Peritoneal Mesothelioma: Diagnosis and Management

Peritoneal mesothelioma (PM) is an uncommon and locally aggressive tumor encompassing a wide spectrum of biological behaviors, from clinically indolent to rapidly fatal disease. PM has been historically treated with debulking (DBK) surgery and/or palliative systemic chemotherapy (sCT), resulting in a median survival of only 1 year in the malignant variants. The biology of this disease has been poorly understood until recent years when mechanisms of resistance to therapy and new potential molecular therapeutic targets have been thoroughly investigated. Pathological and histological classifications of PM are still evolving. The clinical management of these conditions has improved during the last 30 years with the advent of a comprehensive treatment approach involving cytoreductive surgery (CRS) and intraperitoneal drug administration, such as hyperthermic intraperitoneal chemotherapy (HIPEC), resulting in increased patient survival. This review addresses relevant clinical and biological issues of PM, including molecular features, diagnosis, pathology, role of modern systemic and targeted therapies, patient selection for combined surgical treatment, surgical technique, and prognostic factors.