Approximately 7 percent of childhoo...

Approximately 7 percent of childhood malignancies are plastic tissue sarcomas. One half of these malignancies are rhabdomyosarcomas (RMS) originating in striated muscle. Although adult sarcomas primarily involve the extremities, RM in children can come into view in any anatomic location, including locations with no skeletal muscle. The in the greatest degree common sites are in the genitourinary regularitys extremities, head, and neck. A review by way of Andrassy emphasizes the importance of tumor location and individualized management in the prognosis of RM in children.

The clinical presentation hangs on tumor location and size. About the same third of cases originate in the head and neck and 25 to 35 percent of these have central nervous hypothesis involvement. The presentation may be of an unexplained mass or cranial hardihood palsies, meningeal symptoms, or plane respiratory problems caused by brainstem infiltration. About 26 percent of pediatric RM cases befall in the genitourinary system. The painless swelling may initially be diagnosed as a hernia, hydrocele or varicocele in male childs and may cause bleeding, vaginal discharge, or a mass in girls. In adolescents, RM more commonly not absents as a painless mass in the extremities, unless up to one half of cases have spread to lymph nodes at the time of diagnostic biopsy. Regardless of site, biopsy is first note of the scale to diagnosis and staging. Excisional biopsy is possible in a certain quantity of cases, depending on the location and size of the lesion. on a level with clear margins, regional node sampling is commited following wide excisional biopsy to plan radiation therapy or other following treatment and more accurately provide prognosis. Depending upon the situation, computed tomography or magnetic resonance imaging assessment of the primary site, regional nodes, or areas of likely metastasis also may be indicated for diagnosis and staging. The Intergroup RM close attention system of staging correlates with overall survival (see the accompanying table). Five-year survival for patients in dispose I is more than 90 percent following thorough excision, whereas rates for patients in clump IV average 30 to 35 percent

undiminished local excision is the approveed initial treatment of RMS, do not include for lesions of the orbit, vagina, or bladder. In these locations, chemotherapy, with or without radiation, plus limited surgery provides conclusions comparable with more aggressive surgery Regardless of site or exemplar of tumor, surgery must be carefully planned to balance the ne for tumor removal against los of function and mutilation. Overall, surgical treatment has become les aggressive, with more patients being managed through limited initial surgery followed by the agency of chemotherapy or subsequent surgeries. The traditional mainstay of chemotherapy for RM has been combinations of vincristine, dactinomycin, and cyclophosphamide. Other medicines such as doxorubicin, ifosphamide, actinomycin, topotecan, and irinotecan may be used as part of combination therapy targeting specific tumor adumbrations Radiation (usually external beam) is used selectively to enhance local tumor control

The management of RM hangs on the site and archetype of tumor. Surgery is repeatedly limited in the head and neck for cosmetic reasons and because metastasis to cervical nodes is rare. Biopsy plus chemotherapy and radiation can rise in 90 percent survival rates in orbital lesions without the ne for extensive surgery In RM of the bladder, more conservative surgical treatments have riseed in 85 percent survival, with 60 percent of patients retaining bladder function. The treatment of paratesticular RM is controversial because of the high probability of retroperitoneal lymph node involvement, especially in adolescents. Radical inguinal orchiectomy is attract favor toed but experts disagree on the magnitude of retroperitoneal node dissection. Adjuvant chemotherapy combined with surgery comes in survivals of more than 90 percent For lesions of the female genital tract, management is now based forward local resection followed by chemotherapy with or without selective radiotherapy. Radical surgical excision is no longer attract favor toed The worst outlook in RM is in patients with lesions in the extremities. Surgery must balance limb sparing and righteous functional outcome with eradication of disease. Up to united half of patients with RM of the extremities have positive lymph nodes and require radiation therapy.

Andrassy RJ Advances in the surgical management of sarcomas in children. Am J Surg December 2002;184:484-91

COPYRIGHT 2003 American Academy of Family Physicians

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