Available online 17 November 2022

: DICER1 mutation has been linked to development of Sertoli-Leydig cell tumor and cystic nephroma, among other neoplasms.

: We present a unique case of recurrent ovarian Sertoli-Leydig cell tumor in a pediatric patient with a known DICER1 mutation and history of cystic nephroma. She underwent surgical staging and adjuvant chemotherapy and her recurrences have been treated with chemotherapy, whole abdomen radiation therapy, and further surgical debulking.

: This report adds to the small body of evidence about this rare but unexpectedly highly aggressive tumor, especially in the recurrent setting, and reminds the reader of the importance of cancer diagnosis in this population.

Recurrence

Sertoli-Leydig cell tumor

DICER1 mutation

sex cord-stromal tumor

adolescent

case report

© 2022 Published by Elsevier Inc. on behalf of North American Society for Pediatric and Adolescent Gynecology.