Characterization of ovarian adrenal rest tumors in children and adolescent females with congenital adrenal hyperplasia due to 21-hydroxylase deficiency
10.3760/cma.j.issn.0578-1310.2016.06.005
- VernacularTitle:先天性肾上腺皮质增生症21-羟化酶缺陷患者并发卵巢肾上腺残余瘤的临床特征分析
- Author:
Zhe SU
1
;
Yanhong LI
;
Huamei MA
;
Jun ZHANG
;
Minlian DU
Author Information
1. 深圳市儿童医院内分泌科
- Keywords:
Adrenal hyperplasia,congenial;
Steroid 21-hydroxylase;
Ovary;
Adrenal rest tumor
- From:
Chinese Journal of Pediatrics
2016;54(6):414-418
- CountryChina
- Language:Chinese
-
Abstract:
Objective To summarize the characterization of ovarian adrenal rest tumors (OART) in children and adolescent females with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD).Method We have diagnosed 4 cases of CAH 21-OHD with OART in the recent 5 years in pediatric endocrinology unit of the first affiliated hospital of Sun Yat-Sen University.Clinical characterizations were summarized,including symptoms,growth patterns and bone age advancements,serum steroid concentrations,imaging results and the follow-up data.Result The 4 cases of OART accounted for 2.5 ％ of the CAH 21-OHD girls,which was much lower than the detection rate,29.5％,of testicular ART in our patient group during the same period.The 4 cases included 3 salt wasters and 1 with simple virilizm.OART were diagnosed at the age of 8.9 years,15.8 years,21.4 years and 9.3 years,respectively.Before the diagnosis of OART,their daily hydrocortisone doses reached 16 mg/(m2 · d)to 24 mg/(m2 · d).The patients presented with deteriorated acne,deepened voice,amenorrhea (2 cases),accelerated growth and advanced bone age,△BA/△CA reached 1.2 (during GnRHa treatment) and 2.0.Their serum adrenal steroids increased significantly and hyperplasia of adrenal glands were found in all patients.The diagnosis could be confirmed before the operation in only 1 case.The diagnosis could not be made until the planned sub-total adrenalectomy in the other 3 cases.The follow-up periods of OART were 4.8,4.7,3.8and 2.7years,respectively.Removal of OART resulted in symptoms relief at least partialy and lowered daily hydrocortisone doses,14 to 19 mg/(m2 · d).Conclusion The detection rate of OART was much lower than testicular ART.Before the diagnosis of OART,the patients presented with deteriorated virilescence,accelerated growth and advanced bone age,elevated serum adrenal steroids and hyperplasia of adrenal glands.Removal of the OART resulted in symptoms relief at least partialy.
