Abstract

Five cases of functioning adrenocortical carcinoma in children aged 8 mo to 11 yr have been treated in Manchester since 1953. Their endocrine signs and symptoms were those of either Cushing's syndrome or virilization or both. Differentiation of these patients from those with adrenocortical carcinoma was best accomplished by means of measurement of the 24-hr urinary steroid excretion and its response to dexamethazone infusion as well as by the use of an intravenous pyelogram and an angiogram. All children were treated by surgical removal of the tumor and postoperative irradiation with careful management of their steroid balance before, during, and after operation. Four of the five are living and free of recurrence for from 1 to 12 yr after operation.

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