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Adrenocortical Thmors in Children

OUYPORN PANAMONTA, M.D.*, SUCHAT AREEMIT, M.D.**, JIRAPORN SRINAKARIN, M.D.***, SARITHRON SIRITUNYAPORN, M.D.****, SUKANYA TUKSAPUN, M.D.*

Affiliation : *Department of Pediatrics, **Department of Surgery, ***Department of Radiology, ****Department of Pathology, Faculty of Medicine, Srinagarind Hospital, Khan Kaen University, Khan Kaen 40002, Thailand.

Abstract
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.

Keywords : Adrenal Gland Tumor, Adrenocortical Carcinoma, Adrenocortical Tumor, Congenital Adrenal Hyperplasia, Virilization, Hypertension, Cushing's Syndrome


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