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

Our plain-English summary of Familial pleural malignant mesothelioma: clustering in three sisters and one cousin, by V. Ascoli, C.C. Scalzo, C. Bruno, et al (Cancer Letters, 1998).

The study

• Authors: V. Ascoli, C.C. Scalzo, C. Bruno, F. Facciolo, M. Lopergolo, P. Granone, F. Nardi
• Journal: Cancer Letters
• Published: 1998;130(1-2):203-7
• Cited by: 24 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

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.

In plain English

This article was a family case report from Rome, Italy. The authors described three sisters and their male paternal cousin, all non-smokers, who developed pleural malignant mesothelioma. The cases came from a series of 119 mesotheliomas collected at three Rome hospitals since 1980. The sisters were 59, 66 and 53 years old when they fell ill. They had worked as pastry cooks and shop assistants, including in a family-owned pastry business that ran from 1957 to 1974. The authors could not identify definite asbestos exposure in the women. They proposed an asbestos-insulated electric oven as the probable shared source, but they had no exposure measurements or lung-tissue analysis, and they did not name a fiber type. The cousin, who fell ill at 64, had insulated pipes as a heating system installation worker. Other relatives had cancer: the mother had a pleuro-pulmonary tumour, a brother had laryngeal cancer, and an uncle, his son and an aunt had lung cancer. The authors concluded that the cluster was strongly suggestive of a genetic predisposition and pointed to a potential mesothelioma risk among pastry cooks.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report of one family (familial cluster of four mesothelioma cases)
• Who was studied: Three sisters (born 1929, 1930 and 1935) and one male paternal cousin (born 1932), all non-smokers, from one family in central Rome, Italy. They were drawn from a series of 119 mesothelioma cases collected in three community hospitals in Rome since 1980.
• Exposure: Sisters: work as pastry cooks and/or pastry shop assistants. The putative source was an asbestos-insulated electric oven in a family pastry business (1957 to 1974; the oven was replaced in 1964), plus earlier jobs in other confectionery shops and a biscuit factory. Cousin: heating system installation worker who insulated pipes from 1960 to 1996, and a blacksmith from 1957 to 1960. No fiber type was named, and no exposure measurements were made.
• Main results:
  • Case 1 (first-born sister): admitted at age 59 in December 1988 with right pleural effusion. The histology was consistent with epithelial mesothelioma. She died in March 1990.
  • Case 2 (second-born sister): left pleural effusion at age 66 in September 1996. A pleural biopsy showed sarcomatous mesothelioma. She died in September 1997.
  • Case 3 (fourth-born daughter): admitted at age 53 in October 1988 with left pleural effusion. Biopsied nodules were consistent with epithelial mesothelioma. She died in 1990.
  • Case 4 (male cousin): admitted at age 64 in September 1996 with right pleural effusion. The histology showed mixed mesothelioma. He died in December 1997.
  • Family history: the mother had a pleuro-pulmonary neoplasm and the third-born brother had laryngeal cancer. On the paternal side, an uncle, his son and an aunt (the cousin's mother) had lung cancer.
  • Two other siblings who had worked in the family pastry shop had chest X-ray findings: the fifth-born sister showed slight unilateral pleural thickening, and the sixth-born brother showed mild pulmonary fibrosis.
• The authors' conclusion: The authors concluded that the clustering of four mesotheliomas in one family was strongly suggestive of a genetic predisposition, and that the study revealed a potential mesothelioma risk among pastry cooks. They also concluded that inherited factors may be involved in the development of this tumour.
• Limitations the authors state:
  • The authors had no measurements of exposure and no mineralogical analysis of lung tissue.
  • The study could not identify definite asbestos exposure in the three women; it identified only a probable shared source.
  • No material was available for immunohistochemistry in case 1.

Topics

The National Library of Medicine indexes this study under subjects our site covers: Mesothelioma.

Medical terms in this study, explained in our Lexicon: Malignant Mesothelioma, Occupational Exposure, Pleura, Mesothelioma.

Related studies

• Familial clustering of malignant mesothelioma — B. Risberg, J. Nickels, et al. (1980)
• DNA copy number changes in familial malignant mesothelioma — V. Ascoli, Y. Aalto, et al. (2001)
• Malignant mesothelioma: clustering in a family producing asbestos cement in their home — K.E. Otte, T.I. Sigsgaard, et al. (1990)
• Genetic susceptibility to malignant mesothelioma and exposure to asbestos: the influence of the familial factor — D. Ugolini, M. Neri, et al. (2008)
• Familial aggregation of malignant mesothelioma in former workers and residents of Wittenoom, Western Australia — N. de Klerk, H. Alfonso, et al. (2013)
• Familial mesothelioma in first degree relatives — A.M. Kalogeraki, D.J. Tamiolakis, et al. (2013)

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