Recommendations for People with a Genetic Predisposition for Mesothelioma

Cancer expects from Ohio State University have just released an updated version of their recommendations for people with a genetic predisposition for mesothelioma. 

These people have an inherited mutation on their BAP1 tumor suppressor gene. This mutation puts them at risk for several different types of cancer, including malignant mesothelioma. 

It is not possible to prevent cancer in people with this genetic risk factor. But the Ohio State doctors say awareness and screening may help extend the lives of people with a genetic predisposition for mesothelioma. 

BAP1 Tumor Predisposition Syndrome

BAP1 stands for BRCA association protein 1. The BAP1 gene encodes for the BAP1 protein. BAP1 is one of the proteins that helps keep normal cells from turning into cancer cells. 

When the BAP1 gene is missing or has an error on it, a person’s body does not produce the BAP1 protein. Doctors call this BAP1 tumor predisposition syndrome or BAP1-TPDS. 

People with this genetic predisposition for mesothelioma are more likely than others to get malignant mesothelioma. The risk is much higher if they are exposed to asbestos. 

Asbestos is the main cause of mesothelioma. But most people who are exposed to asbestos never develop mesothelioma. For people with BAP1-TPDS, staying away from carcinogens like asbestos could save their lives. 

Managing Patients with a Genetic Predisposition for Mesothelioma

The key to reducing risk in people with a genetic predisposition for mesothelioma is first knowing they have it. Very few mesothelioma patients have the genetic variant so testing is not routine. 

The Ohio State researchers recommend genetic testing for family members of people with certain rare cancers. Cancers linked to BAP1-TPDS include mesothelioma, a rare type of eye cancer, two types of skin cancer, and kidney cancer. If a person’s parent has BAP1-TPDS, there is a 50/50 chance that they have it, too. 

If a person does have a genetic predisposition for mesothelioma, it is especially important that they avoid asbestos. Smoking also increases the risk of a mesothelioma diagnosis. People with a positive BAP1-TPDS test should not smoke. 

But what if a person finds out about their BAP1 syndrome after they have already been exposed? 

There is no screening test for mesothelioma. The researchers recommend these people live a healthy lifestyle and have annual physical exams. If they develop kidney cancer and have to have an MRI, their doctors should examine their chest or abdomen for signs of mesothelioma, too. 

Anyone at higher risk should know the symptoms of mesothelioma. There is no cure, but earlier diagnosis can improve survival. Some mesothelioma patients have lived for many years with a combination of treatments and healthy lifestyles. 

Source:

Pilarski, R, et al, “BAP1 Tumor Predisposition Syndrome”, Updated April 9, 2020, GeneReviews, https://www.ncbi.nlm.nih.gov/books/NBK390611/