A Case of Adrenocortical Tumor in a 16-Month-Old Boy Presenting with Virilization.

Kwak,  Min Jung; Hyun,  Hye Sun; Kim,  Su Jin; Paik,  Kyung Hoon; Jin,  Dong Kyu

Ann Pediatr Endocrinol Metab > Volume 13(1); 2008 > Article

Case Report

Journal of Korean Society of Pediatric Endocrinology 2008;13(1): 110-116.

A Case of Adrenocortical Tumor in a 16-Month-Old Boy Presenting with Virilization.

Min Jung Kwak, Hye Sun Hyun, Su Jin Kim, Kyung Hoon Paik, Dong Kyu Jin

Department of Pediatrics, Samsung Medical Center, School of Medicine Sungkyunkwan University, Seoul, Korea. jindk@skku.edu

Abstract

A 16-month-old boy had signs of virilization such as penile enlargement and pubic hair since 13 months of age. Hormone evaluation indicated increased levels of 17-OH-progesterone, dehydroepiandrosterone- sulfate (DHEA-S) and testosterone. Initially he was diagnosed as simple virilizing type of congenital adrenal hyperplasia and hydrocortisone was given to him. After 2 months, his penile enlargement continued and we did not find gene mutation for 21-hydroxylase deficiency. Adrenal magnetic resonance imaging revealed a 3.5x2.7x4.4 cm size of adrenal mass consistent with an adrenocortical tumor. The patient underwent surgical excision of the well-encapsulated tumor with normalization of his hormone levels. Childhood adrenocortical tumors are rare and are not well characterized. Surgery is the definitive treatment for adrenocortical tumors and prognosis is often poor. We report a case of adrenocortical tumor in a 16-month-old boy presenting with virilization with review of literature.

Keywords: Adrenal cortex neoplasms;Virilism