Extraovarian sex cord tumor with annular tubules discovered arising from a leiomyoma

This is the first extraovarian SCTAT in the published literature arising from a leiomyoma. Our patient had no family history and displayed no syndromic features for Peutz-Jeghers Syndrome. Ultimately, she declined genetic testing. The lack of evidence of ovarian involvement on both imaging and on intraoperative examination made localization to either ovary impossible. The patient is currently being managed with surveillance since the morbidity associated with bilateral oophorectomy in the 4th decade of life exceeds the theoretical risk of SCTAT.