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

被引：3

作者：

Blay, J. Y. ^{[1
,2
]}

Tlemsani, C. ^{[3
]}

Toulmonde, M. ^{[4
]}

Italiano, A. ^{[4
]}

Rios, M. ^{[5
]}

Bompas, E. ^{[6
]}

Valentin, T. ^{[7
,8
]}

Duffaud, F. ^{[9
]}

Le Nail, L. R. ^{[10
]}

Watson, S. ^{[11
,12
]}

Firmin, N. ^{[13
]}

Dubray-Longeras, P. ^{[14
]}

Ropars, M. ^{[15
]}

Perrin, C. ^{[16
]}

Hervieu, A. ^{[17
]}

Lebbe, C. ^{[17
]}

Saada-Bouzid, E. ^{[18
]}

Soibinet, P. ^{[19
]}

Fiorenza, F. ^{[20
]}

Bertucci, F. ^{[21
]}

Boudou, P. ^{[3
]}

Vaz, G. ^{[1
,2
]}

Bonvalot, S. ^{[3
]}

Honore, C. ^{[22
]}

Marec-Berard, P. ^{[1
,2
]}

Minard, V. ^{[22
]}

Cleirec, M. ^{[23
]}

Biau, D. ^{[3
]}

Meeus, P. ^{[1
,2
]}

Babinet, A. ^{[3
]}

Dumaine, V. ^{[3
]}

Carriere, S. ^{[13
]}

Fau, M. ^{[4
]}

Decanter, G. ^{[24
,25
]}

Gouin, F. ^{[1
,2
]}

Ngo, C. ^{[4
]}

Le Loarer, F. ^{[3
]}

Karanian, M. ^{[1
,2
]}

Meurgey, A. ^{[1
,2
]}

Dufresne, A. ^{[1
,2
]}

Brahmi, M. ^{[1
,2
]}

Chemin-Airiau, C. ^{[1
,2
]}

Ducimetiere, F. ^{[1
,2
]}

Penel, N. ^{[24
,25
]}

Le Cesne, A. ^{[20
]}

机构：

[1] Ctr Leon Berard, Lyon, France

[2] Univ Claude Bernard, Lyon, France

[3] Hop Cochin, Paris, France

[4] Inst Bergonie, Dept Med Oncol, Bordeaux, France

[5] Ctr Alexis Vautrin, Inst Cancerol Lorraine, Nancy, France

[6] Inst Cancerol Ouest Nantes, Dept Med Oncol, Nantes, France

[7] Inst Claudius Regaud, Toulouse, France

[8] Inst Univ Cancerol Oncopole, Toulouse, France

[9] La Timone Univ Hosp, Marseille, France

[10] CHU Tours, F-37000 Tours, France

[11] Inst Curie, Dept Med Oncol, Paris, France

[12] Inst Curie Res Ctr, INSERM, U830, Paris, France

[13] Inst Cancerol Montpellier, Montpellier, France

[14] Ctr Jean Perrin, ERTICa EA 4677, Clermont Ferrand, France

[15] Eugene Marquis Comprehens Canc Ctr, Paris, France

[16] CHU, Rennes, France

[17] Ctr George Francois Leclerc, Dijon, France

[18] St Louis Hosp, Dermatooncol Unit, Paris, France

[19] Ctr Antoine Lacassagne, Nice, France

[20] Inst J Godinot Reims, Reims, France

[21] Inst Paoli Calmettes, Marseille, France

[22] Gustave Roussy Canc Campus, Villejuif, France

[23] CHU Nantes, Dept Pediat, Nantes, France

[24] Univ Lille, CHU Lille, ULR 2694 Metr Evaluat Technol Sante & Prat Med, F-59000 Lille, France

[25] Ctr Oscar Lambret, Dept Med Oncol, Lille, France

来源：

EUROPEAN JOURNAL OF CANCER | 2024年 / 196卷

关键词：

Low grade fibromyxoid sarcoma; Sclerosing epithelioid fibrosarcoma; Sarcoma; Rare cancers; Guidelines; Survival; Reference centers; SPINDLE-CELL TUMOR; GIANT ROSETTES; REARRANGEMENTS; RARE;

D O I：

10.1016/j.ejca.2023.113454

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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.

引用

页数：8

共 4 条

[1] Differential presentation and outcome of sclerosing epithelioid fibrosarcoma (SEF) vs low grade fibromyxoid sarcoma (LGFMS): The nationwide series of 330 patients in NETSARC since 2010
Blay, J-Y.
Rouquette, P. Boudou
Toulmonde, M.
Italiano, A.
Penel, N.
Rios, M.
Gouin, F.
Valentin, T.
Duffaud, F.
Le Nail, L. R.
Watson, S.
Firmin, N.
Dubray-Longeras, P.
Ropars, M.
Hervieu, A.
Fiorenza, F.
Lebbe, C.
Saada, E. B.
Oudot, P. Soibinet
Le Cesne, A.
ANNALS OF ONCOLOGY, 2023, 34 : S1036 - S1036
[2] A hybrid lesion: Low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF)
Kesrouani, Carole
Zemoura, Leila
Trassard, Martine
Lae, Marick
ANNALES DE PATHOLOGIE, 2016, 36 (05) : 351 - 354
[3] A Genetic Dichotomy Between Pure Sclerosing Epithelioid Fibrosarcoma (SEF) and Hybrid SEF/Low-Grade Fibromyxoid Sarcoma: A Pathologic and Molecular Study of 18 Cases
Prieto-Granada, Carlos
Zhang, Lei
Chen, Hsiao-Wei
Sung, Yun-Shao
Agaram, Narasimhan P.
Jungbluth, Achim A.
Antonescu, Cristina R.
GENES CHROMOSOMES & CANCER, 2015, 54 (01): : 28 - 38
[4] Translocation-positive low-grade fibromyxoid sarcoma:: Clinicopathologic and molecular analysis of a series expanding the morphologic spectrum and suggesting potential relationship to sclerosing epithelioid fibrosarcoma -: A study from the French Sarcoma Group
Guillou, Louis
Benhattar, Jean
Gengler, Carole
Gallagher, Gabrielle
Ranchere-Vince, Dominique
Collin, Francoise
Terrier, Philippe
Terrier-Lacombe, Marie-Jose
Leroux, Agnes
Marques, Bernard
Somerhausen, Nicolas de Saint Aubain
Keslair, Frederique
Pedeutour, Florence
Coindre, Jean-Michel
AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 2007, 31 (09) : 1387 - 1402

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