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Childhood Soft Tissue Sarcoma Treatment (PDQ®) 1/7—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

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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 Cancer for 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 (rhabdomyosarcomas, 4%; other soft tissue sarcomas, 3%).[5]
The distribution of soft tissue sarcomas by histology and age, on the basis of the Surveillance, Epidemiology, and End Results (SEER) information from 2000 to 2015, 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 2000–2015)a
ENLARGE
 Age <5 yAge 5–9 yAge 10–14 yAge 15–19 yAge <20 yAll Ages (Including Adults)
pPNET = peripheral primitive neuroectodermal tumors; SEER = Surveillance, Epidemiology, and End Results.
aSource: SEER database.[6]
All soft tissue and other extraosseous sarcomas1,1247731,2011,5584,65680,269
 
Rhabdomyosarcomas6684173823271,7943,284
Fibrosarcomas, peripheral nerve, and other fibrous neoplasms137641121814946,645
 Fibroblastic and myofibroblastic tumors114334177265 4,228
 Nerve sheath tumors233170102226 2,303
 Other fibromatous neoplasms00123 114
Kaposi sarcoma21210157,722
Other specified soft tissue sarcomas2372385598651,89949,004
 Ewing tumor and Askin tumor of soft tissue373672113258 596
 pPNET of soft tissue24234256145 402
 Extrarenal rhabdoid tumor7589496 205
 Liposarcomas463779126 10,749
 Fibrohistiocytic tumors4373142223481 13,531
 Leiomyosarcomas1114194185 14,107
 Synovial sarcomas1239141210402 2,608
 Blood vessel tumors129113264 4,238
 Osseous and chondromatous neoplasms of soft tissue16161437 1,018
 Alveolar soft parts sarcoma45223364 211
 Miscellaneous soft tissue sarcomas14194860141 1,339
Unspecified soft tissue sarcomas805314617545413,614
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 (Figure 1), histologic subtype (Figure 2), and tumor site (Figure 3) are shown below.[7]
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 factors and external exposures 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.[8,9]
    • Familial adenomatous polyposis: Patients with familial adenomatous polyposis are at increased risk of developing desmoid-type fibromatosis.[10]
    • RB1 gene: Germline mutations of the RB1 gene have been associated with an increased risk of developing soft tissue sarcoma, particularly leiomyosarcoma, and the risk appears higher among those younger than 1 year who were treated with alkylating agents.[11,12]
    • SMARCB1 gene: Germline mutations or deletions of the SMARCB1 (INI1) gene are associated with an increased risk of developing extrarenal rhabdoid tumors.[13]
    • 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.[14-16]
    • 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.[17]
    • Tuberous sclerosis complex: Tuberous sclerosis complex is associated with the development of various tumors showing perivascular epithelioid cell differentiation (PEComas), including lymphangioleiomyomatosis and hepatic and renal angiomyolipomas.[18-20]
    • Adenosine deaminase-deficient severe combined immunodeficiency: Patients with adenosine deaminase-deficient severe combined immunodeficiency have been reported to be at increased risk of developing multicentric dermatofibrosarcoma protuberans, which usually presents at an average age of 8.9 years.[21]
  • External exposures:
    • Radiation: Some nonrhabdomyosarcomatous soft tissue sarcomas (particularly malignant fibrous histiocytoma) can develop within a previously irradiated site.[3,22-25]
    • 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.[3,26]

Clinical Presentation

Although nonrhabdomyosarcomatous soft tissue sarcomas can develop in any part of the body, they arise most commonly in the trunk and extremities.[27-29] 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 in brain tissue and are treated according to the histotype.[30]
Systemic symptoms (e.g., fever, weight loss, and night sweats) are rare. Hypoglycemia and hypophosphatemic rickets have been reported in cases of hemangiopericytoma (now identified as a solitary fibrous tumor in the revised World Health Organization classification system), whereas hyperglycemia has been noted in patients with fibrosarcoma of the lung.[31]

Diagnostic and Staging Evaluation

When a suspicious lesion is identified, it is crucial that a complete workup, followed by adequate biopsy be performed. The lesion is imaged before initiating any intervention using the following procedures:
  • 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.
    • Rhabdomyosarcoma. In children with rhabdomyosarcoma, PET-CT performed better than conventional imaging in identifying nodal, bone, bone marrow, and soft tissue disease. The authors of this imaging comparison study suggested that bone scans with technetium Tc 99m might be eliminated as a staging procedure.[32]
    • Other soft tissue sarcomas. In a retrospective study, 46 PET scans were completed in 25 pediatric patients with soft tissue sarcoma.[33] The positive predictive value of finding metastatic disease was 89%, and the negative predictive value was 67%. A small study of nine patients with nonrhabdomyosarcomatous soft tissue sarcoma suggested that PET-CT was more accurate and cost-effective than either modality alone in identifying distant metastatic disease.[34] The use of this modality in pediatric nonrhabdomyosarcomatous soft tissue sarcoma has not been studied prospectively.
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.[35]

Biopsy strategies

Although nonrhabdomyosarcomatous soft tissue tumors are pathologically distinct 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 ability to achieve negative margins.
Given the diagnostic importance of translocations and other molecular changes, 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.[36,37] Needle biopsy techniques must ensure adequate tissue sampling. The acquisition of multiple cores of tissue may be required. Of 530 suspected soft tissue masses in (largely adult) patients who underwent core-needle biopsies, 426 (80%) were proven to be soft tissue tumors, 225 (52.8%) of which were malignant. Core-needle biopsy was able to differentiate soft tissue sarcomas from benign lesions with a sensitivity of 96.3% and a specificity of 99.4%. Tumor subtype was accurately assigned in 89.5% of benign lesions and in 88% of soft tissue sarcomas. The complication rate was 0.4%.[38] Considerations related to the biopsy procedure are as follows:
  • Core-needle biopsy for a deep-seated tumor can lead to formation of a hematoma, which affects subsequent resection and/or radiation.
  • 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.[39] Image guidance is particularly helpful in deep lesions and to avoid cystic changes or necrotic tumors.[40]
  • Incisional biopsies must not compromise subsequent wide local excision.
  • Excisional biopsy of the lesion is only appropriate for small superficial lesions (<3 cm in size) and are discouraged.[41,42] 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.[43-48] The utility of sentinel node biopsy is limited to epithelioid sarcoma, clear cell sarcoma, and trunk and extremity rhabdomyosarcoma.[49]
  • Transverse extremity incisions are avoided to reduce skin loss at re-excision 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.[50,51] 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.[52][Level of evidence: 3iiA] In this case, a second resection is expected.

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,[53] Slater et al.,[54] Mertens et al.,[55] Romeo,[56] and Schaefer et al.[57]
Alveolar soft part sarcomat(x;17)(p11.2;q25)ASPL/TFE3 [58-60]
Angiomatoid fibrous histiocytomat(12;16)(q13;p11), t(2;22)(q33;q12), t(12;22)(q13;q12)FUS/ATF1, EWSR1/CREB1,[61] EWSR1/ATF1
BCOR-rearranged sarcomasinv(X)(p11.4;p11.2)BCOR/CCNB3
CIC-rearranged sarcomast(4;19)(q35;q13), t(10;19)(q26;q13)CIC-DUX4
Clear cell sarcomat(12;22)(q13;q12), t(2;22)(q33;q12)ATF1/EWSR1, EWSR1/CREB1[62]
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)EWSR1/WT1 [63,64]
Epithelioid hemangioendotheliomat(1;3)(p36;q25) [65]WWTR1/CAMTA1
Epithelioid sarcomaInactivation of SMARCB1SMARCB1
Extraskeletal myxoid chondrosarcomat(9;22)(q22;q12), t(9;17)(q22;q11), t(9;15)(q22;q21), t(3;9)(q11;q22)EWSR1/NR4A3, TAF2N/NR4A3, TCF12/NR4A3, TGF/NR4A3
Hemangiopericytomat(12;19)(q13;q13.3) and t(13;22)(q22;q13.3)LMNA-NTRK1 [66]
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) [67]TPM3/ALK, TPM4/ALK, CLTC/ALK, RANBP2/ALK, CARS/ALK, RAS
Infantile myofibromatosis PDGFRB [68]
Low-grade fibromyxoid sarcomat(7;16)(q33;p11), t(11;16)(p11;p11)FUS/CREB3L2, FUS/CREB3L1
Malignant peripheral nerve sheath tumor17q11.2, loss or rearrangement of 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)EWSR1/ZNF44, EWSR1/PBX1,EWSR1/POU5F1
Myxoid/round cell liposarcomat(12;16)(q13;p11), t(12;22)(q13;q12)FUS/DD1T3, EWSR1/DD1T3
Primitive myxoid mesenchymal tumor of infancy BCOR internal tandem duplications
Rhabdoid tumorInactivation of SMARCB1SMARCB1
Sclerosing epithelioid fibrosarcoma EWSR1/CREB3L2
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

Prognosis and Prognostic Factors

The prognosis of nonrhabdomyosarcomatous soft tissue sarcoma varies greatly depending on the following factors:[69-71]
  • 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 and site of the metastatic tumor.
  • Resectability of the tumor.
  • Use of radiation therapy.
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.[72]
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.[73] This relationship requires further study to determine the therapeutic implications of the observation.
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.[3,74] 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 (defined by the presence of metastasis, tumor resectability and margins, and tumor size and grade; refer to Figure 4).[75][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:[75]
  • 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.[69,76]; [77,78][Level of evidence: 3iiiA] In an Italian review of 30 patients with nonrhabdomyosarcomatous soft tissue sarcoma at visceral sites, only ten patients survived at 5 years. Unfavorable prognostic factors included inability to achieve complete resection, large tumor size, tumor invasion, histologic subtype, and lung-pleura sites.[79][Level of evidence: 3iiB]
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.[77][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.[28,80-84]

Related Summaries

Refer to the following PDQ summaries for information about other types of sarcoma:
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