Case report: an identical twin with Sertoli-Leydig cell tumor. (3rd July 2018)

• Record Type: Journal Article
• Authors: Cho, Kristy; Havelock, Jon C.; Gilks, Blake; Dunne, Caitlin
• Is Part Of: Gynecological endocrinology. Volume 34:Number 7(2018)
• Page Start: 563
• Page End: 566
• Publication Date: 2018-07-03
• Subjects: Sertoli-Leydig cell tumor -- ovarian neoplasms -- sex cord-gonadal stromal tumor -- testosterone -- DICER; Endocrine gynecology -- Periodicals; Generative organs, Female -- Diseases -- Periodicals; 618.1
• DOI: 10.1080/09513590.2018.1431215
• Languages: English
• ISSNs: 0951-3590

Abstract

Abstract: Our report details the workup and management of a 43-year-old woman with an identical twin who presented with 2 years of virilization and secondary amenorrhea. Serum total testosterone was elevated. An MRI did not identify adnexal or adrenal pathology. Subsequent ovarian vein sampling demonstrated unilateral testosterone elevation. The patient underwent laparoscopic unilateral oophorectomy resulting in the diagnosis of Sertoli-Leydig cell tumor (SLCT). Although SLCT is a rare sex-cord ovarian tumor, it is associated with endometrial hyperplasia and malignancy. Our goals are to review the workup of androgen-secreting tumors and discuss the clinical importance of the DICER1 mutation in the context of SLCT. In this case, an identical twin underwent DICER1 testing which was one of the essential steps in her clinical management.