title: Ovarian Adrenal Rest Tumors Undetected by Imaging Studies and Identified at Surgery in Three Females with Congenital Adrenal Hyperplasia Unresponsive to Increased Hormone Therapy Dosage.
abstract: Patients with congenital adrenal hyperplasia have a predisposition for developing adrenal rest tumors. In contrast to testicular adrenal rest tumors, ovarian adrenal rest tumors are less common, and only a few cases have been reported in the literature. This report presents three Chinese female congenital adrenal hyperplasia patients (9 to 15 years of age) with small ectopic adrenal cortical nodules that were not detected by imaging but were diagnosed at surgery. All three patients developed virilization with elevation of 17- hydroxyprogesterone, androstenedione, and androgen levels despite receiving maximum adrenal hormone replacement therapy. Ultrasound and magnetic resonance imaging of the abdomen and pelvis suggested bilateral expansion of the adrenal glands, but no lesions of the ovaries were observed. Laparoscopy and/or laparotomy revealed small nodular lesions surrounding the pelvic gonad in all three cases. Histopathological examination of the resected tissue in all cases revealed hyperplasic nodules of cells surrounded by fibrous tissue. The cells were arranged as nests with abundant cytoplasm, which were partially lightly stained with a small centered nucleus. Immunohistochemistry staining revealed the cells to be synaptophysin positive, melan-A positive, and chromogranin A negative, indicating the cells were adrenocortical tissue and not adrenal medullary cells. Thus, the findings of the histopathological examination were consistent with ovarian adrenal rest tumors. Female congenital adrenal hyperplasia patients with virilization who showed an inadequate response to hormone therapy and had negative imaging results may benefit from laparoscopic examination or laparotomy in order to confirm the diagnosis of ovarian adrenal rest tumors while receiving unilateral subtotal adrenalectomy or subtotal bilateral adrenalectomy.
authors.affiliation: Pediatric Surgery Department of the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
authors.forename: Hua-Dong
authors.initials: HD
authors.lastname: Chen
authors.affiliation: Pediatric Surgery Department of the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
authors.forename: Li-E
authors.initials: LE
authors.lastname: Huang
authors.affiliation: Pediatric Surgery Department of the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
authors.forename: Zhi-Hai
authors.initials: ZH
authors.lastname: Zhong
authors.affiliation: Pediatric Department of the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
authors.forename: Zhe
authors.initials: Z
authors.lastname: Su
authors.affiliation: Pediatric Surgery Department of the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
authors.forename: Hong
authors.initials: H
authors.lastname: Jiang
authors.affiliation: Department of Pathology, Sun Yat-sen University Cancer Center, Guangzhou, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Zeng
authors.affiliation: Pediatric Surgery Department of the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China. liujcgd@gmail.com.
authors.forename: Jun-Cheng
authors.initials: JC
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000188:drug therapy; D000314:Adrenal Rest Tumor / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D044466:Asian People; D002648:Child; D005260:Female; D020249:Hormone Replacement Therapy; D006801:Humans; D010051:Ovarian Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery
keywords: Adrenal rest tumor; Congenital adrenal hyperplasia; Laparoscopy; Ovarian adrenal rest tumor