Adrenocortical tumors in children.

Adrenocortical tumors are very rare in children. The records of seven patients (four boys and three girls) who attended Srinagarind Hospital between January 1986 and September 2000 were retrospectively reviewed. Virilization and hypertension were found in four patients, two of whom had untreated congenital adrenal hyperplasia. Hypertension or virilization was the single manifestation found in two other patients. Only one patient showed clinical symptoms of Cushing's syndrome and another nonfunctioning tumor in Down's syndrome. Abdominal ultrasonography was helpful in locating the tumors. Unilateral tumors were found in all of the patients and surgical exploration was done in six of them. Pathological examination revealed four adrenocortical carcinomas, one adrenal gland hyperplasia and one lipoma. The two patients in which the cancer metastasized to the liver and lungs died 1 and 1 1/2 months after diagnosis, respectively. The authors concluded that virilization and hypertension remain the primary diagnostic symptoms of adrenocortical tumors in children. Early detection and adrenalectomies prolonged the survival time in these patients.

Insulinoma in childhood.

A 9-year-old boy with convulsions is herein described. He was diagnosed and treated for epilepsy and insufficient adrenal function for four years with no response. Hypoglycemia from hyperinsulinism was found and the source of the hyperinsulinism was a tumor of the tail of the pancreas--located by computerized tomographic scan and magnetic resonance imaging. Distal pancreatectomy was performed with good results. Histology of the tumor showed islet cell tumors with capsular invasion. For this type of patient, long-term follow-up should include: prevention of metastasis or recurrence, and testing for multiple endocrine neoplasia type 1.