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Comparative Study
. 2015 Jan;36(1):76-81.
doi: 10.1093/carcin/bgu227. Epub 2014 Nov 7.

Mesothelioma patients with germline BAP1 mutations have 7-fold improved long-term survival

Francine Baumann  1 , Erin Flores  2 , Andrea Napolitano  3 , Shreya Kanodia  4 , Emanuela Taioli  5 , Harvey Pass  6 , Haining Yang  2 , Michele Carbone  7
Affiliations
Comparative Study

Mesothelioma patients with germline BAP1 mutations have 7-fold improved long-term survival

Francine Baumann et al. Carcinogenesis. 2015 Jan.

Abstract

BRCA1-associated protein-1 (BAP1) mutations cause a new cancer syndrome, with a high rate of malignant mesothelioma (MM). Here, we tested the hypothesis that MM associated with germline BAP1 mutations has a better prognosis compared with sporadic MM. We compared survival among germline BAP1 mutation MM patients with that of all MM (N = 10 556) recorded in the United States Surveillance, Epidemiology, and End Results (SEER) data from 1973 to 2010. We identified 23 MM patients--11 alive--with germline BAP1 mutations and available data on survival. Ten patients had peritoneal MM, ten pleural MM and three MM in both locations. Thirteen patients had one or more malignancies in addition to MM. Actuarial median survival for the MM patients with germline BAP1 mutations was 5 years, as compared with <1 year for the median survival in the United States SEER MM group. Five-year survival was 47%, 95% confidence interval (24-67%), as compared with 6.7% (6.2-7.3%) in the control SEER group. Analysis of the pooled cohort of germline BAP1 mutation MM showed that patients with peritoneal MM (median survival of 10 years, P = 0.0571), or with a second malignancy in addition to MM (median survival of 10 years, P = 0.0716), survived for a longer time compared with patients who only had pleural MM, or MM patients without a second malignancy, respectively. In conclusion, we found that MM patients with germline BAP1 mutations have an overall 7-fold increased long-term survival, independently of sex and age. Appropriate genetic counseling and clinical management should be considered for MM patients who are also BAP1 mutation carriers.

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Figures

Fig. 1.
Fig. 1.
Kaplan–Meier survival curves of the BAP1 MM cohort patients (N = 23) and of the SEER MM cohort (N = 10556). The survival curve for the control group was based on the survival data rounded to the years.
Fig. 2.
Fig. 2.
Kaplan–Meier survival curves of the BAP1 MM cohort according to sex, age, MM site and presence of other cancers.
See this image and copyright information in PMC

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