Jin Hye Young, Choi Jin Ho, Kim Gu Hwan, Lee Chung Sik, Yoo Han Wook
Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
Korean J Pediatr. 2011 Mar;54(3):137-40. doi: 10.3345/kjp.2011.54.3.137. Epub 2011 Mar 31.
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 10×6 cm and 7.5×4.5 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 9α-fludrocortisone; thereafter, the reduced testis size has been maintained.
睾丸肾上腺残余肿瘤（TARTs）被认为是由先天性肾上腺皮质增生症（CAH）男性患者中因促肾上腺皮质激素（ACTH）升高而增生的异常肾上腺组织形成的。一名6岁男孩出现睾丸肿大和阴毛。他被诊断为CAH合并性早熟。然而，他没有接受随访。17岁时，他因睾丸肿大和身材矮小到门诊就诊。他的右侧和左侧睾丸分别为10×6厘米和7.5×4.5厘米。他的身高为155.1厘米（标准差评分[SDS]，-2.90）。通过CYP21A2的突变分析证实了因21羟化酶缺乏导致的CAH诊断。睾丸组织学检查显示有大的、多边形的、嗜酸性细胞，细胞核圆形，核仁突出，提示为TARTs。他接受了3周的地塞米松治疗，肿瘤消退。随后，地塞米松被泼尼松龙和9α-氟氢可的松替代；此后，睾丸缩小后的大小得以维持。
