martes, 28 de febrero de 2017

Childhood Soft Tissue Sarcoma Treatment (PDQ®)—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


General Information About Childhood Soft Tissue Sarcoma

Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality decreased by more than 50%.[1] Childhood and adolescent cancer survivors require close monitoring because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancerfor specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)
Rhabdomyosarcoma, a tumor of striated muscle, is the most common soft tissue sarcoma in children aged 0 to 14 years and accounts for 50% of tumors in this age group.[2] (Refer to the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.) In pediatrics, the remaining soft tissue sarcomas are commonly referred to as nonrhabdomyosarcomatous soft tissue sarcomas and account for approximately 3% of all childhood tumors.[3] This heterogeneous group of tumors includes the following neoplasms:[4]
  • Connective tissue (e.g., desmoid-type fibromatosis).
  • Peripheral nervous system (e.g., malignant peripheral nerve sheath tumor).
  • Smooth muscle (e.g., leiomyosarcoma).
  • Vascular tissue (blood and lymphatic vessels, e.g., angiosarcoma). (Refer to the PDQ summary on Childhood Vascular Tumors Treatment for more information about childhood vascular tumors.)

Distribution of Soft Tissue Sarcoma by Age and Histology

Pediatric soft tissue sarcomas are a heterogenous group of malignant tumors that originate from primitive mesenchymal tissue and account for 7% of all childhood tumors.[5]
The distribution of soft tissue sarcomas by histology and age, based on the Surveillance, Epidemiology, and End Results (SEER) information from 1975 to 2012, is depicted in Table 1. The distribution of histologic subtypes by age is also shown in Figure 2.
Table 1. Age Distribution of Soft Tissue Sarcomas in Children Aged 0 to 19 Years (SEER 1975–2012)a
 Age <5 th="" y="">Age 5–9 yAge 10–14 yAge 15–19 y% of the Total Number of STS Cases <20 th="" y="">
pPNET = peripheral primitive neuroectodermal tumors; SEER = Surveillance, Epidemiology, and End Results; STS = soft tissue sarcoma.
aSEER data is available at
bDermatofibrosarcoma accounts for 75% of these cases.
All soft tissue and other extraosseous sarcomas9236319461,267100
Fibrosarcomas, peripheral nerve, and other fibrous neoplasms116508814110
 Fibroblastic and myofibroblastic tumors97243162 6
 Nerve sheath tumors19265677 5
 Other fibromatous neoplasms0012 0.1
Kaposi sarcoma21190.3
Other specified soft tissue sarcomas19419042470840
 Ewing tumor and Askin tumor of soft tissue27306292 6
 pPNET of soft tissue21183646 3.2
 Extrarenal rhabdoid tumor61373 2
 Liposarcomas352257 2.3
 Fibrohistiocytic tumors b3454108188 10
 Leiomyosarcomas9141536 2
 Synovial sarcomas1034111175 9
 Blood vessel tumors117825 1.4
 Osseous and chondromatous neoplasms of soft tissue161310 0.8
 Alveolar soft parts sarcoma431629 1.4
 Miscellaneous soft tissue sarcomas13163647 3
Unspecified soft tissue sarcomas60401111399.3
Nonrhabdomyosarcomatous soft tissue sarcomas are more common in adolescents and adults,[4] and most of the information regarding treatment and natural history of the disease in younger patients has been based on adult studies. The distributions of these tumors by age according to stage, histologic subtype, and tumor site are shown in Figures 1, 2, and 3, respectively.[6]
ENLARGEChart showing the distribution of nonrhabdomyosarcomatous soft tissue sarcomas by age according to stage.
Figure 1. The distribution of nonrhabdomyosarcomatous soft tissue sarcomas by age according to stage.
ENLARGEChart showing the distribution of nonrhabdomyosarcomatous soft tissue sarcomas by age according to histologic subtype.
Figure 2. The distribution of nonrhabdomyosarcomatous soft tissue sarcomas by age according to histologic subtype.
ENLARGEChart showing the distribution of nonrhabdomyosarcomatous soft tissue sarcomas by age according to tumor site.
Figure 3. The distribution of nonrhabdomyosarcomatous soft tissue sarcomas by age according to tumor site.

Risk Factors

Some genetic and environmental factors have been associated with the development of nonrhabdomyosarcomatous soft tissue sarcoma, including the following:
  • Genetic factors:
    • Li-Fraumeni syndrome: Patients with Li-Fraumeni syndrome (usually due to heritable cancer-associated changes of the TP53 tumor suppressor gene) have an increased risk of developing soft tissue tumors (mostly nonrhabdomyosarcomatous soft tissue sarcomas), bone sarcomas, breast cancer, brain tumors, and acute leukemia.[7,8]
    • Familial adenomatous polyposis: Patients with familial adenomatous polyposis are at increased risk of developing desmoid-type fibromatosis.[9]
    • Retinoblastoma (RB1) gene: Germline mutations of the retinoblastoma gene have been associated with an increased risk of developing soft tissue sarcomas, particularly leiomyosarcoma.[10]
    • Neurofibromatosis type 1: Approximately 4% of patients with neurofibromatosis type 1 develop malignant peripheral nerve sheath tumors, which usually develop after a long latency; some patients develop multiple lesions.[11-13]
    • Werner syndrome: Werner syndrome is characterized by spontaneous chromosomal instability, resulting in increased susceptibility to cancer and premature aging. An excess of soft tissue sarcomas has been reported in patients with Werner syndrome.[14]
  • Environmental factors:
    • Radiation: Some nonrhabdomyosarcomatous soft tissue sarcomas (particularly malignant fibrous histiocytoma) can develop within a previously irradiated site.[3,15]
    • Epstein-Barr virus infection in patients with AIDS: Some nonrhabdomyosarcomatous soft tissue sarcomas (e.g., leiomyosarcoma) have been linked to Epstein-Barr virus infection in patients with AIDS.[3,16]

Clinical Presentation

Although nonrhabdomyosarcomatous soft tissue sarcomas can develop in any part of the body, they arise most commonly in the trunk and extremities.[17-19] These neoplasms can present initially as an asymptomatic solid mass, or they may be symptomatic because of local invasion of adjacent anatomical structures. Although rare, these tumors can arise primarily in brain tissue and are treated according to the histiotype.[20]
Systemic symptoms (e.g., fever, weight loss, and night sweats) are rare. Hypoglycemia and hypophosphatemic rickets have been reported in cases of hemangiopericytoma, whereas hyperglycemia has been noted in patients with fibrosarcoma of the lung.[21]

Diagnostic and Staging Evaluation

When a suspicious lesion is identified, it is crucial that a complete workup, followed by adequate biopsy be performed. It is best to image the lesion using the following procedures before initiating any intervention:
  • Plain films. Plain films can be used to rule out bone involvement and detect calcifications that may be seen in soft tissue tumors such as extraskeletal osteosarcoma or synovial sarcoma.
  • Chest computed tomography (CT). Chest CT is essential to assess the presence of metastases.
  • Abdominal CT or magnetic resonance imaging (MRI). Abdominal CT or MRI can be used to image intra-abdominal tumors, such as liposarcoma.
  • Extremity MRI. MRI is essential for extremity lesions.
  • Positron emission tomography (PET) scan and bone scan. In children with rhabdomyosarcoma, PET-CT performed better than conventional imaging in identifying nodal, bone, bone marrow, and soft tissue disease. The authors of an imaging comparison study suggest that bone scans with Tc99m might be eliminated as a staging procedure.[22] The use of this modality in pediatric nonrhabdomyosarcomatous soft tissue sarcoma has not been studied extensively. However, a small study of nine patients with nonrhabdomyosarcomatous soft tissue sarcoma suggests that PET-CT is more accurate and cost effective than either modality alone in identifying distant metastatic disease.[23]
The imaging characteristics of some tumors can be highly suggestive of this diagnosis. For example, the imaging characteristics of pediatric low-grade fibromyxoid sarcoma and alveolar soft part sarcoma have been described and can aid in the diagnosis of these rare neoplasms.[24]

Biopsy strategies

Although nonrhabdomyosarcomatous soft tissue tumors are fairly readily distinguished pathologically from rhabdomyosarcoma and Ewing sarcoma, the classification of childhood nonrhabdomyosarcomatous soft tissue sarcoma type is often difficult. Core-needle biopsy, incisional biopsy, or excisional biopsy can be used to diagnose a nonrhabdomyosarcomatous soft tissue sarcoma. If possible, the surgeon who will perform the definitive resection needs to be involved in the biopsy decision. Poorly placed incisional or needle biopsies may adversely affect the performance of the primary resection.
Considerations related to the selection of a biopsy procedure are as follows:
  • Given the diagnostic importance of translocations, a core-needle biopsy or small incisional biopsy that obtains adequate tumor tissue is crucial to allow for conventional histology, immunocytochemical analysis, and other studies such as light and electron microscopy, cytogenetics, fluorescence in situ hybridization, and molecular pathology.[25,26] Core-needle biopsy for a deep-seated tumor can lead to formation of a hematoma, which affects subsequent resection and/or radiation; in these cases, incisional biopsy is the preferred procedure.
  • Fine-needle biopsy is usually not recommended because it is difficult to determine the accurate histologic diagnosis and grade of the tumor in this heterogeneous group of tumors.
  • Image guidance using ultrasound, CT scan, or MRI may be necessary to ensure a representative biopsy.[27]
  • Needle biopsy techniques must ensure adequate tissue sampling. The acquisition of multiple cores of tissue may be required.
  • Incisional biopsies must not compromise subsequent wide local excision.
  • Excisional biopsy of the lesion is only appropriate for small superficial lesions (<3 a="" and="" are="" cm="" discouraged.="" href="" in="" size="" style="border: 0px; box-sizing: border-box; color: #2b7bba; font-family: inherit; font-size: inherit; font-stretch: inherit; font-style: inherit; font-variant: inherit; font-weight: inherit; line-height: inherit; margin: 0px; padding: 0px; text-decoration: none; vertical-align: baseline;">28
,29] If an excisional biopsy is contemplated, then MRI of the area is recommended to define the area of involvement as subsequent surgery or radiation therapy is likely.
  • Various institutional series have demonstrated the feasibility and effectiveness of sentinel node biopsy as a staging procedure in pediatric patients with soft tissue sarcomas.[30-35]
  • Transverse extremity incisions are avoided to reduce skin loss and because they require a greater cross-sectional volume of tissue to be covered in the radiation field. Other extensive surgical procedures are also avoided before definitive diagnosis. For these reasons, open biopsy or multiple core-needle biopsies are strongly encouraged so that adequate tumor tissue can be obtained to allow crucial studies to be performed and to avoid limiting future treatment options.
  • Unplanned resection

    In children with unplanned resection of nonrhabdomyosarcomatous soft tissue sarcomas, primary re-excision is frequently recommended because many patients will have tumor present in the re-excision specimen.[36,37] A single-institution analysis of adolescents and adults compared patients with unplanned excision of soft tissue sarcoma to stage-matched controls. In this retrospective analysis, unplanned initial excision of soft tissue sarcoma resulted in increased risk of local recurrence, metastasis, and death; this increase was greatest for high-grade tumors.[38][Level of evidence: 3iiA]

    Chromosomal abnormalities

    Many nonrhabdomyosarcomatous soft tissue sarcomas are characterized by chromosomal abnormalities. Some of these chromosomal translocations lead to a fusion of two disparate genes. The resulting fusion transcript can be readily detected by using polymerase chain reaction-based techniques, thus facilitating the diagnosis of those neoplasms that have translocations.
    Some of the most frequent aberrations seen in nonrhabdomyosarcomatous soft tissue tumors are listed in Table 2.
    Table 2. Frequent Chromosomal Aberrations Seen in Nonrhabdomyosarcomatous Soft Tissue Sarcomaa
    HistologyChromosomal AberrationsGenes Involved
    aAdapted from Sandberg,[39] Slater et al.,[40] Mertens et al.,[41] and Romeo.[42]
    Alveolar soft part sarcomat(x;17)(p11.2;q25)ASPL/TFE3 [43-45]
    Angiomatoid fibrous histiocytomat(12;16)(q13;p11), t(2;22)(q33;q12), t(12;22)(q13;q12)FUS/ATF1EWSR1/CREB1,[46EWS/ATF1
    Clear cell sarcomat(12;22)(q13;q12), t(2;22)(q33;q12)ATF1/EWSEWSR1/CREB1
    Congenital (infantile) fibrosarcoma/mesoblastic nephromat(12;15)(p13;q25)ETV-NTRK3
    Dermatofibrosarcoma protuberanst(17;22)(q22;q13)COL1A1/PDGFB
    Desmoid fibromatosisTrisomy 8 or 20, loss of 5q21CTNNB1 or APC mutations
    Desmoplastic small round cell tumorst(11;22)(p13;q12)EWS/WT1 [47,48]
    Epithelioid hemangioendotheliomat(1;3)(p36;q25) [49]WWTR1/CAMTA1
    Epithelioid sarcomaInactivation SMARCB1SMARCB1
    Extraskeletal myxoid chondrosarcomat(9;22)(q22;q12), t(9;17)(q22;q11), t(9;15)(q22;q21), t(3;9)(q11;q22)EWSR1/NR4A3TAF2N/NR4A3TCF12/NR4A3TGF/NR4A3
    Hemangiopericytomat(12;19)(q13;q13.3) and t(13;22)(q22;q13.3) 
    Infantile fibrosarcomat(12;15)(p13;q25)ETV6/NTRK3
    Inflammatory myofibroblastic tumort(1;2)(q23;q23), t(2;19)(q23;q13), t(2;17)(q23;q23), t(2;2)(p23;q13), t(2;11)(p23;p15) [50]TPM3/ALKTPM4/ALKCLTC/ALKRANBP2/ALKCARS/ALKRAS
    Low-grade fibromyxoid sarcomat(7;16)(q33;p11), t(11;16)(p11;p11)FUS/CREB3L2FUS/CREB3L1
    Malignant peripheral nerve sheath tumor17q11.2, loss or rearrangement 10p, 11q, 17q, 22qNF1
    Mesenchymal chondrosarcomaDel(8)(q13.3q21.1)HEY1/NCOA2
    Myoepitheliomat(19;22)(q13;q12), t(1;22)(q23;q12), t(6;22)(p21;q12)EWSR/ZNF44EWSR/PBX1,EWSR/POU5F1
    Myxoid/round cell liposarcomat(12;16)(q13;p11), t(12;22)(q13;q12)FUS/DD1T3EWSR/DD1T3
    Rhabdoid tumorInactivation SMARCB1SMARCB1
    Solitary fibrous tumorInv(12)(q13q13)NAB2/STAT6
    Synovial sarcomat(x;18)(p11.2;q11.2)SYT/SSX
    Tenosynovial giant cell tumort(1;2)(p13;q35)COL6A3/CSF1


    The prognosis of nonrhabdomyosarcomatous soft tissue sarcoma varies greatly depending on the following factors:[51-53]
    • Site of the primary tumor.
    • Tumor size.
    • Tumor grade. (Refer to the Prognostic Significance of Tumor Grading section of this summary for more information.)
    • Tumor histology.
    • Depth of tumor invasion.
    • Presence of metastases.
    • Resectability of the tumor.
    • Use of radiation therapy.
    Several adult and pediatric series have shown that patients with large or invasive tumors have a significantly worse prognosis than do those with small, noninvasive tumors. A retrospective review of soft tissue sarcomas in children and adolescents suggests that the 5 cm cutoff used for adults with soft tissue sarcoma may not be ideal for smaller children, especially infants. The review identified an interaction between tumor diameter and body surface area.[54] This relationship requires further study to determine the therapeutic implications of the observation.
    In a review of a large adult series of nonrhabdomyosarcomatous soft tissue sarcomas, superficial extremity sarcomas had a better prognosis than did deep tumors. Thus, in addition to grade and size, the depth of invasion of the tumor should be considered.[55]
    Some pediatric nonrhabdomyosarcomatous soft tissue sarcomas are associated with a better outcome. For instance, infantile fibrosarcoma, presenting in infants and children younger than 5 years, has an excellent prognosis given that surgery alone can cure a significant number of these patients and the tumor is highly chemosensitive.[3]
    Soft tissue sarcomas in older children and adolescents often behave similarly to those in adult patients.[3,25] A large, prospective, multinational Children's Oncology Group study (ARST0332 [NCT00346164]) enrolled newly diagnosed patients younger than 30 years. Patients were assigned to treatment on the basis of their risk group (refer to Figure 4).[56][Level of evidence: 2A]
    ENLARGEChart showing risk stratification and treatment assignment for the Children's Oncology Group ARST0332 trial.
    Figure 4. Risk stratification and treatment assignment for the Children's Oncology Group ARST0332 trial. Credit: Sheri L. Spunt, M.D., M.B.A.
    1. Arm A (grossly excised low-grade tumor and ≤5 cm widely excised high-grade tumor): Surgery only.
    2. Arm B (≤5 cm marginally resected high-grade tumor): 55.8 Gy of radiation therapy.
    3. Arm C (>5 cm grossly resected tumor ± metastases): Ifosfamide/doxorubicin chemotherapy and 55.8 Gy of radiation therapy.
    4. Arm D (>5 cm unresected tumor ± metastases): Preoperative ifosfamide/doxorubicin chemotherapy and 45 Gy of radiation therapy, and then surgery and a radiation boost that was based on margins.
    Of 551 patients enrolled, at a median follow-up of 2.6 years, the preliminary analysis estimated the following 3-year survival rates:[56]
    • Arm A: 91% event-free survival (EFS); 99% overall survival (OS).
    • Arm B: 79% EFS; 100% OS.
    • Arm C: 68% EFS; 81% OS.
    • Arm D: 52% EFS; 66% OS.
    Pediatric patients with unresected localized nonrhabdomyosarcomatous soft tissue sarcomas have a poor outcome. Only about one-third of patients treated with multimodality therapy remain disease free.[51,57]; [58,59][Level of evidence: 3iiiA]
    In a pooled analysis from U.S. and European pediatric centers, outcome was better for patients whose tumor removal procedure was deemed complete than for patients whose tumor removal was incomplete. Outcome was better for patients who received radiation therapy than for patients who did not.[58][Level of evidence: 3iiiA]
    Because long-term related morbidity must be minimized while disease-free survival is maximized, the ideal therapy for each patient must be carefully and individually determined utilizing these prognostic factors before initiating therapy.[18,60-64]

    Related Summaries

    Refer to the following PDQ summaries for information about other types of sarcoma:
    1. Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014. [PUBMED Abstract]
    2. Ries LA, Smith MA, Gurney JG, et al., eds.: Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995. Bethesda, Md: National Cancer Institute, SEER Program, 1999. NIH Pub.No. 99-4649. Also available online. Last accessed September 19, 2016.
    3. 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.
    4. Weiss SW, Goldblum JR: General considerations. In: Weiss SW, Goldblum JR: Enzinger and Weiss's Soft Tissue Tumors. 5th ed. St. Louis, Mo: Mosby, 2008, pp 1-14.
    5. Pappo AS, Pratt CB: Soft tissue sarcomas in children. Cancer Treat Res 91: 205-22, 1997. [PUBMED Abstract]
    6. 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]
    7. Chang F, Syrjänen S, Syrjänen K: Implications of the p53 tumor-suppressor gene in clinical oncology. J Clin Oncol 13 (4): 1009-22, 1995. [PUBMED Abstract]
    8. Plon SE, Malkin D: Childhood cancer and hereditary. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams and Wilkins, 2015, pp 13-31.
    9. Groen EJ, Roos A, Muntinghe FL, et al.: Extra-intestinal manifestations of familial adenomatous polyposis. Ann Surg Oncol 15 (9): 2439-50, 2008. [PUBMED Abstract]
    10. 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]
    11. Weiss SW, Goldblum JR: Benign tumors of peripheral nerves. In: Weiss SW, Goldblum JR: Enzinger and Weiss's Soft Tissue Tumors. 5th ed. St. Louis, Mo: Mosby, 2008, pp 825-901.
    12. deCou JM, Rao BN, Parham DM, et al.: Malignant peripheral nerve sheath tumors: the St. Jude Children's Research Hospital experience. Ann Surg Oncol 2 (6): 524-9, 1995. [PUBMED Abstract]
    13. Stark AM, Buhl R, Hugo HH, et al.: Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature. Acta Neurochir (Wien) 143 (4): 357-63; discussion 363-4, 2001. [PUBMED Abstract]
    14. Goto M, Miller RW, Ishikawa Y, et al.: Excess of rare cancers in Werner syndrome (adult progeria). Cancer Epidemiol Biomarkers Prev 5 (4): 239-46, 1996. [PUBMED Abstract]
    15. Weiss SW, Goldblum JR: Malignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma). In: Weiss SW, Goldblum JR: Enzinger and Weiss's Soft Tissue Tumors. 5th ed. St. Louis, Mo: Mosby, 2008, pp 403-27.
    16. McClain KL, Leach CT, Jenson HB, et al.: Association of Epstein-Barr virus with leiomyosarcomas in children with AIDS. N Engl J Med 332 (1): 12-8, 1995. [PUBMED Abstract]
    17. Dillon P, Maurer H, Jenkins J, et al.: A prospective study of nonrhabdomyosarcoma soft tissue sarcomas in the pediatric age group. J Pediatr Surg 27 (2): 241-4; discussion 244-5, 1992. [PUBMED Abstract]
    18. Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Semin Surg Oncol 9 (6): 524-31, 1993 Nov-Dec. [PUBMED Abstract]
    19. Zeytoonjian T, Mankin HJ, Gebhardt MC, et al.: Distal lower extremity sarcomas: frequency of occurrence and patient survival rate. Foot Ankle Int 25 (5): 325-30, 2004. [PUBMED Abstract]
    20. Benesch M, von Bueren AO, Dantonello T, et al.: Primary intracranial soft tissue sarcoma in children and adolescents: a cooperative analysis of the European CWS and HIT study groups. J Neurooncol 111 (3): 337-45, 2013. [PUBMED Abstract]
    21. Weiss SW, Goldblum JR: Miscellaneous tumors of intermediate malignancy. In: Weiss SW, Goldblum JR: Enzinger and Weiss's Soft Tissue Tumors. 5th ed. St. Louis, Mo: Mosby, 2008, pp 1093-1160.
    22. Federico SM, Spunt SL, Krasin MJ, et al.: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer 60 (7): 1128-34, 2013. [PUBMED Abstract]
    23. Tateishi U, Hosono A, Makimoto A, et al.: Accuracy of 18F fluorodeoxyglucose positron emission tomography/computed tomography in staging of pediatric sarcomas. J Pediatr Hematol Oncol 29 (9): 608-12, 2007. [PUBMED Abstract]
    24. 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]
    25. Weiss SW, Goldblum JR: Enzinger and Weiss's Soft Tissue Tumors. 4th ed. St. Louis, Mo: Mosby, 2001.
    26. Recommendations for the reporting of soft tissue sarcomas. Association of Directors of Anatomic and Surgical Pathology. Mod Pathol 11 (12): 1257-61, 1998. [PUBMED Abstract]
    27. Chowdhury T, Barnacle A, Haque S, et al.: Ultrasound-guided core needle biopsy for the diagnosis of rhabdomyosarcoma in childhood. Pediatr Blood Cancer 53 (3): 356-60, 2009. [PUBMED Abstract]
    28. Coffin CM, Dehner LP, O'Shea PA: Pediatric Soft Tissue Tumors: A Clinical, Pathological, and Therapeutic Approach. Baltimore, Md: Williams and Wilkins, 1997.
    29. Smith LM, Watterson J, Scott SM: Medical and surgical management of pediatric soft tissue tumors. In: Coffin CM, Dehner LP, O'Shea PA: Pediatric Soft Tissue Tumors: A Clinical, Pathological, and Therapeutic Approach. Baltimore, Md: Williams and Wilkins, 1997, pp 360-71.
    30. Neville HL, Andrassy RJ, Lally KP, et al.: Lymphatic mapping with sentinel node biopsy in pediatric patients. J Pediatr Surg 35 (6): 961-4, 2000. [PUBMED Abstract]
    31. Neville HL, Raney RB, Andrassy RJ, et al.: Multidisciplinary management of pediatric soft-tissue sarcoma. Oncology (Huntingt) 14 (10): 1471-81; discussion 1482-6, 1489-90, 2000. [PUBMED Abstract]
    32. Kayton ML, Delgado R, Busam K, et al.: Experience with 31 sentinel lymph node biopsies for sarcomas and carcinomas in pediatric patients. Cancer 112 (9): 2052-9, 2008. [PUBMED Abstract]
    33. Dall'Igna P, De Corti F, Alaggio R, et al.: Sentinel node biopsy in pediatric patients: the experience in a single institution. Eur J Pediatr Surg 24 (6): 482-7, 2014. [PUBMED Abstract]
    34. Parida L, Morrisson GT, Shammas A, et al.: Role of lymphoscintigraphy and sentinel lymph node biopsy in the management of pediatric melanoma and sarcoma. Pediatr Surg Int 28 (6): 571-8, 2012. [PUBMED Abstract]
    35. Alcorn KM, Deans KJ, Congeni A, et al.: Sentinel lymph node biopsy in pediatric soft tissue sarcoma patients: utility and concordance with imaging. J Pediatr Surg 48 (9): 1903-6, 2013. [PUBMED Abstract]
    36. 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]
    37. 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]
    38. Qureshi YA, Huddy JR, Miller JD, et al.: Unplanned excision of soft tissue sarcoma results in increased rates of local recurrence despite full further oncological treatment. Ann Surg Oncol 19 (3): 871-7, 2012. [PUBMED Abstract]
    39. Sandberg AA: Translocations in malignant tumors. Am J Pathol 159 (6): 1979-80, 2001. [PUBMED Abstract]
    40. Slater O, Shipley J: Clinical relevance of molecular genetics to paediatric sarcomas. J Clin Pathol 60 (11): 1187-94, 2007. [PUBMED Abstract]
    41. Mertens F, Antonescu CR, Hohenberger P, et al.: Translocation-related sarcomas. Semin Oncol 36 (4): 312-23, 2009. [PUBMED Abstract]
    42. Romeo S, Dei Tos AP: Clinical application of molecular pathology in sarcomas. Curr Opin Oncol 23 (4): 379-84, 2011. [PUBMED Abstract]
    43. 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]
    44. Ladanyi M: The emerging molecular genetics of sarcoma translocations. Diagn Mol Pathol 4 (3): 162-73, 1995. [PUBMED Abstract]
    45. 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]
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    • Updated: February 15, 2017

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