Malignant mesothelioma is a rare but aggressive tumor, with a high misdiagnosis rate and overall bleak prognosis. In 0.7% of all cases, the origin is the pericardium.

The present study is a review of the literature published in recent decades focusing on the advances in clinical manifestations, radiological findings, diagnosis, differential diagnosis, and treatment of malignant pericardial mesothelioma (MPM).

No clear relationship has been established between the etiologies and the development of MPM. Clinical symptoms and signs are nonspecific when present. The main presentations are chest pain and dyspnea. Imaging plays an important role in the detection, characterization, staging, and posttreatment follow-up. The definitive diagnosis is made on the basis of pathological findings. Chest radiography and echocardiography are common techniques used initially, but their roles are limited. Computed tomography and magnetic resonance imaging have an advantage in depicting the thickened pericardium, mediastinal lymph node, tumor, and the extension of adjacent structures. Surgery is the most important treatment modality and remains palliative in most cases, while the roles of chemo- and radiotherapy are unsatisfactory.

Clinical trials of malignant pleural and peritoneal mesothelioma remain important for MPM management. Multimodality treatment of surgery, chemotherapy, radiotherapy, and immunotherapy is expected to have a role in the treatment of MPM.