Outcomes in ovarian Sertoli-Leydig cell tumor: A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries Journal Article
| Authors: | Nelson, A. T.; Harris, A. K.; Watson, D.; Kamihara, J.; Chen, K. S.; Stall, J. N.; Devins, K. M.; Young, R. H.; Olson, D. R.; Mallinger, P. H. R.; Mitchell, S. G.; Hoffman, L. M.; Halliday, G.; Suleymanova, A. M.; Glade Bender, J. L.; Messinger, Y. H.; Herzog, C. E.; Field, A. L.; Frazier, A. L.; Stewart, D. R.; Dehner, L. P.; Hill, D. A.; Billmire, D. F.; Schneider, D. T.; Schultz, K. A. P. |
| Article Title: | Outcomes in ovarian Sertoli-Leydig cell tumor: A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries |
| 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. © 2024 Elsevier Inc. |
| Keywords: | adolescent; adult; child; human tissue; aged; middle aged; unclassified drug; major clinical study; overall survival; histopathology; cancer recurrence; cisplatin; paclitaxel; cancer radiotherapy; cancer staging; outcome assessment; recurrence risk; follow up; gene; cancer susceptibility; carboplatin; etoposide; tumor differentiation; cohort analysis; genetic variability; alkylating agent; cyclophosphamide; vincristine; risk factor; cancer mortality; ifosfamide; high risk patient; germ line; infant; adjuvant chemotherapy; newborn; medical record; dactinomycin; bleomycin; cancer registry; platinum derivative; hazard ratio; rhabdomyosarcoma; mesenchyme; tissue differentiation; ribonuclease iii; recurrence free survival; rupture; clinical outcome; international federation of gynecology and obstetrics; cancer prognosis; asymptomatic disease; androblastoma; human; female; article; dicer1; dicer1 gene; sertoli-leydig cell tumor; sex cord-stromal tumor; ovarian sex cord stromal tumor; dicer1-related tumor predisposition; gynandroblastoma; ribonuclease iiib; malignant sex cord stromal tumor of ovary; mesenchymal heterologous element |
| Journal Title: | Gynecologic Oncology |
| Volume: | 186 |
| ISSN: | 0090-8258 |
| Publisher: | Elsevier Inc. |
| Date Published: | 2024-07-01 |
| Start Page: | 117 |
| End Page: | 125 |
| Language: | English |
| DOI: | 10.1016/j.ygyno.2024.04.005 |
| PROVIDER: | scopus |
| PUBMED: | 38657450 |
| PMCID: | PMC11216876 |
| DOI/URL: | |
| Notes: | Article -- Source: Scopus |
