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Key Features

  • Most common soft tissue sarcoma occurring in childhood

  • Accounts for 10% of solid tumors in childhood

  • Peak incidence occurs at ages 2–5 years; diagnosed before age 10 in 70% of children

  • Males are affected more commonly than females

  • Can occur anywhere in the body

  • Classified into subtypes based on pathologic features

    • Embryonal (60–80%), of which botryoid is a variant

    • Alveolar (~15–20%)

    • Undifferentiated sarcoma (8%)

    • Pleomorphic, which is seen in adults (1%)

    • Other (11%)

Clinical Findings

  • Presenting symptoms and signs result from disturbances of normal body function due to tumor growth

  • For example, patients with orbital rhabdomyosarcoma present with proptosis, whereas patients with rhabdomyosarcoma of the bladder can present with hematuria, urinary obstruction, or a pelvic mass


  • Plain radiograph and a CT or MRI scan should be obtained to determine the extent of the primary tumor and to assess regional lymph nodes

  • Chest CT scan is obtained to rule out pulmonary metastasis, the most common site of metastatic disease at diagnosis

  • A skeletal survey and a bone scan are obtained to determine whether bony metastases are present

  • Bilateral bone marrow biopsies and aspirates are obtained to rule out bone marrow infiltration

  • When rhabdomyosarcoma imitates striated muscle and cross-striations are seen by light microscopy, the diagnosis is straightforward

  • Immunohistochemistry, electron microscopy, or chromosomal analysis is sometimes necessary to make the diagnosis


  • Chemotherapy

    • Indicated for all patients even when the tumor is fully resected

    • Can often convert an inoperable tumor to a resectable one

    • Exact regimen and duration of chemotherapy are determined by primary site, group, and tumor node metastasis classification

    • Vincristine, dactinomycin, and cyclophosphamide have shown the greatest efficacy

  • Radiation therapy

    • An effective method of local tumor control for both microscopic and gross residual disease

    • Generally administered to all patients, the only exception being those with a localized tumor that has been completely resected

  • Surgery

    • Tumor should be excised when possible

    • When only partial tumor resection is feasible, the procedure is usually limited to biopsy and sampling of lymph nodes

    • Debulking of unresectable tumor may improve outcomes by decreasing the assigned stage/group

    • A second-look procedure to remove residual disease and confirm the clinical response to chemotherapy and radiation therapy is generally performed at about week 20 of therapy

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