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CASE REPORT
Korean J Pediatr 2011 March;54(3) :137-140.
doi:https://doi.org/10.3345/kjp.2011.54.3.137
Testicular adrenal rest tumors in a patient with untreated congenital adrenal hyperplasia
Hye Young Jin (Jin HY)1, Jin Ho Choi (Choi JH)1, Gu Hwan Kim (Kim GH)2, Chung Sik Lee (Lee CS)3, Han Wook Yoo (Yoo HW)
Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, Medical Genetics Clinic and Laboratory, Asan Medical Center Childrens Hospital, University of Ulsan College of Medicine, Seoul, Korea
1Division of Pediatric Endocrinology and Metabolism, Asan Medical Center Childrens Hospital, niversity of Ulsan College of Medicine, Seoul, Korea
2Department of Pediatrics, Medical Genetics Clinic and Laboratory, Asan Medical Center Childrens Hospital, University of Ulsan College of Medicine, Seoul, Korea
3Department of Pathology3, Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon, Korea
Corresponding Author: Jin Ho Choi ,Tel: +82.2-3010-3388, Fax: +82.2-473-3725, Email: jhc@amc.seoul.kr
Copyright © 2011 by The Korean Pediatric Society
ABSTRACT
Testicular adrenal rest tumors (TARTs) are considered to be formed from aberrant adrenal tissue that has become hyperplastic because of elevated adrenocorticotropic hormone (ACTH) in male patients with congenital adrenal hyperplasia (CAH). A 6-year-old boy presented with testicular enlargement and pubic hair. He was diagnosed with CAH complicated by precocious puberty. However, he was not followed-up. At the age of 17, he visited the outpatient clinic because of testicular enlargement and short stature. His right and left testicles were 106 cm and 7.54.5 cm, respectively. His height was 155.1 cm (standard deviation score [SDS], -2.90). The diagnosis of CAH due to 21 hydroxylase deficiency was confirmed by mutation analysis of CYP21A2. Histological examination of the testes showed large, polygonal, eosinophilic cells with round nuclei and prominent nucleoli, which were suggestive of TARTs. He was treated with dexamethasone for 3 weeks and tumors regressed. Subsequently, dexamethasone was replaced by prednisolone and 9-fludrocortisone; thereafter, the reduced testis size has been maintained.
Keywords: Congenital adrenal hyperplasia | Testicular adrenal rest tumors | Precocious puberty | Child
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