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,通常局限于腹腔内。该疾病具有侵袭性自然病程,其发病率和死亡率源于腹膜腔内进行性局部区域影响。首例病例报道于20世纪初,但由于该疾病的罕见性以及对临床病理学影响认识的巨大空白,后续MPM病例的报道直至五年后才得以发表。此后,对该疾病的认识呈指数级增长,然而目前仍缺乏前瞻性数据,且关于治疗管理的文献稀少。传统上,患者接受全身性治疗,预后极差,中位生存期不足一年。但随着细胞减灭术和局部区域化疗的应用,患者生存期已得到显著改善。近年来,随着对MPM分子发病机制认识的深化,已有报道显示新型疗法可改善患者预后。鉴于MPM的自然病程凶险、现有数据有限且缺乏统一治疗指南,深入回顾MPM的过去、现在与未来,对于改进治疗方案并最终提升患者预后至关重要。