TY - JOUR
T1 - Outcomes in ovarian Sertoli-Leydig cell tumor
T2 - A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries
AU - Nelson, Alexander T.
AU - Harris, Anne K.
AU - Watson, Dave
AU - Kamihara, Junne
AU - Chen, Kenneth S.
AU - Stall, Jennifer N.
AU - Devins, Kyle M.
AU - Young, Robert H.
AU - Olson, Damon R.
AU - Mallinger, Paige H.R.
AU - Mitchell, Sarah G.
AU - Hoffman, Lindsey M.
AU - Halliday, Gail
AU - Suleymanova, Amina M.
AU - Glade Bender, Julia L.
AU - Messinger, Yoav H.
AU - Herzog, Cynthia E.
AU - Field, Amanda L.
AU - Frazier, A. Lindsay
AU - Stewart, Douglas R.
AU - Dehner, Louis P.
AU - Hill, D. Ashley
AU - Billmire, Deborah F.
AU - Schneider, Dominik T.
AU - Schultz, Kris Ann P.
N1 - Publisher Copyright:
© 2024 Elsevier Inc.
PY - 2024/7
Y1 - 2024/7
N2 - 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.
AB - 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.
KW - DICER1
KW - DICER1-related tumor predisposition
KW - Gynandroblastoma
KW - Sertoli-Leydig cell tumor
KW - Sex cord-stromal tumor
UR - http://www.scopus.com/inward/record.url?scp=85190805636&partnerID=8YFLogxK
U2 - 10.1016/j.ygyno.2024.04.005
DO - 10.1016/j.ygyno.2024.04.005
M3 - Article
C2 - 38657450
AN - SCOPUS:85190805636
SN - 0090-8258
VL - 186
SP - 117
EP - 125
JO - Gynecologic oncology
JF - Gynecologic oncology
ER -