Author of

• 14661

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  P2.08 - Poster Session/ Thymoma, Mesothelioma and Other Thoracic Malignancies (ID 225)

  • Event: WCLC 2015
  • Type: Poster
  • Track: Thymoma, Mesothelioma and Other Thoracic Malignancies
  • Presentations: 1
  • Moderators:
  • Coordinates: 9/08/2015, 09:30 - 17:00, Exhibit Hall (Hall B+C)

  • 14663

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    P2.08-002 - Germline BAP1 Mutation Is Associated with a Significant Increased Survival and Multiple Cancer in Mesothelioma Patients (ID 961)

    09:30 - 09:30  |  Author(s): E. Flores
    • Abstract

    Background:
    Because the diagnosis is often made at a late stage, malignant mesothelioma (MM) prognosis is very poor, with a median survival of 6-12 months and a five-year survival of less than 5%. We found that germline BAP1 mutation is associated with a new cancer syndrome, including rare malignancies such as MM and uveal carcinoma (UV), and other cancers. We noted that some MM cases that we followed from BAP1 mutated families had prolonged survival. We carried out a pooled analysis of BAP1 mutated MM patients to test the hypothesis that they had a better survival compared to sporadic MM.
    
    Methods:
    : We included all published BAP1 germline mutated MMs with available data on BAP1 status, site of MM, age at diagnosis, gender, and age at death or status at end of follow-up, in addition to the BAP1 mutated MM cases from families that we are following. Twenty-three BAP1 MM patients were included. Using the Kaplan-Meier method and Wilcoxon test, we compared survival among BAP1 mutated MM patients with that of all MMs (N = 10 556) recorded in the US SEER data from 1973 to 2010.
    
    Results:
    In our BAP1 cohort, 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 five years, as compared with less than one year in the SEER MM control group. Five-year survival was 47%, 95%CI [24-67%], as compared with 6.7% [6.2-7.3%] in the SEER MM control group. The small size of our BAP1 cohort did not allow for significant statistical comparisons. However, 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 to patients who only had pleural MM, or MM patients without a second malignancy, respectively. Figure 1
    
    
    
    Conclusion:
    MM patients with germline BAP1 mutations have an overall seven-fold increased survival, independently of sex and age. This better prognosis was associated with multiple cancer and/or peritoneal MM. Appropriate genetic counseling and clinical management should be considered for MM patients who are also BAP1 mutation carriers.