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Sarcoma

1260 - Adult Translocation-related soft tissue sarcomas (TRS): presentation, management and outcome of 2,143 cases confirmed by expert pathologists

Date

11 Sep 2017

Session

Sarcoma

Presenters

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

Author affiliations

  • 1 Medical Oncology, Centre Oscar Lambret, 59020 - Lille/FR
  • 2 Department Of Biopathology, Institute Bergonié, 33076 - Bordeaux/FR
  • 3 Clinical And Epidemiological Research Unit, Institute Bergonié, 33076 - Bordeaux/FR
  • 4 Department Of Pathology, Institut Gustave Roussy, Villejuif/FR
  • 5 Biopathology, Centre Leon Berard, Lyon/FR
  • 6 Pathology, Centre GF Leclerq, Dijon/FR
  • 7 Pathology, Institut Universitaire de Cancérologie de Toulouse, Toulouse/FR
  • 8 Pathological Anatomy, University Hospital, Caen/FR
  • 9 Pathology, Institut Curie, Paris/FR
  • 10 Pathology, University Hospital, Tours/FR
  • 11 Medical Oncology, Centre Léon Bérard, Lyon/FR
  • 12 Medical Oncology & Surgery, Gustave Roussy Institut de Cancérologie, 94805 - Villejuif/FR
  • 13 Pathology, Centre Oscar Lambret, 59020 - Lille/FR
  • 14 Surgery, Institute Bergonié, 33076 - Bordeaux/FR
  • 15 Medical Oncology, Centre Léon Bérard, 69008 - Lyon/FR
  • 16 Medical Oncology, Institute Bergonié, 33076 - Bordeaux/FR
  • 17 Medical Oncology, Centre Leon Berard, 69008 - Lyon/FR
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Resources

Abstract 1260

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.

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