Context.-Malignant mesothelioma (MM) is a component of the BAP1 tumor predisposition syndrome. Other than in BAP1 familial studies, nonmesothelial neoplasms in individuals with MM has not been comprehensively assessed.Objective.-To assess the spectrum and prevalence of nonmesothelial neoplasms in individuals with MM.Design.-Individuals with MM and second neoplasms were identified from a database of 3900 MM cases. The expected prevalence of each type of neoplasm was calculated and compared with the actual prevalence in the study population using available Surveillance, Epidemiology, and End Results data and other published data.Results.-Two hundred seventy nonmesothelial neoplasms were identified in 241 individuals (6% of the study population) with MM. Prostate adenocarcinoma was most common. Non-Hodgkin lymphoma, Hodgkin lymphoma, lung carcinoma, urothelial carcinoma, breast carcinoma, chronic lymphocytic leukemia, clear cell renal cell carcinoma, head and neck squamous cell carcinoma, papillary renal cell carcinoma, multiple myeloma/plasmacytoma, meningioma, pleomorphic undifferentiated sarcoma, chronic myelogenous leukemia, ocular melanoma, hepatocellular carcinoma, liposarcoma, and Wilms tumor all were more prevalent than expected.Conclusions.-Nonmesothelial neoplasms are uncommon in individuals with MM, but certain tumor types are increased in prevalence. In an unselected study population with respect to BAP1 status, the prevalence of several tumor types described in BAP1 mutation carriers, including lung carcinoma, clear cell renal cell carcinoma, breast carcinoma, meningioma, pleomorphic undifferentiated sarcoma, and ocular melanoma, was increased.( Although the frequency of second neoplasms in individuals with MM has been examined in the context of familial studies of BAP1 mutation carriers, the prevalence and types of second neoplasms among individuals with MM unselected with regard to BAP1 status are unknown.1 The purpose of this study is to comprehensively assess the spectrum and prevalence of nonmesothelial neoplasms in individuals with MM.
DESIGNThis study was approved by the institutional review board at Duke University Medical Center, Durham, North Carolina. A database of one of the authors consisting of 3900 MM cases received for professional and medicolegal consultation from 1982 through 2016 was searched retrospectively for individuals with MM who also had a history of one or more antecedent or synchronous (ie, diagnosed during same hospital admission) nonmesothelial neoplasms. The expected prevalence of each type of nonmesothelial neoplasm was calculated based on the overall estimated prevalence reported by the Surveillance, Epidemiology, and End Results (SEER) program for 2013 and US Census Bureau population data from the same year. 4 As SEER data do not separate renal cancers by histologic type, the expected prevalence for all renal cancers was calculated and we extrapolated the expected prevalence of clear cell renal cell carcinoma and papillary renal cell carcinoma to be less than that of a...