**Abstract**

Malignant pleural mesothelioma (MPM) is a neoplasm strongly associated with past exposure to asbestos. In general, the prognosis of patients with MPM is poor; however, in recent years, some encouraging results have been reported for systemic therapies for MPM. In a randomized phase III study, the combination of nivolumab and ipilimumab improved overall survival, compared to the standard platinum-based chemotherapy. An important clinical issue is whether the outcome of patients with MPM might be further improved by combining immunotherapies with cytotoxic chemotherapy and/or angiogenesis inhibitors. This chapter covers recent findings on systemic therapies, including cytotoxic chemotherapy, anti-angiogenic inhibitors, and/or immune checkpoint inhibitors.

**Keywords:** anti-angiogenic inhibitors, asbestos, immune checkpoint inhibitors, Ipilimumab, nivolumab
