Effective adjuvant chemotherapy for advanced nasopharyngeal carcinoma in children: A final update of a long-term prospective study in a single institution

Purpose: The purpose of this study was to determine the efficacy and toxicity of a doxorubicin/cyclophosphamide-based chemotherapy and local radiation therapy in children with locally advanced or metastatic nasopharyngeal carcinoma (NPC). Patients and Methods: Twelve patients aged 6 to 20 years old were treated with a chemotherapy regimen comprised of vincristine (1.5 mg/m(2)) and doxorubicin (45 mg/m(2)) on day 1 and cyclophosphamide (210 mg/m(2)) and 5-fluorouracil (240 mg/m(2)) on days 1 to 5. Chemotherapy was administered every 3 weeks for 1 to 2 years. Radiotherapy to the primary site (59 to 68 Gy) and to the neck (59 to 66 Gy) was given before or after 2 to 4 courses of chemotherapy. Results: All patients achieved a complete response 4 to 16 months from the start of therapy (median 7 months). Nine patients have remained tumor free from 2 to 21 years (median 11 years) from diagnosis. One child was lost to follow-up and one died of tuberculosis; both were disease-free. One child developed a secondary osteosarcoma in the left mandible. Chemotherapy caused grade 4 neutropenia and thrombocytopenia in four patients. There were no therapy-related deaths and the most common late effect of therapy was neck fibrosis, which was observed in all patients. We conclude that the chemotherapy and radiotherapy regimen used in this study is highly effective for children and adolescents with locally advanced NPC and is associated with tolerable toxicity.