Adrenal cortical tumors in childhood

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