Cancer Letters

Cancer Letters

Volume 130, Issues 1–2, 14 August 1998, Pages 203-207
Cancer Letters

Familial pleural malignant mesothelioma: clustering in three sisters and one cousin

https://doi.org/10.1016/S0304-3835(98)00142-6Get rights and content

Abstract

Malignant mesothelioma is associated with asbestos, yet its occurrence in genetically-related individuals suggests a role of host predisposition. We have identified a cluster of pleural malignant mesothelioma in three sisters and one cousin (male). The women had worked in the same confectionery shop as pastry cooks and/or pastry shop assistants; the use of an asbestos-insulated oven was the putative source of exposure. The man had occupational exposure as a heating system installation worker. The family history reported malignant cancers in first-degree (larynx, brother; pleura and lung, mother), second-degree (lung, aunt and uncle) and third-degree (lung, cousin) relatives. The study reveals the potential existence of the mesothelioma risk among pastry cooks and highlights the fact that inherited factors may be involved in the development of this tumour.

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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