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Immunohistochemistry of our patient\u2019s malignant cytology sample showed loss of Breast Cancer Gene 1-associated protein-1 expression (BAP1). The patient had synchronous diagnoses of primary squamous cell carcinoma of the anus, benign schwannoma and meningioma. Following the completion of 18 cycles of pemetrexed and bevacizumab, the patient has remained in clinical remission for 8\u2009months. We examine the unusual susceptibility of unresectable MPeM to systemic chemotherapy and attribute susceptibility to the molecular milieu created by mutations in multiple DNA repair pathways. We encourage increased testing for and analysis of mutations in DNA repair pathways to improve future treatment outcomes in this rare malignancy.<\/jats:p>","DOI":"10.1136\/bcr-2023-255916","type":"journal-article","created":{"date-parts":[[2023,12,23]],"date-time":"2023-12-23T09:55:18Z","timestamp":1703325318000},"page":"e255916","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Complete clinical remission of malignant peritoneal mesothelioma with systemic pemetrexed and bevacizumab in a patient with a BAP1 mutation"],"prefix":"10.1136","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-0789-8941","authenticated-orcid":false,"given":"Jimmy","family":"Lee","sequence":"first","affiliation":[{"name":"University of Florida College of Medicine, Gainesville, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jordan","family":"Turetsky","sequence":"additional","affiliation":[{"name":"University of Florida College of Medicine, Gainesville, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elham","family":"Nasri","sequence":"additional","affiliation":[{"name":"Department of Pathology, Immunology and Laboratory Health, University of Florida Health, Gainesville, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sherise C","family":"Rogers","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Hematology & Oncology, University of Florida Health, Gainesville, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2023,12,23]]},"reference":[{"key":"2025082709081633000_16.12.e255916.1","doi-asserted-by":"crossref","first-page":"3057","DOI":"10.1007\/s00423-022-02594-6","article-title":"Ten-year single-center experience with treatment of primary diffuse malignant peritoneal mesothelioma (DMPM) by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC)","volume":"407","author":"Acs","year":"2022","journal-title":"Langenbecks Arch Surg"},{"key":"2025082709081633000_16.12.e255916.2","doi-asserted-by":"publisher","DOI":"10.1245\/s10434-014-3978-x"},{"key":"2025082709081633000_16.12.e255916.3","doi-asserted-by":"crossref","first-page":"942","DOI":"10.3390\/cancers14040942","article-title":"Incidence, survival analysis and future perspective of primary peritoneal mesothelioma (PPM): a population-based study from SEER database","volume":"14","author":"Ullah","year":"2022","journal-title":"Cancers (Basel)"},{"key":"2025082709081633000_16.12.e255916.4","first-page":"147","article-title":"Diffuse malignant peritoneal mesothelioma in a 31-year-old patient--case report","volume":"28","author":"Prorocic","year":"2007","journal-title":"Eur J Gynaecol Oncol"},{"key":"2025082709081633000_16.12.e255916.5","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1097\/00000421-199212000-00013","article-title":"Successful therapy of peritoneal mesothelioma with intraperitoneal chemotherapy alone. 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