Future Perspective of Chemotherapy and Pharmacotherapy in Thymic Carcinoma

SIMPLE SUMMARY: Thymic carcinoma is a rare cancer, and its biology remains largely unknown. Although complete surgical resection is a standard treatment for thymic carcinoma, systemic chemotherapy is frequently administered in metastatic or recurrent cases. Given the rarity, therapeutic agents are often confirmed on the basis of the results of phase II trials or retrospective studies. Platinum-based combination chemotherapy has long been employed for treating thymic carcinoma. Recently, biomarkers have been explored, and molecular profiles and major oncogenic pathways have gradually been revealed by next-generation sequencing, resulting in the development of targeted therapies. Moreover, clinical trials assessing combination therapy with immune checkpoint inhibitors are ongoing and are expected to be efficacious for treating thymic epithelial tumors. We reviewed the current role of systemic chemotherapy, including targeted therapies and immune checkpoint inhibitors, considering recent findings regarding its biology. ABSTRACT: Thymic carcinoma is a rare cancer that arises from thymic epithelial cells. Its nature and pathology differ from that of benign thymoma, presenting a poorer prognosis. If surgically resectable, surgery alone or surgery followed by chemoradiotherapy or radiotherapy is recommended by the National Comprehensive Cancer Network Guidelines. Metastatic and refractory thymic carcinomas require systemic pharmacotherapy. Combined carboplatin and paclitaxel, and cisplatin and anthracycline-based regimens have been shown a fair response rate and survival to provide a de facto standard of care when compared with other drugs employed as first-line chemotherapy. Cytotoxic agents have been pivotal for treating thymic carcinoma, as little is known regarding its tumorigenesis. In addition, genetic alterations, including driver mutations, which play an important role in treatments, have not yet been discovered. However, molecular pathways and biomarker studies assessing thymic epithelial tumors have been reported recently, resulting in the development of new agents, such as molecular targeted agents and immune checkpoint inhibitors. As treatment options are currently limited and the prognosis remains poor in metastases and recurrent thymic carcinoma, genetic alterations need to be assessed. In the present review, we focused on the current role of targeted therapies and immune checkpoint inhibitors in treating thymic carcinoma.