Nasopharyngeal carcinoma in children and adolescents in Tunisia: clinical aspects and the paraneoplastic syndrome.

Of 485 cases of NPC collected from the files of our institute between March 1969 and December 1974, 82 (17%) were in children and adolescents (0--19 years old). This relatively high frequency of NPC in young people was not suggested by reports from high-incidence areas (Southern China, for example) but appears to be a characteristic feature of areas of intermediate incidence (Uganda, Kenya, Sudan, Tunisia). NPC is the tumour that occurs most frequently in young people between 1 and 20 years old in Tunisia, showing a peak in those 16 years of age. The male:female sex ratio was 3:1. Advanced stages of the disease occured frequently; no clinical anomalies were noted in this age group. The 'nasopharyngeal' type of carcinoma was the histological form seen most frequently; poorly-differentiated squamous-cell carcinomas were not uncommon; and well-differentiated squamous-cell carcinomas were not seen in this series. The five-year survival rate was 32.5% for children and only 20% for adults. This can perhaps be explained by the fact that the 'nasopharyngeal' type of tumour has a better prognosis than other histological types, and, compared to adults, younger patients more frequently have distant metastases (37%) than local recurrences (18.5%). A paraneoplastic syndrome, consisting of hypertrophic pulmonary osteoarthropathy with occasional generalized lowering of pituitary function, was seen in 12 of the patients. This syndrome has been described only in children, and no cases were found in our series of adult NPC patients. Epidemiological, clinical and histological aspects suggest that NPC in young people is different from that found in adults.