Pediatric nasopharyngeal carcinoma: Long term follow-up of 29 patients

Twenty-nine untreated children diagnosed with nasopharyngeal carcinoma were consecutively admitted to St. Jude Children's Research Hospital from 1962 to 1986. The age of the patients ranged from 6 to 19 years (median of 13) at diagnosis. Histologically, all had lymphoepithelioma. Patients were retrospectively staged in the American Joint Committee System. Disease extent was T₁ (n = 5), T₂ (n = 7), T3 (n = 9), T₄ (n = 8); N₀ (n = 1), N₂ (n = 7), N₃ (n = 21). Two patients had distant metastasis (M₁) on admission, both ultimately succumbed to their disease. Twenty-seven patients were seen initially without metastatic disease: one received pre-irradiation vincristine, 17 were treated with concomitant radiotherapy and cyclophosphamide. From 1981 to the present, four patients received pre-irradiation and one received post-irradiation cisplatin-bleomycin, vinblastine (CDDP-BLEO-VLB) regimens. Four patients received radiotherapy alone. All patients completed chemotherapy and radiation therapy. Twenty-five patients had complete tumor clearance and four had a partial response. Overall, 14 patients are alive continuously without relapse with a median follow-up of 11 years (range 4 to 20). All patients who relapsed did so within 2 years postirradiation. Four patients failed locally-all had advanced (T₃-T₄) local disease at presentation and three of the failures were at the margin of treatment portals. Thirteen patients failed with distant metastasis. The major prognostic factor in these patients was the local extent of disease. Among the 27 M₀ patients, all ten patients with T_1-2 tumors are disease-free, whereas four of nine patients with T3 and two of eight patients with T₄ tumors are alive and well. In the 16 patients who are long term survivors, eight have mild neck atrophy, two have shortening of the clavicles; except for one patient who required a neck brace for shoulder drop, all had normal function. Among the seven pre-pubertal patients who are long term survivors, three have decreased growth, including one with documented decreased growth hormone. Two patients developed irregular menstrual periods. One patient developed hypothyroidism, and another had a thyroid adenoma. One patient developed bleomycin pneumonitis and one patient who received pre- and post-irradiation chemotherapy died of laryngeal edema and fibrosis, in remission. Radiotherapy is the major modality in the therapy of childhood nasopharyngeal carcinoma. The long term toxicity of radiotherapy plus or minus chemotherapy is acceptable. In early stage tumors (T_1-2, N_1-2), radiotherapy alone (55-60 Gy) appears to be sufficient for disease control. The more advanced stage tumors require aggressive chemotherapy, since distant failure is the major problem.