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 T1-2
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 (T1-2, N1-2),
radiotherapy alone (55-60 Gy) appears to be sufficient for disease control.(ABSTRACT TRUNCATED AT 400 WORDS)