Familial pleural malignant mesothelioma: clustering in three sisters and one cousin
Introduction
Clustering of malignant mesothelioma (MM) within families has been reported as involving sibling–sibling 1, 2, 3, 4, 5, 6, parent–child 5, 7, 8, 9, 10and husband–wife combinations 10, 11; its occurrence among sisters is rare 4, 6. Familial MM may result from a common exposure to asbestos at work or in the home. The striking aggregation in genetically-related members suggests also a possible role of genetic predisposition [12]. MM may occur in those individuals with a cancer-prone genotype susceptible to the toxic effect of asbestos 13, 14.
We describe herein familial pleural MM in three sisters whose paternal cousin was also affected by MM. These cases belong to a series of 119 MMs that we have being collecting in three community hospitals in Rome, Italy, since 1980 [15].
Section snippets
Case reports
We present a family whose abridged pedigree is shown in Fig. 1, in which three sisters and one paternal cousin developed pleural MM. The diagnosis was based on (i) radiographic/endoscopic/surgical gross morphology and clinical presentation, (ii) the absence of another primary tumour, (iii) characteristic histologic findings on thoracoscopic/thoracotomic specimens and (iv) consistent immunohistochemical results using a panel of monoclonal antibodies against cytokeratin, vimentin, Leu-M1 (CD15),
Discussion
We described the unusual clustering of MM among three sisters with a history of cancer in first-degree and second-degree relatives and evidence of MM in a third-degree relative, that to our knowledge has never been reported previously. Several reports have documented asbestos-related familial MM in genetically-related individuals with a history of occupational 1, 2, 3, 5, 6, 10, or domestic exposure 2, 5, 6, 7, 8, 9. There have also been articles reporting familial MM in patients with
Acknowledgements
This work was supported by grants from the Ministero dell'Università e della Ricerca Scientifica (MURST). The authors gratefully acknowledge Dr Roberto Calisti (USL 34-Regione Piemonte) for his relevant contribution concerning asbestos-insulated ovens and Dr Fabrizio Magrelli (USL RM/B-Regione Lazio) for collecting information on the occupational history of one of the patients.
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