A Case of adrenocortical oncocytoma Presenting with Peripheral Precocious Puberty.

Oh,  Seong Hee; Jung,  Chang Woo; Lee,  Jin; Lee,  Beom Hee; Kim,  Dae Yeon; No,  Dae Yoon; Yoo,  Soo Hyun; Choi,  Jin Ho; Yoo,  Han Wook

doi:2012.17.1.49

Ann Pediatr Endocrinol Metab > Volume 17(1); 2012 > Article

Case Report

Annals of Pediatric Endocrinology & Metabolism 2012;17(1): 49-52.

DOI: https://doi.org/10.6065/apem.2012.17.1.49

A Case of adrenocortical oncocytoma Presenting with Peripheral Precocious Puberty.

Seong Hee Oh, Chang Woo Jung, Jin Lee, Beom Hee Lee, Dae Yeon Kim, Dae Yoon No, Soo Hyun Yoo, Jin Ho Choi, Han Wook Yoo

¹Department of Pediatrics, Asan Medical Center Children's hospital, University of Ulsan College of Medicine, Seoul, Korea. hwyoo@amc.seoul.kr
²Department of Surgery, Asan Medical Center Children's hospital, University of Ulsan College of Medicine, Seoul, Korea.
³Department of Pathology, Asan Medical Center Children's hospital, University of Ulsan College of Medicine, Seoul, Korea.

Abstract

Adrenocortical oncocytoma is a rare adrenal gland tumor, and in most cases, benign and non-functioning. Functional adrenocortical oncocytoma has only been reported in eleven cases worldwide, including three children. These cases all showed clinical symptoms and signs, such as virilization, feminization, Cushing syndrome and Conn syndrome, as a result of overproduction of adrenal steroid hormones. We report a 2-year-old girl who presented with precocious puberty with premature pubarche. Dehydroepiandrosterone sulfate (DHEA-S) and testosterone levels were elevated and a 1.9 cm right adrenocortical oncocytoma was found. After tumor resection, her hormone levels were normalized.

Keywords: Adrenocortical;Oncocytoma;Puberty, Precocious