Clinical and research progress of congenital adrenal hyperplasia with adrenal rest tumor

VernacularTitle: 先天性肾上腺皮质增生症伴肾上腺残余肿瘤的临床及研究进展
Author: Lingli XIE ¹ ; Qing ZHANG ² ; Wenfang XIA ¹
Author Information

1. Department of Endocrinology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China
2. Department of Endocrinology, the Third People′s Hospital of Hubei Province, Wuhan 430032, China
Publication Type:Review
Keywords: Congenital adrenal hyperplasia; Adrenal rest tumor; Steroid 21-hydroxylase; Testicular; Ovarian
From: Chinese Journal of Endocrinology and Metabolism 2019;35(9):795-800
CountryChina
Language:Chinese
Abstract: Adrenal rest tumor (ART) is a well-known complication in congenital adrenal hyperplasia (CAH), which may occur in all age groups, especially in adolescence and adulthood. Tumors that occur in the testicular known as testicularadrenal rest tumor (TART), in the ovarian and parovarian tissue known as ovarian adrenal rest tumor (OART). ART is one of the major causes of reproductive dysfunction in adult with CAH. Early prevention and treatment is the key to avoid irreversible damage to gonadal function. In the past, we paid more attention to TART in children, while ignored TART and OART in adults. This article will review the origin of tumor cells, pathogenesis, epidemiology, diagnosis, treatment and prognosis of CAH associated with ART, which aims to guide clinical work through the profound understanding of CAH with ART.