Systemic treatment for thymic malignancies.

Abstract

The management of thymic epithelial tumors is a paradigm of multidisciplinary collaboration. Chemotherapy may be administered as part of curative-intent sequential strategy integrating subsequent surgery or radiotherapy, or as an exclusive treatment if local treatment is not achievable. Recurrences of thymic epithelial tumors should be managed according to the same strategy as newly diagnosed tumors. More options have become available for advanced, refractory, and recurrent thymic epithelial tumors, which include cytotoxic agents such as carboplatin-paclitaxel, pemetrexed, and oral etoposide. Angiogenesis targeting is a standard in advanced lines of treatment, after results of a phase II trial with sunitinib were reported. Ongoing studies are assessing the opportunity of targeting the immune-response checkpoint programmed death-1/programmed death ligand-1, with preliminary promising results whereas safety, with a higher risk of auto-immunity, may represent a concern. Overall, a dramatic improvement in our knowledge of the management of thymic tumors has occurred in the past few years, resulting in the development of databases, translational research programmes, and clinical trials. Although access to innovative strategies represents a major challenge, as the rarity of the tumor precludes specific approval of drugs to be obtained, patient-centered initiatives, such as the establishment of dedicated networks, are warranted.