Contemporary concepts in the management of pediatric rhabdomyosarcoma

Rhabdomyosarcoma is the most common soft-tissue sarcoma of childhood and adolescence, and just over one third of pediatric rhabdomyosarcoma occurs in the head and neck region. Over the past 25 years, the Intergroup Rhabdomyosarcoma Study Group protocols have increased 5-year survival rate from 55 to 71%. Although the Intergroup Rhabdomyosarcoma Study Group approach advocates primary chemotherapy and adjuvant radiation therapy, surgery still plays an important role in the multidisciplinary management of pediatric rhabdomyosarcoma. As survival rates have improved, more long-term, treatment-associated complications have been identified. The most frequent sequelae are related to radiation therapy, but occasional problems are encountered due to the cytotoxic effects of chemotherapy. Finally, molecular cytogenetics now plays a crucial role in the diagnosis and management of pediatric rhabdomyosarcoma.