RHABDOMYOSARCOMA OF THE NOSE AND PARANASAL SINUSES IN ADULTS AND CHILDREN

OBJECTIVE: To determine the biologic behavior, risk factors for recurr ence, and influence of multimodality therapy on outcome for children a nd adults with rhabdomyosarcoma of the nose and paranasal sinuses. DES IGN: Retrospective review of medical records. PATIENTS: During a 36-ye ar period 37 patients (median age, 23.0 years) with histologically con firmed rhabdomyosarcoma of the nose and paranasal sinuses were treated for cure, Median follow-up was 28 months (range, 1 to 260 months). Hi stologic subtypes included embryonal, 16; alveolar, 15; mixed, 1; and unclassified, 5. Cervical metastases were present in 38%. Patients wer e treated with surgery, 4; radiotherapy, 3; chemotherapy, 3; surgery a nd radiotherapy 1; surgery and chemotherapy, 1; chemotherapy and radio therapy, 24; and chemotherapy, radiotherapy, and surgery, 1. RESULTS: The overall 5-year survival was 44%. For patients treated with chemoth erapy and radiotherapy or chemotherapy, radiotherapy, and surgery, the 5-year survival was 60%, compared with 19% for patients treated with the other forms of therapy. Factors associated with poorer survival we re adult onset of disease, alveolar histology, and treatment with syst emic chemotherapy for less than 1 year. Patients receiving chemotherap y for greater than 1 year had a 5-year survival of 82%, compared with 71% for those with less than 1 year of treatment. Improved survival wa s associated with a lower incidence of distant metastasis. CONCLUSIONS : A combination of chemotherapy and radiotherapy may provide Che best means of obtaining local-regional control for rhabdomyosarcoma arising in the nose and paranasal sinuses. The risk of regional disease is hi gh, requiring comprehensive radiotherapy to the neck in addition Co th e primary site. Surgical resection should be reserved for patients wit h residual disease after chemotherapy and radiotherapy. Administration of chemotherapy for more than 1 year is associated with improved surv ival because of a decreased incidence of metastatic disease.