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Histopathological Classification

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

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

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    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.
      • Rhabdomyosarcoma (embryonal, alveolar, and pleomorphic forms). (Refer to the Childhood Rhabdomyosarcoma Treatment summary for more information.)
    • 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).

    a Not a high-grade tumor; b The category of fibrosarcoma can be inclusive of fibrosarcomatous differentiation in dermatofibrosarcoma protuberans; c Cutaneous angiosarcoma may be difficult to stage using the American Joint Committee on Cancer system.

    References:

    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.
    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.
    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.
    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.
    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.
    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.

      WebMD Public Information from the National Cancer Institute

      Last Updated: February 25, 2014
      This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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