1478PD - Adult Translocation-related soft tissue sarcomas (TRS): presentation, management and outcome of 2,143 cases confirmed by expert pathologists

Date 11 September 2017
Event ESMO 2017 Congress
Session Sarcoma
Topics Cancer in Adolescents
Soft Tissue Sarcomas
Sarcoma
Presenter Nicolas Penel
Citation Annals of Oncology (2017) 28 (suppl_5): v521-v538. 10.1093/annonc/mdx387
Authors N. Penel1, J. Coindre2, A. Giraud3, P. Terrier4, D. Ranchere-Vince5, F. Collin6, S. Le Guellec7, C. Bazille8, M. Lae9, G. De Pinieux10, I. Ray-Coquard11, S. Bonvalot12, A. Le Cesne12, Y. Robin13, E. Stoeckle14, F. Ducimetiere15, M. Toulmonde16, J. Blay17
  • 1Medical Oncology, Centre Oscar Lambret, 59020 - Lille/FR
  • 2Department Of Biopathology, Institute Bergonié, 33076 - Bordeaux/FR
  • 3Clinical And Epidemiological Research Unit, Institute Bergonié, 33076 - Bordeaux/FR
  • 4Department Of Pathology, Institut Gustave Roussy, Villejuif/FR
  • 5Biopathology, Centre Leon Berard, Lyon/FR
  • 6Pathology, Centre GF Leclerq, Dijon/FR
  • 7Pathology, Institut Universitaire de Cancérologie de Toulouse, Toulouse/FR
  • 8Pathological Anatomy, University Hospital, Caen/FR
  • 9Pathology, Institut Curie, Paris/FR
  • 10Pathology, University Hospital, Tours/FR
  • 11Medical Oncology, Centre Léon Bérard, Lyon/FR
  • 12Medical Oncology & Surgery, Gustave Roussy Institut de Cancérologie, 94805 - Villejuif/FR
  • 13Pathology, Centre Oscar Lambret, 59020 - Lille/FR
  • 14Surgery, Institute Bergonié, 33076 - Bordeaux/FR
  • 15Medical Oncology, Centre Léon Bérard, 69008 - Lyon/FR
  • 16Medical Oncology, Institute Bergonié, 33076 - Bordeaux/FR
  • 17Medical Oncology, Centre Leon Berard, 69008 - Lyon/FR

Abstract

Background

To better characterize the TRS patterns compared to other sarcomas.

Methods

Retrospective multicenter study of 12,262 patients treated between 01/1980 and 12/2013 in one of 22 French Referral Sarcoma Center and enrolled in the “Conticabase”. Diagnoses were systematically reviewed by expert pathologists and entities classified according to the 2013 WHO Classification.

Results

The median follow-up was 4.9 years (95%-CI: 4.7-5.0). TRS included 13 entities: synovial S (760 cases; 7.4%/5-y OS: 64%), myxoid LPS (436; 4.2%/5-y OS:88%), PNET (205; 2.0%/5-y OS:58%), round cell LPS (183; 1.8%/5-yOS: 70%), alveolar RMS (122; 1.2%/5-yOS: 25%), malignant SFT (86; 0.8%/5-yOS: 77%), clear cell sarcoma (63; 0.6%/5-yOS: 67%), LGFMS (60; 0.6%/5-yOS: 82%), desmoplastic round cell tumor (56, 0.5%/5-y OS: 11%), ESMCS (54; 0.5%/5-yOS:78%), ASPS (48; 0.5%/5-yOS: 66%), EHE (42; 0.4%/5-yOS: 55%), and sclerosing epithelioid fibrosarcoma (28; 0.3%/5-y OS: 70%). All TRS (2,143 cases; 20.8%) are associated with younger age (40.6 versus 60.0; p 

Conclusions

TRS display specific pattern compared to other sarcomas. Second opinion by expert pathologist and use of molecular biology confirmatory test are of major importance to recognize this population and discuss multimodal approach at early stage of the disease.

Clinical trial identification

Legal entity responsible for the study

Centre Oscar Lambret, Lille, France

Funding

None

Disclosure

All authors have declared no conflicts of interest.