-82, 1999. [PUBMED Abstract]
  194. Brennan B, Stevens M, Kelsey A, et al.: Synovial sarcoma in childhood and adolescence: a retrospective series of 77 patients registered by the Children's Cancer and Leukaemia Group between 1991 and 2006. Pediatr Blood Cancer 55 (1): 85-90, 2010. [PUBMED Abstract]
  195. Ferrari A, Miceli R, Rey A, et al.: Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: results of a pooled analysis from United States and European groups. Eur J Cancer 47 (5): 724-31, 2011. [PUBMED Abstract]
  196. Raney RB: Synovial sarcoma in young people: background, prognostic factors, and therapeutic questions. J Pediatr Hematol Oncol 27 (4): 207-11, 2005. [PUBMED Abstract]
  197. Orbach D, Mc Dowell H, Rey A, et al.: Sparing strategy does not compromise prognosis in pediatric localized synovial sarcoma: experience of the International Society of Pediatric Oncology, Malignant Mesenchymal Tumors (SIOP-MMT) Working Group. Pediatr Blood Cancer 57 (7): 1130-6, 2011. [PUBMED Abstract]
  198. Ladenstein R, Treuner J, Koscielniak E, et al.: Synovial sarcoma of childhood and adolescence. Report of the German CWS-81 study. Cancer 71 (11): 3647-55, 1993. [PUBMED Abstract]
  199. Venkatramani R, Anderson JR, Million L, et al.: Risk-based treatment for synovial sarcoma in patients under 30 years of age: Children’s Oncology Group study ARST0332. [Abstract] J Clin Oncol 33 (15 Suppl): A-10012, 2015. Also available online. Last accessed April 18, 2019.
  200. Ferrari A, De Salvo GL, Brennan B, et al.: Synovial sarcoma in children and adolescents: the European Pediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005). Ann Oncol 26 (3): 567-72, 2015. [PUBMED Abstract]
  201. Ferrari A, De Salvo GL, Dall'Igna P, et al.: Salvage rates and prognostic factors after relapse in children and adolescents with initially localised synovial sarcoma. Eur J Cancer 48 (18): 3448-55, 2012. [PUBMED Abstract]
  202. Scheer M, Dantonello T, Hallmen E, et al.: Synovial Sarcoma Recurrence in Children and Young Adults. Ann Surg Oncol 23 (Suppl 5): 618-626, 2016. [PUBMED Abstract]
  203. Chbani L, Guillou L, Terrier P, et al.: Epithelioid sarcoma: a clinicopathologic and immunohistochemical analysis of 106 cases from the French sarcoma group. Am J Clin Pathol 131 (2): 222-7, 2009. [PUBMED Abstract]
  204. Hornick JL, Dal Cin P, Fletcher CD: Loss of INI1 expression is characteristic of both conventional and proximal-type epithelioid sarcoma. Am J Surg Pathol 33 (4): 542-50, 2009. [PUBMED Abstract]
  205. Knutson SK, Warholic NM, Wigle TJ, et al.: Durable tumor regression in genetically altered malignant rhabdoid tumors by inhibition of methyltransferase EZH2. Proc Natl Acad Sci U S A 110 (19): 7922-7, 2013. [PUBMED Abstract]
  206. Guzzetta AA, Montgomery EA, Lyu H, et al.: Epithelioid sarcoma: one institution's experience with a rare sarcoma. J Surg Res 177 (1): 116-22, 2012. [PUBMED Abstract]
  207. Hawkins DS, Spunt SL, Skapek SX, et al.: Children's Oncology Group's 2013 blueprint for research: Soft tissue sarcomas. Pediatr Blood Cancer 60 (6): 1001-8, 2013. [PUBMED Abstract]
  208. Casanova M, Ferrari A, Collini P, et al.: Epithelioid sarcoma in children and adolescents: a report from the Italian Soft Tissue Sarcoma Committee. Cancer 106 (3): 708-17, 2006. [PUBMED Abstract]
  209. Italiano A, Soria JC, Toulmonde M, et al.: Tazemetostat, an EZH2 inhibitor, in relapsed or refractory B-cell non-Hodgkin lymphoma and advanced solid tumours: a first-in-human, open-label, phase 1 study. Lancet Oncol 19 (5): 649-659, 2018. [PUBMED Abstract]
  210. Orbach D, Brennan B, Casanova M, et al.: Paediatric and adolescent alveolar soft part sarcoma: A joint series from European cooperative groups. Pediatr Blood Cancer 60 (11): 1826-32, 2013. [PUBMED Abstract]
  211. Ferrari A, Sultan I, Huang TT, et al.: Soft tissue sarcoma across the age spectrum: a population-based study from the Surveillance Epidemiology and End Results database. Pediatr Blood Cancer 57 (6): 943-9, 2011. [PUBMED Abstract]
  212. Wang HW, Qin XJ, Yang WJ, et al.: Alveolar soft part sarcoma of the oral and maxillofacial region: clinical analysis in a series of 18 patients. Oral Surg Oral Med Oral Pathol Oral Radiol 119 (4): 396-401, 2015. [PUBMED Abstract]
  213. Kayton ML, Meyers P, Wexler LH, et al.: Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults. J Pediatr Surg 41 (1): 187-93, 2006. [PUBMED Abstract]
  214. Sparber-Sauer M, Seitz G, von Kalle T, et al.: Alveolar soft-part sarcoma: Primary metastatic disease and metastatic relapse occurring during long-term follow-up: Treatment results of four Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry. Pediatr Blood Cancer : e27405, 2018. [PUBMED Abstract]
  215. Flores RJ, Harrison DJ, Federman NC, et al.: Alveolar soft part sarcoma in children and young adults: A report of 69 cases. Pediatr Blood Cancer 65 (5): e26953, 2018. [PUBMED Abstract]
  216. Ladanyi M, Lui MY, Antonescu CR, et al.: The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Oncogene 20 (1): 48-57, 2001. [PUBMED Abstract]
  217. Williams A, Bartle G, Sumathi VP, et al.: Detection of ASPL/TFE3 fusion transcripts and the TFE3 antigen in formalin-fixed, paraffin-embedded tissue in a series of 18 cases of alveolar soft part sarcoma: useful diagnostic tools in cases with unusual histological features. Virchows Arch 458 (3): 291-300, 2011. [PUBMED Abstract]
  218. Lieberman PH, Brennan MF, Kimmel M, et al.: Alveolar soft-part sarcoma. A clinico-pathologic study of half a century. Cancer 63 (1): 1-13, 1989. [PUBMED Abstract]
  219. Casanova M, Ferrari A, Bisogno G, et al.: Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group. Ann Oncol 11 (11): 1445-9, 2000. [PUBMED Abstract]
  220. Pennacchioli E, Fiore M, Collini P, et al.: Alveolar soft part sarcoma: clinical presentation, treatment, and outcome in a series of 33 patients at a single institution. Ann Surg Oncol 17 (12): 3229-33, 2010. [PUBMED Abstract]
  221. Roozendaal KJ, de Valk B, ten Velden JJ, et al.: Alveolar soft-part sarcoma responding to interferon alpha-2b. Br J Cancer 89 (2): 243-5, 2003. [PUBMED Abstract]
  222. Conde N, Cruz O, Albert A, et al.: Antiangiogenic treatment as a pre-operative management of alveolar soft-part sarcoma. Pediatr Blood Cancer 57 (6): 1071-3, 2011. [PUBMED Abstract]
  223. Stacchiotti S, Negri T, Zaffaroni N, et al.: Sunitinib in advanced alveolar soft part sarcoma: evidence of a direct antitumor effect. Ann Oncol 22 (7): 1682-90, 2011. [PUBMED Abstract]
  224. Jagodzińska-Mucha P, Świtaj T, Kozak K, et al.: Long-term results of therapy with sunitinib in metastatic alveolar soft part sarcoma. Tumori 103 (3): 231-235, 2017. [PUBMED Abstract]
  225. Kummar S, Allen D, Monks A, et al.: Cediranib for metastatic alveolar soft part sarcoma. J Clin Oncol 31 (18): 2296-302, 2013. [PUBMED Abstract]
  226. Kim M, Kim TM, Keam B, et al.: A Phase II Trial of Pazopanib in Patients with Metastatic Alveolar Soft Part Sarcoma. Oncologist : , 2018. [PUBMED Abstract]
  227. Stacchiotti S, Mir O, Le Cesne A, et al.: Activity of Pazopanib and Trabectedin in Advanced Alveolar Soft Part Sarcoma. Oncologist 23 (1): 62-70, 2018. [PUBMED Abstract]
  228. Coindre JM, Hostein I, Terrier P, et al.: Diagnosis of clear cell sarcoma by real-time reverse transcriptase-polymerase chain reaction analysis of paraffin embedded tissues: clinicopathologic and molecular analysis of 44 patients from the French sarcoma group. Cancer 107 (5): 1055-64, 2006. [PUBMED Abstract]
  229. Meis-Kindblom JM: Clear cell sarcoma of tendons and aponeuroses: a historical perspective and tribute to the man behind the entity. Adv Anat Pathol 13 (6): 286-92, 2006. [PUBMED Abstract]
  230. Dim DC, Cooley LD, Miranda RN: Clear cell sarcoma of tendons and aponeuroses: a review. Arch Pathol Lab Med 131 (1): 152-6, 2007. [PUBMED Abstract]
  231. Blazer DG 3rd, Lazar AJ, Xing Y, et al.: Clinical outcomes of molecularly confirmed clear cell sarcoma from a single institution and in comparison with data from the Surveillance, Epidemiology, and End Results registry. Cancer 115 (13): 2971-9, 2009. [PUBMED Abstract]
  232. Fujimura Y, Siddique H, Lee L, et al.: EWS-ATF-1 chimeric protein in soft tissue clear cell sarcoma associates with CREB-binding protein and interferes with p53-mediated trans-activation function. Oncogene 20 (46): 6653-9, 2001. [PUBMED Abstract]
  233. Hisaoka M, Ishida T, Kuo TT, et al.: Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases. Am J Surg Pathol 32 (3): 452-60, 2008. [PUBMED Abstract]
  234. Ferrari A, Casanova M, Bisogno G, et al.: Clear cell sarcoma of tendons and aponeuroses in pediatric patients: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Cancer 94 (12): 3269-76, 2002. [PUBMED Abstract]
  235. Karita M, Tsuchiya H, Yamamoto N, et al.: Caffeine-potentiated chemotherapy for clear cell sarcoma: a report of five cases. Int J Clin Oncol 18 (1): 33-7, 2013. [PUBMED Abstract]
  236. Schöffski P, Wozniak A, Stacchiotti S, et al.: Activity and safety of crizotinib in patients with advanced clear-cell sarcoma with MET alterations: European Organization for Research and Treatment of Cancer phase II trial 90101 'CREATE'. Ann Oncol 28 (12): 3000-3008, 2017. [PUBMED Abstract]
  237. Tsuneyoshi M, Enjoji M, Iwasaki H, et al.: Extraskeletal myxoid chondrosarcoma--a clinicopathologic and electron microscopic study. Acta Pathol Jpn 31 (3): 439-47, 1981. [PUBMED Abstract]
  238. Hachitanda Y, Tsuneyoshi M, Daimaru Y, et al.: Extraskeletal myxoid chondrosarcoma in young children. Cancer 61 (12): 2521-6, 1988. [PUBMED Abstract]
  239. Hisaoka M, Ishida T, Imamura T, et al.: TFG is a novel fusion partner of NOR1 in extraskeletal myxoid chondrosarcoma. Genes Chromosomes Cancer 40 (4): 325-8, 2004. [PUBMED Abstract]
  240. Enzinger FM, Shiraki M: Extraskeletal myxoid chondrosarcoma. An analysis of 34 cases. Hum Pathol 3 (3): 421-35, 1972. [PUBMED Abstract]
  241. McGrory JE, Rock MG, Nascimento AG, et al.: Extraskeletal myxoid chondrosarcoma. Clin Orthop Relat Res (382): 185-90, 2001. [PUBMED Abstract]
  242. Drilon AD, Popat S, Bhuchar G, et al.: Extraskeletal myxoid chondrosarcoma: a retrospective review from 2 referral centers emphasizing long-term outcomes with surgery and chemotherapy. Cancer 113 (12): 3364-71, 2008. [PUBMED Abstract]
  243. Stacchiotti S, Pantaleo MA, Astolfi A, et al.: Activity of sunitinib in extraskeletal myxoid chondrosarcoma. Eur J Cancer 50 (9): 1657-64, 2014. [PUBMED Abstract]
  244. Leuschner I, Radig K, Harms D: Desmoplastic small round cell tumor. Semin Diagn Pathol 13 (3): 204-12, 1996. [PUBMED Abstract]
  245. Kushner BH, LaQuaglia MP, Wollner N, et al.: Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol 14 (5): 1526-31, 1996. [PUBMED Abstract]
  246. Saab R, Khoury JD, Krasin M, et al.: Desmoplastic small round cell tumor in childhood: the St. Jude Children's Research Hospital experience. Pediatr Blood Cancer 49 (3): 274-9, 2007. [PUBMED Abstract]
  247. Wang LL, Perlman EJ, Vujanic GM, et al.: Desmoplastic small round cell tumor of the kidney in childhood. Am J Surg Pathol 31 (4): 576-84, 2007. [PUBMED Abstract]
  248. Hayes-Jordan A, LaQuaglia MP, Modak S: Management of desmoplastic small round cell tumor. Semin Pediatr Surg 25 (5): 299-304, 2016. [PUBMED Abstract]
  249. Arora VC, Price AP, Fleming S, et al.: Characteristic imaging features of desmoplastic small round cell tumour. Pediatr Radiol 43 (1): 93-102, 2013. [PUBMED Abstract]
  250. Gerald WL, Ladanyi M, de Alava E, et al.: Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants. J Clin Oncol 16 (9): 3028-36, 1998. [PUBMED Abstract]
  251. Lal DR, Su WT, Wolden SL, et al.: Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg 40 (1): 251-5, 2005. [PUBMED Abstract]
  252. Philippe-Chomette P, Kabbara N, Andre N, et al.: Desmoplastic small round cell tumors with EWS-WT1 fusion transcript in children and young adults. Pediatr Blood Cancer 58 (6): 891-7, 2012. [PUBMED Abstract]
  253. Sedig L, Geiger J, Mody R, et al.: Paratesticular desmoplastic small round cell tumors: A case report and review of the literature. Pediatr Blood Cancer 64 (12): , 2017. [PUBMED Abstract]
  254. Subbiah V, Lamhamedi-Cherradi SE, Cuglievan B, et al.: Multimodality Treatment of Desmoplastic Small Round Cell Tumor: Chemotherapy and Complete Cytoreductive Surgery Improve Patient Survival. Clin Cancer Res 24 (19): 4865-4873, 2018. [PUBMED Abstract]
  255. Schwarz RE, Gerald WL, Kushner BH, et al.: Desmoplastic small round cell tumors: prognostic indicators and results of surgical management. Ann Surg Oncol 5 (5): 416-22, 1998 Jul-Aug. [PUBMED Abstract]
  256. Goodman KA, Wolden SL, La Quaglia MP, et al.: Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor. Int J Radiat Oncol Biol Phys 54 (1): 170-6, 2002. [PUBMED Abstract]
  257. Osborne EM, Briere TM, Hayes-Jordan A, et al.: Survival and toxicity following sequential multimodality treatment including whole abdominopelvic radiotherapy for patients with desmoplastic small round cell tumor. Radiother Oncol 119 (1): 40-4, 2016. [PUBMED Abstract]
  258. Atallah V, Honore C, Orbach D, et al.: Role of Adjuvant Radiation Therapy After Surgery for Abdominal Desmoplastic Small Round Cell Tumors. Int J Radiat Oncol Biol Phys 95 (4): 1244-53, 2016. [PUBMED Abstract]
  259. Cook RJ, Wang Z, Arora M, et al.: Clinical outcomes of patients with desmoplastic small round cell tumor of the peritoneum undergoing autologous HCT: a CIBMTR retrospective analysis. Bone Marrow Transplant 47 (11): 1455-8, 2012. [PUBMED Abstract]
  260. Tarek N, Hayes-Jordan A, Salvador L, et al.: Recurrent desmoplastic small round cell tumor responding to an mTOR inhibitor containing regimen. Pediatr Blood Cancer 65 (1): , 2018. [PUBMED Abstract]
  261. Kodet R, Newton WA Jr, Sachs N, et al.: Rhabdoid tumors of soft tissues: a clinicopathologic study of 26 cases enrolled on the Intergroup Rhabdomyosarcoma Study. Hum Pathol 22 (7): 674-84, 1991. [PUBMED Abstract]
  262. Biegel JA, Zhou JY, Rorke LB, et al.: Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res 59 (1): 74-9, 1999. [PUBMED Abstract]
  263. Eaton KW, Tooke LS, Wainwright LM, et al.: Spectrum of SMARCB1/INI1 mutations in familial and sporadic rhabdoid tumors. Pediatr Blood Cancer 56 (1): 7-15, 2011. [PUBMED Abstract]
  264. Lee RS, Stewart C, Carter SL, et al.: A remarkably simple genome underlies highly malignant pediatric rhabdoid cancers. J Clin Invest 122 (8): 2983-8, 2012. [PUBMED Abstract]
  265. Sultan I, Qaddoumi I, Rodríguez-Galindo C, et al.: Age, stage, and radiotherapy, but not primary tumor site, affects the outcome of patients with malignant rhabdoid tumors. Pediatr Blood Cancer 54 (1): 35-40, 2010. [PUBMED Abstract]
  266. Puri DR, Meyers PA, Kraus DH, et al.: Radiotherapy in the multimodal treatment of extrarenal extracranial malignant rhabdoid tumors. Pediatr Blood Cancer 50 (1): 167-9, 2008. [PUBMED Abstract]
  267. Madigan CE, Armenian SH, Malogolowkin MH, et al.: Extracranial malignant rhabdoid tumors in childhood: the Childrens Hospital Los Angeles experience. Cancer 110 (9): 2061-6, 2007. [PUBMED Abstract]
  268. Bourdeaut F, Fréneaux P, Thuille B, et al.: Extra-renal non-cerebral rhabdoid tumours. Pediatr Blood Cancer 51 (3): 363-8, 2008. [PUBMED Abstract]
  269. Wetmore C, Boyett J, Li S, et al.: Alisertib is active as single agent in recurrent atypical teratoid rhabdoid tumors in 4 children. Neuro Oncol 17 (6): 882-8, 2015. [PUBMED Abstract]
  270. Martignoni G, Pea M, Reghellin D, et al.: Molecular pathology of lymphangioleiomyomatosis and other perivascular epithelioid cell tumors. Arch Pathol Lab Med 134 (1): 33-40, 2010. [PUBMED Abstract]
  271. Bissler JJ, McCormack FX, Young LR, et al.: Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med 358 (2): 140-51, 2008. [PUBMED Abstract]
  272. Davies DM, Johnson SR, Tattersfield AE, et al.: Sirolimus therapy in tuberous sclerosis or sporadic lymphangioleiomyomatosis. N Engl J Med 358 (2): 200-3, 2008. [PUBMED Abstract]
  273. Agaram NP, Sung YS, Zhang L, et al.: Dichotomy of Genetic Abnormalities in PEComas With Therapeutic Implications. Am J Surg Pathol 39 (6): 813-25, 2015. [PUBMED Abstract]
  274. Folpe A, Inwards C, eds.: Bone and Soft Tissue Pathology: A Volume in the Foundations in Diagnostic Pathology. Philadelphia, Pa: WB Saunders Co, 2010.
  275. Armah HB, Parwani AV: Perivascular epithelioid cell tumor. Arch Pathol Lab Med 133 (4): 648-54, 2009. [PUBMED Abstract]
  276. Alaggio R, Cecchetto G, Martignoni G, et al.: Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature. J Pediatr Surg 47 (6): e31-40, 2012. [PUBMED Abstract]
  277. Wagner AJ, Malinowska-Kolodziej I, Morgan JA, et al.: Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. J Clin Oncol 28 (5): 835-40, 2010. [PUBMED Abstract]
  278. Spunt SL, Million L, Anderson JR, et al.: Risk-based treatment for nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) in patients under 30 years of age: Children’s Oncology Group study ARST0332. [Abstract] J Clin Oncol 32 (Suppl 15): A-10008, 2014. Also available online. Last accessed April 18, 2019.
  279. Laetsch TW, Roy A, Xu L, et al.: Undifferentiated Sarcomas in Children Harbor Clinically Relevant Oncogenic Fusions and Gene Copy-Number Alterations: A Report from the Children's Oncology Group. Clin Cancer Res 24 (16): 3888-3897, 2018. [PUBMED Abstract]
  280. Randall RL, Albritton KH, Ferney BJ, et al.: Malignant fibrous histiocytoma of soft tissue: an abandoned diagnosis. Am J Orthop 33 (12): 602-8, 2004. [PUBMED Abstract]
  281. Alaggio R, Collini P, Randall RL, et al.: Undifferentiated high-grade pleomorphic sarcomas in children: a clinicopathologic study of 10 cases and review of literature. Pediatr Dev Pathol 13 (3): 209-17, 2010 May-Jun. [PUBMED Abstract]
  282. Le Guellec S, Chibon F, Ouali M, et al.: Are peripheral purely undifferentiated pleomorphic sarcomas with MDM2 amplification dedifferentiated liposarcomas? Am J Surg Pathol 38 (3): 293-304, 2014. [PUBMED Abstract]
  283. Daw NC, Billups CA, Pappo AS, et al.: Malignant fibrous histiocytoma and other fibrohistiocytic tumors in pediatric patients: the St. Jude Children's Research Hospital experience. Cancer 97 (11): 2839-47, 2003. [PUBMED Abstract]
  284. Schaefer IM, Fletcher CDM: Recent advances in the diagnosis of soft tissue tumours. Pathology 50 (1): 37-48, 2018. [PUBMED Abstract]
  285. Kao YC, Owosho AA, Sung YS, et al.: BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas. Am J Surg Pathol 42 (5): 604-615, 2018. [PUBMED Abstract]
  286. Machado I, Navarro S, Llombart-Bosch A: Ewing sarcoma and the new emerging Ewing-like sarcomas: (CIC and BCOR-rearranged-sarcomas). A systematic review. Histol Histopathol 31 (11): 1169-81, 2016. [PUBMED Abstract]
  287. Cohen-Gogo S, Cellier C, Coindre JM, et al.: Ewing-like sarcomas with BCOR-CCNB3 fusion transcript: a clinical, radiological and pathological retrospective study from the Société Française des Cancers de L'Enfant. Pediatr Blood Cancer 61 (12): 2191-8, 2014. [PUBMED Abstract]
  288. Antonescu CR, Owosho AA, Zhang L, et al.: Sarcomas With CIC-rearrangements Are a Distinct Pathologic Entity With Aggressive Outcome: A Clinicopathologic and Molecular Study of 115 Cases. Am J Surg Pathol 41 (7): 941-949, 2017. [PUBMED Abstract]
  289. Coffin CM, Dehner LP: Vascular tumors in children and adolescents: a clinicopathologic study of 228 tumors in 222 patients. Pathol Annu 28 Pt 1: 97-120, 1993. [PUBMED Abstract]
  290. Mehrabi A, Kashfi A, Fonouni H, et al.: Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer 107 (9): 2108-21, 2006. [PUBMED Abstract]
  291. Haro A, Saitoh G, Tamiya S, et al.: Four-year natural clinical course of pulmonary epithelioid hemangioendothelioma without therapy. Thorac Cancer 6 (4): 544-7, 2015. [PUBMED Abstract]
  292. Sardaro A, Bardoscia L, Petruzzelli MF, et al.: Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor. Oncol Rev 8 (2): 259, 2014. [PUBMED Abstract]
  293. Dong K, Wang XX, Feng JL, et al.: Pathological characteristics of liver biopsies in eight patients with hepatic epithelioid hemangioendothelioma. Int J Clin Exp Pathol 8 (9): 11015-23, 2015. [PUBMED Abstract]
  294. Adams DM, Hammill A: Other vascular tumors. Semin Pediatr Surg 23 (4): 173-7, 2014. [PUBMED Abstract]
  295. Xiao Y, Wang C, Song Y, et al.: Primary epithelioid hemangioendothelioma of the kidney: the first case report in a child and literature review. Urology 82 (4): 925-7, 2013. [PUBMED Abstract]
  296. Reich S, Ringe H, Uhlenberg B, et al.: Epithelioid hemangioendothelioma of the lung presenting with pneumonia and heart rhythm disturbances in a teenage girl. J Pediatr Hematol Oncol 32 (4): 274-6, 2010. [PUBMED Abstract]
  297. Daller JA, Bueno J, Gutierrez J, et al.: Hepatic hemangioendothelioma: clinical experience and management strategy. J Pediatr Surg 34 (1): 98-105; discussion 105-6, 1999. [PUBMED Abstract]
  298. Ackermann O, Fabre M, Franchi S, et al.: Widening spectrum of liver angiosarcoma in children. J Pediatr Gastroenterol Nutr 53 (6): 615-9, 2011. [PUBMED Abstract]
  299. Stacchiotti S, Provenzano S, Dagrada G, et al.: Sirolimus in Advanced Epithelioid Hemangioendothelioma: A Retrospective Case-Series Analysis from the Italian Rare Cancer Network Database. Ann Surg Oncol 23 (9): 2735-44, 2016. [PUBMED Abstract]
  300. Semenisty V, Naroditsky I, Keidar Z, et al.: Pazopanib for metastatic pulmonary epithelioid hemangioendothelioma-a suitable treatment option: case report and review of anti-angiogenic treatment options. BMC Cancer 15: 402, 2015. [PUBMED Abstract]
  301. Raheja A, Suri A, Singh S, et al.: Multimodality management of a giant skull base hemangioendothelioma of the sphenopetroclival region. J Clin Neurosci 22 (9): 1495-8, 2015. [PUBMED Abstract]
  302. Ahmad N, Adams DM, Wang J, et al.: Hepatic epithelioid hemangioendothelioma in a patient with hemochromatosis. J Natl Compr Canc Netw 12 (9): 1203-7, 2014. [PUBMED Abstract]
  303. Otte JB, Zimmerman A: The role of liver transplantation for pediatric epithelioid hemangioendothelioma. Pediatr Transplant 14 (3): 295-7, 2010. [PUBMED Abstract]
  304. Cioffi A, Reichert S, Antonescu CR, et al.: Angiosarcomas and other sarcomas of endothelial origin. Hematol Oncol Clin North Am 27 (5): 975-88, 2013. [PUBMED Abstract]
  305. Jeng MR, Fuh B, Blatt J, et al.: Malignant transformation of infantile hemangioma to angiosarcoma: response to chemotherapy with bevacizumab. Pediatr Blood Cancer 61 (11): 2115-7, 2014. [PUBMED Abstract]
  306. Dehner LP, Ishak KG: Vascular tumors of the liver in infants and children. A study of 30 cases and review of the literature. Arch Pathol 92 (2): 101-11, 1971. [PUBMED Abstract]
  307. Ferrari A, Casanova M, Bisogno G, et al.: Malignant vascular tumors in children and adolescents: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Med Pediatr Oncol 39 (2): 109-14, 2002. [PUBMED Abstract]
  308. Deyrup AT, Miettinen M, North PE, et al.: Pediatric cutaneous angiosarcomas: a clinicopathologic study of 10 cases. Am J Surg Pathol 35 (1): 70-5, 2011. [PUBMED Abstract]
  309. Elliott P, Kleinschmidt I: Angiosarcoma of the liver in Great Britain in proximity to vinyl chloride sites. Occup Environ Med 54 (1): 14-8, 1997. [PUBMED Abstract]
  310. Lezama-del Valle P, Gerald WL, Tsai J, et al.: Malignant vascular tumors in young patients. Cancer 83 (8): 1634-9, 1998. [PUBMED Abstract]
  311. Fata F, O'Reilly E, Ilson D, et al.: Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer 86 (10): 2034-7, 1999. [PUBMED Abstract]
  312. Lahat G, Dhuka AR, Hallevi H, et al.: Angiosarcoma: clinical and molecular insights. Ann Surg 251 (6): 1098-106, 2010. [PUBMED Abstract]
  313. Orlando G, Adam R, Mirza D, et al.: Hepatic hemangiosarcoma: an absolute contraindication to liver transplantation--the European Liver Transplant Registry experience. Transplantation 95 (6): 872-7, 2013. [PUBMED Abstract]
  314. Sanada T, Nakayama H, Irisawa R, et al.: Clinical outcome and dose volume evaluation in patients who undergo brachytherapy for angiosarcoma of the scalp and face. Mol Clin Oncol 6 (3): 334-340, 2017. [PUBMED Abstract]
  315. Dickson MA, D'Adamo DR, Keohan ML, et al.: Phase II Trial of Gemcitabine and Docetaxel with Bevacizumab in Soft Tissue Sarcoma. Sarcoma 2015: 532478, 2015. [PUBMED Abstract]
  316. North PE, Waner M, Mizeracki A, et al.: A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol 137 (5): 559-70, 2001. [PUBMED Abstract]
  317. Boye E, Yu Y, Paranya G, et al.: Clonality and altered behavior of endothelial cells from hemangiomas. J Clin Invest 107 (6): 745-52, 2001. [PUBMED Abstract]
  318. Ravi V, Patel S: Vascular sarcomas. Curr Oncol Rep 15 (4): 347-55, 2013. [PUBMED Abstract]
  319. Grassia KL, Peterman CM, Iacobas I, et al.: Clinical case series of pediatric hepatic angiosarcoma. Pediatr Blood Cancer 64 (11): , 2017. [PUBMED Abstract]

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