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

• 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.