Sertoli-Leydig cell tumor associated with a germline DICER1 pathogenic variant diagnosed during pregnancy: Considerations for treatment, surveillance, and prevention

Joyce Y Wang; Kimberly K Ma; Daniel J Reiter; Ana Torvie; Elizabeth M Swisher

doi:10.1016/j.gore.2023.101215

Sertoli-Leydig cell tumor associated with a germline DICER1 pathogenic variant diagnosed during pregnancy: Considerations for treatment, surveillance, and prevention

Gynecol Oncol Rep. 2023 Jun 2:48:101215. doi: 10.1016/j.gore.2023.101215. eCollection 2023 Aug.

Authors

Joyce Y Wang¹, Kimberly K Ma¹, Daniel J Reiter², Ana Torvie³, Elizabeth M Swisher^{1

4}

Affiliations

¹ Department of Obstetrics & Gynecology, University of Washington, Seattle, WA, United States.
² Department of Pathology, University of Washington, Seattle, WA, United States.
³ Northwest Women's HealthCare, Seattle, WA, United States.
⁴ Fred Hutchinson Cancer Center, Seattle, WA, United States.

Abstract

•This is the first report of a germline DICER1-associated Sertoli-Leydig cell tumor (SLCT) diagnosed in pregnancy.•SLCT is linked to DICER1 pathogenic variants, but little is known about management of DICER1-associated SLCT.•There is an extended risk for metachronous SLCT in patients with germline DICER1 pathogenic variants who retain an ovary.•Prophylactic contralateral salpingo-oophorectomy may be offered with shared decision making to patients with inherited SLCT.•Genetic testing for DICER1 should be offered to all patients with moderately or poorly differentiated SLCT.

Keywords: DICER1; Genetic testing; Metachronous tumor; Post-treatment surveillance; Pregnancy; Sertoli-Leydig cell tumor.

Publication types

Case Reports