Karpes Josh B, Shamavonian Raphael, Dewhurst Suzannah, Cheng Ernest, Wijayawardana Ru, Ahmadi Nima, Morris David L
Hepatobiliary and Surgical Oncology Unit, Department of Surgery, St George Hospital, Kogarah, NSW 2217, Australia.
St George and Sutherland Clinical School, University of New South Wales, Sydney, NSW 2217, Australia.
Cancers (Basel). 2023 Sep 25;15(19):4704. doi: 10.3390/cancers15194704.
Malignant peritoneal mesothelioma (MPM) is an extremely rare malignancy usually confined to the abdominal cavity. With an aggressive natural history, morbidity and mortality are consequences of progressive locoregional effects within the peritoneal cavity. The first reported case was in the early 20th century, however, due to the rare nature of the disease and a large gap in understanding of the clinicopathological effects, the next reported MPM cases were only published half a decade later. Since then, there has been exponential growth in our understanding of the disease, however, there are no prospective data and a paucity of literature regarding management. Traditionally, patients were treated with systemic therapy and the outcomes were very poor, with a median survival of less than one year. However, with the advent of cytoreductive surgery and locoregional chemotherapy, there have been significant improvements in survival. Even more recently, with an improved understanding of the molecular pathogenesis of MPM, there have been reports of improved outcomes with novel therapies. Given the disastrous natural history of MPM, the limited data, and the lack of universal treatment guidelines, an in-depth review of the past, present, and future of MPM is critical to improve treatment regimens and, subsequently, patient outcomes.
恶性腹膜间皮瘤（MPM）是一种极为罕见的恶性肿瘤，通常局限于腹腔。其自然病程具有侵袭性，发病率和死亡率是腹腔内局部进展效应的结果。首例报告病例出现在20世纪初，然而，由于该疾病的罕见性以及对临床病理效应的认识存在较大差距，接下来报告的MPM病例直到五年后才得以发表。从那时起，我们对该疾病的认识呈指数级增长，然而，关于治疗管理方面尚无前瞻性数据且文献匮乏。传统上，患者接受全身治疗，结果非常糟糕，中位生存期不到一年。然而，随着细胞减灭术和局部区域化疗的出现，生存率有了显著提高。甚至最近，随着对MPM分子发病机制的认识不断加深，有报道称新型疗法的疗效有所改善。鉴于MPM灾难性的自然病程、有限的数据以及缺乏通用的治疗指南，对MPM的过去、现在和未来进行深入回顾对于改善治疗方案以及随后改善患者预后至关重要。
