Primary ovarian leiomyosarcoma: results from an analysis by the French Sarcoma Group (Ovarian SArcoma MAnagement - OSAMA Study)
- Domenico Ferraioli 1, Fulvio Borella 2, Marie Meurer 3, Coriolan Lebreton 4, Nicolas Penel 5, Francois Bertucci 6, Emmanuelle Bompas 7, Antoine Italiano 4, Nelly Firmin 8, Luca Bertero 9, Isabelle Peyrottes 10, Julien Mancini 11, Nicolas Isambert 12, Christine Chevreau 13, Gwenael Ferron 14, Cristophe Perrin 15, Sophie Piperno-Neumann 16, Stefano Cosma 2, Patricia Pautier 17, Cécile Loaec 18, Stephanie Aust 19, Nicolas Chopin 1, Pierre Meeus 1, Jean-Yves Blay 19, Jeremy Gonin 16, Florence Duffaud 3, Pierre-Emmanuel Colombo 20, Isabelle Ray-Coquard 19
- 1Centre Leon Berard and Claude Bernard University, Department of Surgical Oncology, Lyon, France.
- 2University of Turin, Sant'Anna Hospital, "Città della Salute e della Scienza," Department of Surgical Sciences, Turin, Italy; University of Turin, Sant'Anna Hospital, "Città della Salute e della Scienza," Gynecology and Obstetrics 1U, Turin, Italy.
- 3AP-HM and Aix-Marseille University, CHU Timone, Department of Medical Oncology, Marseille, France.
- 4Institut Bergonié, Department of Medical Oncology, Bordeaux, France.
- 5Oscar Lambret Center, Department of General Cancerology, Lille, France.
- 6Institut Paoli-Calmettes, Department of Medical Oncology, Marseille, France.
- 7Institut de Cancérologie de l'Ouest, Department of Medical Oncology, Angers-Nantes, France.
- 8ICM, Department of Medical Oncology, Montpellier, France.
- 9University of Turin and City of Health and Science University Hospital, Department of Medical Sciences, Pathology Unit, Turin, Italy.
- 10Antoine Lacassagne Center, Department of Medical Oncology, Nice, France.
- 11Aix-Marseille Univ, INSERM, IRD, UMR 1252, SESSTIM, Cancers, Biomedicine ans Society group, APHM, Timone Hospital, Public Health Department (BIOSTIC), Marseille, France.
- 12Georges-François Leclerc Center, Department of Medical Oncology, Dijon, France.
- 13IUCT, Department of Medical Oncology, Toulouse, France.
- 14IUCT, Department of Surgical Oncology, Toulouse, France.
- 15Eugene Marquis Center, Department of Medical Oncology, Rennes, France.
- 16Institut Curie, Paris, France.
- 17Gustave Roussy, Department of Medicine, Gynecology Unit, Villejuif, France.
- 18Boulevard Professor Monod, Institut de Cancerologie de l'Ouest, Department of Surgery, Saint Herblain, France.
- 19Centre Leon Berard, and Claude Bernard University, Department of Adult Medical Oncology, Lyon, France.
- 20Val d'Aurelle, Institut régional du Cancer de Montpellier, Montpellier, France.
- 0Centre Leon Berard and Claude Bernard University, Department of Surgical Oncology, Lyon, France.
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March 5, 2025
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View abstract on PubMed
Summary
This summary is machine-generated.Primary ovarian leiomyosarcomas are rare tumors with poor prognosis. Surgery is key for early stages, but adjuvant treatment roles are unclear, and expert referral is recommended.
Area Of Science
- Gynecologic Oncology
- Surgical Pathology
- Medical Oncology
Background
- Primary ovarian leiomyosarcomas are rare, aggressive tumors, often diagnosed late.
- Limited data exists on optimal management and prognostic factors for these rare ovarian neoplasms.
Purpose Of The Study
- To evaluate clinical, surgical, and pathologic characteristics of primary ovarian leiomyosarcomas.
- To assess the clinical outcomes and survival of patients diagnosed with this rare malignancy.
Main Methods
- Retrospective study of 39 patients with primary ovarian leiomyosarcomas.
- Data collected from 18 French Sarcoma Group centers via the national database (NetSarc).
Main Results
- High mitotic counts and progesterone receptor negativity were associated with worse survival in early-stage disease.
- Surgery is the primary treatment; adjuvant chemotherapy (35%) and radiotherapy (23%) roles are uncertain.
- 44% of patients died of disease, with recurrence patterns including pelvic and distant metastases.
Conclusions
- Surgery is the cornerstone for early-stage ovarian leiomyosarcoma.
- Pathologic features like high mitotic counts and progesterone receptor status impact survival.
- Referral to expert sarcoma centers is crucial due to the rarity and complexity of managing ovarian leiomyosarcomas.
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