Treatment of high risk Sertoli–Leydig cell tumors of the ovary using a gonadotropin releasing hormone (GnRH) analog

Harsha Prasada Lashkari,Assunta Albanese,Bruce Okoye,Robert Millar,Ruth Nash,Kathy Pritchard‐Jones

doi:10.1002/pbc.24382

Abstract

Sertoli–Leydig cell tumors are rare ovarian neoplasms. We report two unusual cases with bilateral SLCTs suggesting evidence of genetic predisposition and at high risk of recurrence. To reduce this risk, we exploited the use of GnRH analog to lower gondadotropin and potentially directly inhibit the tumors through expressed GnRH receptors. We used it as maintenance antitumor therapy for 2 years after completion of chemotherapy, to cover the period of risk for recurrence. Both patients remain in complete remission at >2 years after completing leuprorelin therapy. Of note, both patients carry DICER1 mutations, frequently found in pleuropulmonary blastoma syndrome. Pediatr Blood Cancer 2013; 60: E16–E18. © 2012 Wiley Periodicals, Inc.

Highlights

Sertoli–Leydig cell tumor (SLCT) is a type of ovarian sex cord-stromal tumors [1] which occurs most frequently in the second and third decades
We present two unusual high-risk cases where there was evidence of genetic predisposition
Chemotherapy consisting of cisplatin, ifosfamide, and etoposide (PIE) was administered as per the schedule used by the German Pediatric Oncology Group for germ cell tumor [5]

Summary

Introduction

Sertoli–Leydig cell tumor (SLCT) is a type of ovarian sex cord-stromal tumors [1] which occurs most frequently in the second and third decades. We chose a long acting GnRH analog (leuprorelin) to suppress any residual tumor cell proliferation. A left ovarian and a right para-aortic lymph node mass were detected on routine ultrasound scan.

Results

Conclusion