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/m2) and doxorubicin (45 mg/m2) on day 1 and cyclophosphamide (210 mg/m2) and 5-fluorouracil (240 mg/m2) 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.

Adjuvant chemotherapy for advanced nasopharyngeal carcinoma in childhood.

Seven children with advanced nasopharyngeal carcinoma younger than 20 years of age diagnosed between 1975 and 1986 (inclusive) were treated with a uniform adjuvant chemotherapy regimen, which consisted of vincristine (1.5 mg/m2; day 1), doxorubicin (45 mg/m2; day 1), 5-fluorouracil (8 mg/kg; days 1 through 5), and cyclophosphamide (7 mg/kg; days 1 through 5). This combination chemotherapy was given for 12 to 24 months after completion of radiation therapy. The radiation doses to the primary sites ranged from 6000 cGy to a maximum of 6800 cGy. The radiation doses for neck prophylaxis ranged from 4500 cGy to a total of 5000 cGy. Involved sites were irradiated to at least an additional boost of 1000 cGy. One patient had an external dose 6000 cGy to the primary site boosted with brachytherapy of 3000 cGy at the surface of an ovoid. After chemotherapy myelosuppression occurred in all patients and was tolerable. All seven patients are surviving, six disease-free, for 22 months to 12 years (median, 4 years). This study suggests that the combination of radiation therapy and chemotherapy as used here has acceptable toxicity and is effective and further suggests that children with nasopharyngeal carcinoma, even in its advanced stage at diagnosis, may be curable.