Sclerosing Epithelioid Fibrosarcoma (SEF) versus Low Grade Fibromyxoid Sarcoma (LGFMS): Presentation and outcome in the nationwide NETSARC+ series of 330 patients over 13 years.

Autor:	Blay JY; Centre Léon Bérard & Université Claude Bernard, Lyon, France. Electronic address: jean-yves.blay@lyon.unicancer.fr., Tlemsani C; Hopital Cochin, Paris, France., Toulmonde M; Departement of Medical Oncology, Institut Bergonié, Bordeaux, France., Italiano A; Departement of Medical Oncology, Institut Bergonié, Bordeaux, France., Rios M; Institut Cancerologie Lorraine, Centre Alexis Vautrin, Nancy, France., Bompas E; Department of Medical Oncology, Institut de Cancerologie Ouest Nantes, France., Valentin T; Institut Claudius Regaud & Institut Universitaire de Cancerologie, Oncopole, Toulouse, France., Duffaud F; La Timone University Hospital, Marseille, France., Le Nail LR; CHU Tours, 37000 Tours, France., Watson S; Dept of Medical Oncology, Institut Curie & INSERM U830, Institut Curie Research Center, Paris, France., Firmin N; Institut de Cancérologie de Montpellier, Montpellier, France., Dubray-Longeras P; Centre Jean Perrin/ERTICa EA 4677, Clermont-Ferrand, France., Ropars M; Eugene Marquis Comprehensive Cancer Center, France., Perrin C; CHU, Rennes, France., Hervieu A; Centre George Francois Leclerc, Dijon, France., Lebbe C; Centre Georges François Leclerc, Dijon, France., Saada-Bouzid E; Dermato-Oncology Unit, Saint Louis Hospital, Paris, France., Soibinet P; Centre Antoine-Lacassagne, Nice, France., Fiorenza F; Institut J Godinot Reims, France., Bertucci F; Institut Paoli-Calmettes, Marseille, France., Boudou P; Hopital Cochin, Paris, France., Vaz G; Centre Léon Bérard & Université Claude Bernard, Lyon, France., Bonvalot S; Hopital Cochin, Paris, France., Honoré C; Gustave Roussy Cancer Campus, Villejuif, France., Marec-Berard P; Centre Léon Bérard & Université Claude Bernard, Lyon, France., Minard V; Gustave Roussy Cancer Campus, Villejuif, France., Cleirec M; Dept pédiatrie, CHU Nantes, France. Electronic address: morgane.cleirec@chu-nantes.fr., Biau D; Hopital Cochin, Paris, France., Meeus P; Centre Léon Bérard & Université Claude Bernard, Lyon, France., Babinet A; Hopital Cochin, Paris, France., Dumaine V; Hopital Cochin, Paris, France., Carriere S; Institut de Cancérologie de Montpellier, Montpellier, France., Fau M; Departement of Medical Oncology, Institut Bergonié, Bordeaux, France., Decanter G; Univ. Lille, CHU Lille, ULR 2694 - Metrics: Evaluation des technologies de santé et des pratiques médicales, F-59000 Lille, France; Department of Medical Oncology, Centre Oscar Lambret, Lille, France., Gouin F; Centre Léon Bérard & Université Claude Bernard, Lyon, France., Ngo C; Departement of Medical Oncology, Institut Bergonié, Bordeaux, France., Le Loarer F; Hopital Cochin, Paris, France., Karanian M; Centre Léon Bérard & Université Claude Bernard, Lyon, France., Meurgey A; Centre Léon Bérard & Université Claude Bernard, Lyon, France., Dufresne A; Centre Léon Bérard & Université Claude Bernard, Lyon, France., Brahmi M; Centre Léon Bérard & Université Claude Bernard, Lyon, France., Chemin-Airiau C; Centre Léon Bérard & Université Claude Bernard, Lyon, France., Ducimetiere F; Centre Léon Bérard & Université Claude Bernard, Lyon, France. Electronic address: francoise.ducimetiere@lyon.unicancer.fr., Penel N; Univ. Lille, CHU Lille, ULR 2694 - Metrics: Evaluation des technologies de santé et des pratiques médicales, F-59000 Lille, France; Department of Medical Oncology, Centre Oscar Lambret, Lille, France., Le Cesne A; Institut J Godinot Reims, France.
Jazyk:	angličtina
Zdroj:	European journal of cancer (Oxford, England : 1990) [Eur J Cancer] 2024 Jan; Vol. 196, pp. 113454. Date of Electronic Publication: 2023 Nov 23.
DOI:	10.1016/j.ejca.2023.113454
Abstrakt:	Sclerosing Epithelioid Fibrosarcoma (SEF) and Low Grade Fibromyxoid Sarcoma (LGFMS) are ultrarare sarcomas sharing common translocations whose natural history are not well known. We report on the nationwide exhaustive series of 330 patients with SEF or LGFMS in NETSARC+ since 2010. Patients and Methods: NETSARC (netsarc.org) is a network of 26 reference sarcoma centers with specialized multidisciplinary tumor boards (MDTB). Since 2010, (i) pathological review has been mandatory for sarcoma,and (ii) tumour/patients' characteristics have been collected in the NETSARC+ nationwide database. The characteristics of patients with SEF and LGFMS and their outcome are compared. Results: 35/73 (48%) and 125/257(49%) of patients with SEF and LGFMS were female. More visceral, bone and trunk primary sites were observed in SEF (p < 0.001). 30% of SEF vs 4% of LGFMS patients had metastasis at diagnosis (p < 0.0001). Median size of the primary tumor was 51 mm (range 10-90) for LGFMS vs 80 (20-320) for SEF (p < 0.001). Median age for LGFMS patients was 12 years younger than that of SEF patients (43 [range 4-98] vs 55 [range 10-91], p < 0.001). Neoadjuvant treatment was more often given to SEF (16% vs 9%, p = 0.05). More patients with LGFMS were operated first in reference centers (51% vs 26%, p < 0.001). The R0 rate on the operative specimen was 41% in LGFMS vs 16% in SEF (p < 0.001). Median event-free survival (EFS) of patients with SEF and LGFMS were 32 vs 136 months (p < 0.0001). The median overall survival (OS) was not reached. Fifty-months OS was 93% vs 81% for LGFMS vs SEF (p = 0.05). Median OS was 77 months after first relapse, similar for SEF and LGFMS. In multivariate analysis, age, tumor size, metastasis at diagnosis were independent prognostic factors for OS in LGFMS. Conclusions: Although sharing close molecular alterations, SEF and LGFMS have a different natural history, clinical presentation and outcome, with a higher risk of metastatic relapse in SEF. Survival after relapse is longer than with other sarcomas, and similar for SEF and LGFMS. Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)
Databáze:	MEDLINE
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