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. 2024 Jul:186:117-125.
doi: 10.1016/j.ygyno.2024.04.005. Epub 2024 Apr 23.

Outcomes in ovarian Sertoli-Leydig cell tumor: A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries

Alexander T Nelson  1 , Anne K Harris  2 , Dave Watson  3 , Junne Kamihara  4 , Kenneth S Chen  5 , Jennifer N Stall  6 , Kyle M Devins  7 , Robert H Young  7 , Damon R Olson  8 , Paige H R Mallinger  2 , Sarah G Mitchell  9 , Lindsey M Hoffman  10 , Gail Halliday  11 , Amina M Suleymanova  12 , Julia L Glade Bender  13 , Yoav H Messinger  2 , Cynthia E Herzog  14 , Amanda L Field  15 , A Lindsay Frazier  4 , Douglas R Stewart  16 , Louis P Dehner  17 , D Ashley Hill  18 , Deborah F Billmire  19 , Dominik T Schneider  20 , Kris Ann P Schultz  21
Affiliations

Outcomes in ovarian Sertoli-Leydig cell tumor: A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries

Alexander T Nelson et al. Gynecol Oncol. 2024 Jul.

Abstract

Objective: Sertoli-Leydig cell tumors (SLCTs) are rare sex cord-stromal tumors, representing <0.5% of all ovarian tumors. We sought to describe prognostic factors, treatment and outcomes for individuals with ovarian SLCT.

Methods: Individuals with SLCT were enrolled in the International Pleuropulmonary Blastoma/DICER1 Registry and/or the International Ovarian and Testicular Stromal Tumor Registry. Medical records were systematically abstracted, and pathology was centrally reviewed when available.

Results: In total, 191 participants with ovarian SLCT enrolled, with most (92%, 175/191) presenting with FIGO stage I disease. Germline DICER1 results were available for 156 patients; of these 58% had a pathogenic or likely pathogenic germline variant. Somatic (tumor) DICER1 testing showed RNase IIIb hotspot variants in 97% (88/91) of intermediately and poorly differentiated tumors. Adjuvant chemotherapy was administered in 40% (77/191) of cases, and among these, nearly all patients received platinum-based regimens (95%, 73/77), and 30% (23/77) received regimens that included an alkylating agent. Three-year recurrence-free survival for patients with stage IA tumors was 93.6% (95% CI: 88.2-99.3%) compared to 67.1% (95% CI: 55.2-81.6%) for all stage IC and 60.6% (95% CI: 40.3-91.0%) for stage II-IV (p < .001) tumors. Among patients with FIGO stage I tumors, those with mesenchymal heterologous elements treated with surgery alone were at higher risk for recurrence (HR: 74.18, 95% CI: 17.99-305.85).

Conclusion: Most individuals with SLCT fare well, though specific risk factors such as mesenchymal heterologous elements are associated with poor prognosis. We also highlight the role of DICER1 surveillance in early detection of SLCT, facilitating stage IA resection.

Keywords: DICER1; DICER1-related tumor predisposition; Gynandroblastoma; Sertoli-Leydig cell tumor; Sex cord-stromal tumor.

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Conflict of interest statement

Declaration of competing interest Dr. Hill is owner of ResourcePath LLC, a company which does research and development of laboratory tests including for DICER1 cancers. That work is unrelated to the information presented in this article. Dr. Stewart provides telegenetics services for Genome Medical, Inc., in accordance with relevant National Cancer Institute policies. The remaining authors have no conflicts to disclose.

Figures

Figure 1:
Figure 1:
A. SLCT recurrence-free survival and B. overall survival stratified by FIGO stage IA (blue), IC (yellow) and II-IV (grey) with 95% confidence interval shading included. Well-differentiated tumors excluded from analysis.
Figure 2:
Figure 2:
A. SLCT recurrence-free survival and B. overall survival by level of differentiation; well-differentiated (blue), intermediately differentiated (yellow), and poorly differentiated (grey) with 95% confidence interval shading included. *p-values exclude well-differentiated.
Figure 3:
Figure 3:
SLCT recurrence-free (RFS) and overall survival (OS) stratified by presence of mesenchymal heterologous elements (red) and no mesenchymal heterologous elements (blue) with 95% confidence interval shading included. A) SLCT RFS and B) OS among patients with FIGO stage I disease who received chemotherapy. C) SLCT RFS and D) OS among patients with FIGO stage I disease who did not receive chemotherapy. E) SLCT RFS and F) OS among patients with FIGO stage IA disease.
See this image and copyright information in PMC

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