Fukasawa Naoto, Agemi Yoko, Shiba Aya, Aga Masaharu, Hamakawa Yusuke, Miyazaki Kazuhito, Taniguchi Yuri, Misumi Yuki, Shimokawa Tsuneo, Ono Kyoko, Hayashi Hiroyuki, Okamoto Hiroaki
Department of Respiratory Medicine Yokohama Municipal Citizen's Hospital Yokohama Japan.
Department of Pathology Kanagawa Cancer Center Yokohama Japan.
Respirol Case Rep. 2022 Aug 6;10(9):e01004. doi: 10.1002/rcr2.1004. eCollection 2022 Sep.
Malignant pericardial mesothelioma (MPM) is a rare tumour that arises from the mesothelial cells of the pericardium. No standard treatment has been established owing to a poor treatment response; therefore, MPM has a poor prognosis. We herein report a rare case of MPM in a 70-year-old man that was diagnosed immunohistopathologically using cell block sections of pericardial fluid and in which long-term survival for more than 3 years was achieved with only periodic pericardial drainage. Immunohistopathological staining investigations, especially BRCA1-associated protein 1 (BAP1) immunostaining using cell block sections of pericardial effusion, are effective in making a diagnosis of MPM. Well-differentiated papillary mesothelioma (WDPM) with BAP1 loss progresses to MPM in the long term, showing that BAP1 loss may induce phenotypical evolution of WDPM. BAP1 loss may also progress to malignant mesothelioma in situ and then to invasive mesothelioma. BAP1 immunohistochemistry should be considered for the early diagnosis of MPM.
恶性心包间皮瘤（MPM）是一种罕见的肿瘤，起源于心包的间皮细胞。由于治疗反应不佳，尚未确立标准治疗方法；因此，MPM的预后较差。我们在此报告一例70岁男性的罕见MPM病例，该病例通过心包积液的细胞块切片进行免疫组织病理学诊断，并且仅通过定期心包引流就实现了3年以上的长期生存。免疫组织病理学染色检查，特别是使用心包积液细胞块切片进行的乳腺癌1号基因相关蛋白1（BAP1）免疫染色，对MPM的诊断有效。伴有BAP1缺失的高分化乳头状间皮瘤（WDPM）长期会进展为MPM，这表明BAP1缺失可能诱导WDPM的表型演变。BAP1缺失也可能进展为原位恶性间皮瘤，然后发展为浸润性间皮瘤。应考虑将BAP1免疫组化用于MPM的早期诊断。
