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Annals of Oncology Advance Access originally published online on October 31, 2007
Annals of Oncology 2007 18(12):2030-2036; doi:10.1093/annonc/mdm381
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© 2007 European Society for Medical Oncology

sarcomas

Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 cases

J. Fayette1,2,*, E. Martin3, S. Piperno-Neumann4, A. Le Cesne5, C. Robert5, S. Bonvalot6, D. Ranchère7, P. Pouillart4, J. M. Coindre8 and J. Y. Blay1,2

1 Hospices Civils de Lyon, Université Lyon1, Hôpital Edouard. Herriot, Oncologie Médicale, Pavillon E, Lyon
2 Unité Institut National de la Santé et de la Recherche Médicale 590, Université Lyon 1, Centre Léon Bérard, Lyon
3 Santé Publique, Centre Léon Bérard, Lyon
4 Oncologic Médicale Institut Curie, Paris
5 Oncologic Médicale Service de Chirurgie Institut Gustave Roussy, Villejuif
6 Service de Chirurgie
7 Service d'anatomo-pathologie, Institut Curie, Paris
8 Service d'Anatomo-Pathologie, Institut Bergonié, Bordeaux, France

* Correspondence to: Dr J. Fayette, Hospices Civils de Lyon, Université Lyon1, Hôpital E. Herriot, Oncologie Médicale, Pavillon E, 5 place d'Arsonval, 69003 Lyon, France. Tel: +33-472-117398; Fax: +33-472-117328; E-mail: jfayette{at}9online.fr

Background: Angiosarcomas are rare, heterogeneous and a retrospective study was conducted to describe their natural history.

Patients and methods: We reviewed 161 files of angiosarcoma treated in three institutions of the French Sarcoma Group from 1980 to 2004. Survival and prognostic factors for survival were analyzed.

Results: Median age was 52 years. Primary sites were the breast (35%), skin (20%) and soft tissues (13%). At initial diagnosis, 31 (19%) had metastases. Surgery was the first treatment in 121 (75%) patients combined with chemotherapy or radiotherapy in 34 and 32, respectively. Ninety (74%) of these 121 patients relapsed, mostly locally (50). With an average time since initial diagnosis of 8.1 years, 123 (76%) patients progressed and 76 (47%) died. Median survival was 3.4 years [95% confidence interval (CI) 2.4–5.8], and the 5-year overall survival (OS) rate was 43% (95% CI 33–53). In multivariate analysis, liver primary site [relative risk (RR) = 12.62], performance status (PS) of two or more (RR = 3.83), presence of metastases at diagnosis (RR = 2.50), soft tissue tumor (RR = 0.31) were correlated to OS. PS, liver and soft tissue tumors were identified as independent prognostic factors for progression-free survival.

Conclusions: Angiosarcomas have an overall poor outcome, but with a clearly distinct prognosis depending on the primary site.

Key words: angiosarcoma, retrospective study, sarcoma

Received for publication January 23, 2007. Revision received June 28, 2007. Accepted for publication July 7, 2007.


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