2018
DOI: 10.5858/arpa.2017-0307-oa
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Malignant Mesothelioma in Individuals With Nonmesothelial Neoplasms

Abstract: 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 preva… Show more

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Cited by 10 publications
(6 citation statements)
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References 28 publications
(19 reference statements)
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“…Out of 4,432 unique liposarcoma records in SEER (1973-2015 cohort), 2968 (0.00063%) had a recording of other concurrent cancers. Liposarcoma have concurrent diagnoses in ovarian cancer 39 , hereditary nonpolyposis colorectal cancer 40 , Muire-Torre syndrome 41 , multiple myeloma 42 and CLL (also our recent unpublished data and infiltration in TCGA-SARC sample) 8 . Identifying these predisposition genes will enable us to interpret mutations in sporadic cases, as illustrated by the discovery of the telomere protection gene, POT1, as predisposing to angiosarcoma and cardiac sarcomas 39 .…”
Section: Liposarcoma Formation Through Genetic Lossmentioning
confidence: 83%
“…Out of 4,432 unique liposarcoma records in SEER (1973-2015 cohort), 2968 (0.00063%) had a recording of other concurrent cancers. Liposarcoma have concurrent diagnoses in ovarian cancer 39 , hereditary nonpolyposis colorectal cancer 40 , Muire-Torre syndrome 41 , multiple myeloma 42 and CLL (also our recent unpublished data and infiltration in TCGA-SARC sample) 8 . Identifying these predisposition genes will enable us to interpret mutations in sporadic cases, as illustrated by the discovery of the telomere protection gene, POT1, as predisposing to angiosarcoma and cardiac sarcomas 39 .…”
Section: Liposarcoma Formation Through Genetic Lossmentioning
confidence: 83%
“…[26][27][28][29] The full spectrum of this syndrome is still being characterised through the discovery of new associated tumours, including cutaneous squamous cell carcinoma, 30 basal cell carcinoma, 31,32 lung adenocarcinoma, 33 oesophageal adenocarcinoma, 34 breast cancer, 35 rhabdoid meningioma, 36 neuroendocrine tumours, 37 and certain types of sarcoma. 38,39 Some types of cancer associated with this syndrome may also have a poor prognosis. In the present study, significant associations of TC were identified with a cancer family history of cancers related to the BAP1 tumour predisposition syndrome, including mesothelioma, melanoma, squamous cell carcinomas, adenocarcinomas, sarcomas and breast cancer.…”
Section: Discussionmentioning
confidence: 99%
“…Reprint requests: Arman Mashayekhi, MD, Ocular Oncology Service, 840 Walnut Street, Suite 1440, Philadelphia, PA 19107; e-mail: arman@shields.md patients, higher than the expected prevalence of 0.65% which was based on the overall estimated prevalence reported by the Surveillance, Epidemiology, and End Results (SEER) program for 2013 and U.S. Census Bureau population data from the same year. 6 In the general population, 80% of malignant pleural mesotheliomas occur in patients with a history of asbestos exposure, while the risk factors for malignant peritoneal mesothelioma are still not well established. 5 Interestingly, however, while only 20 to 30% of malignant pleural mesotheliomas were found to harbor BAP1 alterations, nearly 85% of malignant peritoneal mesotheliomas are associated with BAP1 mutation.…”
Section: Discussionmentioning
confidence: 99%
“…Choroidal melanoma and peritoneal mesothelioma are two rare cancers that can occur together in the setting of BAP1-TPDS. This novel hereditary cancer syndrome is characterized by autosomal dominant germline mutations in the BAP1 gene 1,6 and an increased risk of developing several benign and malignant tumors, including (in descending order of likelihood), choroidal melanoma, malignant mesothelioma, cutaneous melanoma, clear cell renal cell carcinoma, basal cell carcinoma, and atypical cutaneous Spitz tumor (melanocytic BAP-1 mutated atypical intradermal tumor, MBAIT). 1 Other related tumors include breast cancer, prostate cancer, cholangiocarcinoma, meningioma, neuroendocrine tumors, nonsmall cell lung adenocarcinoma, and thyroid cancer.…”
Section: Discussionmentioning
confidence: 99%