Head and neck rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) accounts for approximately half of all pediatric soft tissue sarcomas and 15% of all pediatric solid tumors. The head and neck site accounts for 35% of all RMS. The head and neck site can be divided into three broad groups: cranial paramengingeal, orbital, and nonorbital-nonparameningeal. The predominant specific sites are orbit and nasopharynx, each accounting for 25%, with paranasal sinuses, ear, intraoral, neck, and parotid each accounting for about 10%. RMS in the orbit has the best outlook; parameningeal involvement is associated with the poorest outcome. Treatment is determined by the extent of disease, ease of resectability, and precise site. Superficially placed tumors can be managed either with excision and relatively nontoxic adjuvant chemotherapy or by biopsy, radiation, and similar chemotherapy, with equally good results (survival rate, 90%). More deeply placed lesions, especially those with parameningeal locations or extension, generally are not removable and require radiation therapy, including radiation to the entire cranial neuraxis, intrathecal chemotherapy, and very aggressive chemotherapy. The more intensified therapy resulted in increased survival rates of 57%. Non-RMS soft tissue sarcomas occur infrequently in the head and neck. Resection is the most effective treatment for this diverse group of tumors. Wide resection is best, if feasible. The role of chemotherapy is unclear because most of these tumors are radiosensitive. These tumors are often managed by protocols used for the treatment of RMS or extraosseous Ewing's sarcoma.