Malignant Peritoneal Mesothelioma: An In-Depth and Up-to-Date Review of Pathogenesis, Diagnosis, Management and Future Directions

SIMPLE SUMMARY: Mesothelioma is a malignancy of mesothelial tissues. The majority arise from the pleura, but approximately thirty percent of cases arise from the peritoneum. Malignant peritoneal mesothelioma was previously a largely unknown disease, and the extremely poor survival rate reflected that. However, more recently, our understanding has improved, and so has survival. There is, however, an urgent need to investigate further to better understand the disease process, tumour biology, and molecular pathogenesis to establish gold-standard therapeutic strategies. This is, therefore, an in-depth review of the current literature reporting malignant peritoneal mesothelioma with the objective of establishing areas for future research. ABSTRACT: 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.