Malignant mesothelioma in subjects with Marfan's syndrome and Ehlers-Danlos syndrome: Only an apparent association

Malignant mesothelioma is a rare neoplasm which could be favored by an here ditary predisposing factor. So far, malignant mesothelioma have never been described in patients with hereditary diseases of the connective tissue. He re, we report some cases of mesothelioma affecting subjects who were not ex posed to inhalation of asbestos. One of these subjects was affected by Ehle rs-Danlos syndrome, whereas in two brothers, mesothelioma was associated wi th Marfan's syndrome. The observation of the same histologic subtype of mes othelioma in two brothers and the coexistence of two pathologic conditions of mesodermal origin indicate the presence of hereditary factors predisposi ng to the cancerogenic action of even small amounts of asbestos. Structural alterations of collagen and primary immunodeficiency may represent the hos t factor inducing development of the neoplasm. We conclude that the associa tion between these rare disorders of the connective tissue and mesothelioma may not be coincidental, but could be the result of the exposition to smal l amounts of asbestos in predisposed individuals. Copyright (C) 2000 S. Kar ger AG, Basel.