Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ 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 +++ Diagnosis ++ 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 +++ Treatment ++ 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 Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Download the Access App: iOS | Android Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.