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Childhood Soft Tissue Sarcoma Treatment (PDQ®)

  • Last Modified: 09/08/2014

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Histopathological Classification

World Health Organization (WHO) Classification of Soft Tissue Sarcomas (STSs)

World Health Organization (WHO) Classification of Soft Tissue Sarcomas (STSs)

The WHO lists the following cell types in its classification of STSs:[1,2]

This summary focuses on high-grade sarcomas and low-grade tumors that present special problems in the pediatric and adolescent population, including desmoid tumor and infantile fibrosarcoma. For many low-grade STSs, surgical resection is curative and there is no need for additional therapy.

  • Adipocytic tumors.
    • Liposarcoma—myxoid or well-differentiated.
  • Chondro-osseous tumors.
    • Extraskeletal chondrosarcoma (mesenchymal and other variants).[3]
    • Extraskeletal osteosarcoma.
  • Fibroblastic/myofibroblastic tumors.
    • Desmoid tumor (also called aggressive fibromatosis).a
    • Fibrosarcoma.b[4]
    • Inflammatory myofibroblastic tumor.a
    • Low-grade fibromyxoid sarcoma.[5]
    • Myxofibrosarcoma, low grade.
    • Sclerosing epithelioid fibrosarcoma.
  • Skeletal muscle tumors.
  • Smooth muscle tumors.
    • Leiomyosarcoma.
  • So-called fibrohistiocytic tumors.
    • Plexiform fibrohistiocytic tumor.a
    • Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma (including pleomorphic, giant cell, myxoid/high-grade myxofibrosarcoma, and inflammatory forms).
  • Tumors of peripheral nerves.
    • Malignant peripheral nerve sheath tumor.
  • Tumors of uncertain differentiation.
    • Alveolar soft part sarcoma.
    • Clear cell sarcoma of soft tissue.
    • Desmoplastic small round cell tumor.[6]
    • Epithelioid sarcoma.
    • Extrarenal rhabdoid tumor.
    • Extraskeletal myxoid chondrosarcoma.
    • Primitive neuroectodermal tumor (PNET)/extraskeletal Ewing tumor.
    • Synovial sarcoma.
    • Undifferentiated sarcoma; sarcoma, not otherwise specified (NOS).[7]
  • Vascular tumors.
    • Angiosarcoma, deep.c
    • Epithelioid hemangioendothelioma.
    • Hemangiopericytoma (infantile).

 [Note: aNot a high-grade tumor; bThe category of fibrosarcoma can be inclusive of fibrosarcomatous differentiation in dermatofibrosarcoma protuberans; cCutaneous angiosarcoma may be difficult to stage using the American Joint Committee on Cancer system.]

  1. Soft tissue sarcoma. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 291-6. 

  2. Brodowicz T, Schwameis E, Widder J, et al.: Intensified Adjuvant IFADIC Chemotherapy for Adult Soft Tissue Sarcoma: A Prospective Randomized Feasibility Trial. Sarcoma 4 (4): 151-60, 2000.  [PUBMED Abstract]

  3. 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]

  4. Steelman C, Katzenstein H, Parham D, et al.: Unusual presentation of congenital infantile fibrosarcoma in seven infants with molecular-genetic analysis. Fetal Pediatr Pathol 30 (5): 329-37, 2011.  [PUBMED Abstract]

  5. 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]

  6. Barnoud R, Sabourin JC, Pasquier D, et al.: Immunohistochemical expression of WT1 by desmoplastic small round cell tumor: a comparative study with other small round cell tumors. Am J Surg Pathol 24 (6): 830-6, 2000.  [PUBMED Abstract]

  7. 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]