Peritoneal Mesothelioma: Clinical and Therapeutic Aspects

Mesothelioma is a very rare malignant disease that originates from mesothelial cells that line the serosa: pleura, peritoneum, pericardium, or testicular vaginal tunic. Peritoneal mesothelioma accounts for 7–10% of all mesotheliomas diagnosed, and ranks second after pleural localization of mesothelioma. The incidence of peritoneal mesothelioma is 0.5–3 cases per million in men and 0.2–2 cases per million in women. Diagnosis of peritoneal mesothelioma is difficult due to nonspecific symptoms and because of this patients present in advanced stages of the disease. Histologically there are three major categories of malignant peritoneal mesothelioma: epithelioid, sarcomatoid, and biphasic. The differential diagnosis of peritoneal mesothelioma is made with peritoneal pseudomyxoma, ovarian tumors, and peritoneal metastases from colorectal cancer. An important role in differential diagnosis, in addition to immunohistochemistry, is played by various tumor markers and genetic tests. The treatment of peritoneal mesothelioma is performed by cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), with good results for patients in the early stages of the disease. For patients with advanced disease, a new treatment has been proposed: pressurized intraperitoneal aerosol chemotherapy (PIPAC). For patients who cannot use CRS and HIPEC, the only therapeutic option remains chemotherapy (systemic + intraperitoneal).