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

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

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. NETSARC (netsarc....

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Veröffentlicht in:European journal of cancer (1990) 2024-01, Vol.196, p.113454-113454, Article 113454
Hauptverfasser: Blay, J Y, Tlemsani, C, Toulmonde, M, Italiano, A, Rios, M, Bompas, E, Valentin, T, Duffaud, F, Le Nail, L R, Watson, S, Firmin, N, Dubray-Longeras, P, Ropars, M, Perrin, C, Hervieu, A, Lebbe, C, Saada-Bouzid, E, Soibinet, P, Fiorenza, F, Bertucci, F, Boudou, P, Vaz, G, Bonvalot, S, Honoré, C, Marec-Berard, P, Minard, V, Cleirec, M, Biau, D, Meeus, P, Babinet, A, Dumaine, V, Carriere, S, Fau, M, Decanter, G, Gouin, F, Ngo, C, Le Loarer, F, Karanian, M, Meurgey, A, Dufresne, A, Brahmi, M, Chemin-Airiau, C, Ducimetiere, F, Penel, N, Le Cesne, A
Format: Artikel
Sprache:eng
Schlagworte:
Child
Female
Fibrosarcoma
Fibrosarcoma - surgery
Gene Rearrangement
Humans
Life Sciences
Male
Recurrence
Sarcoma
Sarcoma - pathology
Soft Tissue Neoplasms
Soft Tissue Neoplasms - pathology
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Zusammenfassung: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. 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. 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 
ISSN:0959-8049
1879-0852
DOI:10.1016/j.ejca.2023.113454