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

Respiration. 2000;67(2):223-8. doi: 10.1159/000029493.


Malignant mesothelioma is a rare neoplasm which could be favored by an hereditary predisposing factor. So far, malignant mesothelioma have never been described in patients with hereditary diseases of the connective tissue. Here, we report some cases of mesothelioma affecting subjects who were not exposed to inhalation of asbestos. One of these subjects was affected by Ehlers-Danlos syndrome, whereas in two brothers, mesothelioma was associated with Marfan's syndrome. The observation of the same histologic subtype of mesothelioma in two brothers and the coexistence of two pathologic conditions of mesodermal origin indicate the presence of hereditary factors predisposing to the cancerogenic action of even small amounts of asbestos. Structural alterations of collagen and primary immunodeficiency may represent the host factor inducing development of the neoplasm. We conclude that the association between these rare disorders of the connective tissue and mesothelioma may not be coincidental, but could be the result of the exposition to small amounts of asbestos in predisposed individuals.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Biopsy
  • Ehlers-Danlos Syndrome / complications*
  • Ehlers-Danlos Syndrome / diagnosis
  • Fatal Outcome
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Lip Neoplasms / genetics
  • Male
  • Marfan Syndrome / complications*
  • Marfan Syndrome / diagnosis
  • Mesothelioma / diagnosis
  • Mesothelioma / genetics*
  • Middle Aged
  • Peritoneal Neoplasms / diagnosis
  • Peritoneal Neoplasms / genetics*
  • Pleural Neoplasms / diagnosis
  • Pleural Neoplasms / genetics*