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Frequency of genetic disease and previous cancer in 493 adults with visceral or soft tissue sarcomas


Bulletin du Cancer. Volume 90, Number 10, 887-95, Octobre 2003, Article original

Résumé   Article gratuit  

Author(s) : Nicolas Penel, Gérard Depadt, Marie‐OdileVilain, Luc Vanseymortier, Luc Ceugnart, Sophie Taieb, Xavier Mirabel, Nathalie Deligny, Annick Chevalier, Marie‐Christine Baranzelli, Fabienne Pichon, Dominique Hoguet, Yves‐Marie Robin, Éric Lartigau

Summary : Little is known about epidemiology of adults soft tissue and visceral sarcomas (ASTS). The frequency of previous cancers and associated genetic diseases has been analyzed out of 493 ASTS, treated between 1997 and 2002 at Oscar Lambret Cancer Center. Median age is 51, sex ratio is close to 1. Liposarcomas and malignant fibrous histiocytofibromas are the two main types (respectively 104 and 86 cases). Upper and lower limbs are the two main locations (respectively 176 and 75 cases). Fifteen patients had associated genetic disease, including 12 cases of Recklinghausen diseases. 7 out of those 15 patients have neurosarcoma. 30 patients have previous cancers, including 7 breast cancers, 3 lymphomas and 3 chronic lymphocytic leukemias. Four out of those 30 patients have two different previous cancers. 13 patients have radiation‐induced sarcomas, after an average 10‐year‐period, and an average dose of 53 Gy. Undifferenciated sarcomas are the main histologic type (8\\13), followed by angiosarcomas (2\\13). Radiation‐induced sarcomas are located in the chest wall (7\\13), in pelvis (2\\13) and head and neck (2\\13). Those sarcomas are high grade (10 grade III tumours). ASTS epidemiology is complex with different risk factors depending on histologic type.

Keywords : sarcoma, soft tissue, second cancer, neurofibromatosis, radiation‐induced tumor, breast cancer, epidemiology

 

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