Korean J Pediatr.
2011 Mar;54(3):137-140. 10.3345/kjp.2011.54.3.137.
Testicular adrenal rest tumors in a patient with untreated congenital adrenal hyperplasia
- Affiliations
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- 1Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. jhc@amc.seoul.kr
- 2Department of Pediatrics, Medical Genetics Clinic and Laboratory, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
- 3Department of Pathology, Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon, Korea.
- KMID: 2284380
- DOI: http://doi.org/10.3345/kjp.2011.54.3.137
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 10x6 cm and 7.5x4.5 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 9alpha-fludrocortisone; thereafter, the reduced testis size has been maintained.
MeSH Terms
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Adrenal Hyperplasia, Congenital
Adrenal Rest Tumor
Adrenocorticotropic Hormone
Ambulatory Care Facilities
Child
Dexamethasone
Eosinophils
Hair
Humans
Male
Prednisolone
Puberty, Precocious
Steroid 21-Hydroxylase
Testis
Adrenal Hyperplasia, Congenital
Adrenocorticotropic Hormone
Dexamethasone
Prednisolone
Steroid 21-Hydroxylase
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