Current Concepts in the Diagnosis and Management of Patients With Malignant Peritoneal Mesothelioma

Abstract

Malignant peritoneal mesothelioma (MPM) is a malignancy that arises from the mesothelial lining of the abdominal cavity and largely manifests as a diffuse process. There are two hallmark features of MPM. First, MPM may progress and present itself diversely among patients. While some patients may endure a quick tumor progression that is refractory to seemingly successful initial therapeutic cytoreductive surgery, other patients many survive several years despite the presence of disease. Second, the disease usually progresses in the abdominal cavity with clinically relevant systemic metastases being rare and therefore patients suffer morbidity and mortality from loco-regional disease progression. When MPM disseminates outside the abdomen, it only occurs in the setting of advanced intra-abdominal disease. The majority of patients present with nonspecific signs and symptoms, which often results in a diagnosis of MPM when the condition is already fairly advanced. As the diagnosis is often made late, patients who are treated with only supportive care have a median survival of less than one year. The combination of systemic cisplatin with pemetrexed has an overall response rate of approximately 25%. However, as primary therapy, these agents have not been shown to meaningfully alter the natural history of the disease. Operative cytoreduction and regional chemotherapy administered as hyperthermic intraoperative peritoneal chemotherapy or early postoperative intraperitoneal chemotherapy has been found to improve survival in appropriately selected patients.