The bad news is that they have a significantly higher risk of contracting malignant mesothelioma and several other types of cancer than people without this genetic mutation. The good news is that, people with the BAP1 mutation who do get mesothelioma, have a seven-fold increase in long-term survival over mesothelioma patients without this genetic anomaly.
Now, a multicenter study involving the University of Hawaii Cancer Center, the Samuel Oschin Comprehensive Cancer Institute-Cedars-Sinai in Los Angeles, the Hofstra-North Shore LIJ School of Medicine in New York, and the New York University Langone Medical Center finds that BAP1 mesothelioma may actually be more survivable. The team used data from the U.S. SEER database of 10,556 mesothelioma patients between 1973 and 2010 to identify 23 with the BAP1 mutation and survival data.
While it is rare for mesothelioma patients to live more than about a year after diagnosis, the BAP1 mesothelioma patients from the SEER database had an overall median survival rate of five years. Forty-seven percent of these patients lived for five years, compared to just 6.7% in the control group. Patients with BAP1-associated peritoneal mesothelioma had an even better outlook than those with the pleural form of the disease with a median overall survival of 10 years.
Interestingly, study subjects who had a second malignancy in addition to mesothelioma had a better overall survival than those who had only pleural mesothelioma. The increased survival rates applied regardless of the gender or age of the patients.
Almost all cases of mesothelioma are linked to asbestos exposure. But given that the prognosis and clinical management may be different for mesothelioma patients who also have a BAP1 mutation, the authors of the new study conclude that these patients should have access to appropriate genetic counseling. The new research was published in Carcinogenesis.
Baumann, F, et al, “Mesothelioma Patients with Germline BAP1 Mutations have Seven=Fold Improved Long-term Survival”, November 7, 2014, Carcinogenesis