Malignant pleural mesothelioma (MPM) is an aggressive malignancy with a 5-year survival rate of similar to 10%. Since most patients present with irresectable disease, the vast majority is treated... Show moreMalignant pleural mesothelioma (MPM) is an aggressive malignancy with a 5-year survival rate of similar to 10%. Since most patients present with irresectable disease, the vast majority is treated with chemotherapy. The only registered therapy for MPM is platinum-pemetrexed doublet therapy, although only up to half of patients have clinical benefit from this palliative treatment. Of the anti-angiogenesis agents, only bevacizumab and nintedanib have shown activity with platinum-pemetrexed doublet therapy. Other anti-angiogenesis agents like thalidomide did not prolong (progression free) survival or response rate. Eventually, all patients will get a recurrence and no active second line therapy has been identified to date. The clinical benefit of (switch) maintenance therapy after first line treatment and combination strategies of different chemotherapies with angiogenesis inhibitors are currently under investigation. The major challenges are finding optimal treatment combinations and to select the adequate treatment for an individual patient. This review focusses on the current standard of chemotherapy and new systemic therapy strategies under investigation. Show less
