ADRENAL-CORTICAL TUMORS IN CHILDHOOD

In a 41-year period 28 children with a diagnosis of an adrenal cortica l tumor were identified (14 carcinoma : 4 adenoma). The majority of pa tients had clinical signs of endocrine dysfunction at presentation wit h virilization (11 patients) and a cushingoid appearance (8 patients) the commonest findings. Abnormal biochemical activity was identified i n 16 tumors (94 %). The primary treatment in 17 patients was surgical. In addition, 12 children, all with carcinomas, had radiotherapy. Of t hose children with a carcinoma, 12 are dead with a median survival of 52 months (range 1-317 months). The three second primary tumors all de veloped at sites within the field of previous radiotherapy, and proved fatal at 127, 176 and 317 months (median 207 months). This series con firms the poor prognosis in adrenocortical carcinoma in childhood but a complete resection is compatible with cure of the primary disease. T he frequency of second, fatal, primary tumors is of particular concern and long-term follow-up is mandatory in survivors, especially if radi otherapy was part of the treatment protocol.