Thymic malignancies focusing on systemic treatment

Malignancies originating from thymic epithelial tissue are rare and treatment approach depends on the individual situation. There are no randomized trials that provide evidence of therapy for patients with thymoma and thymic carcinoma. Treatment includes surgery, radiation therapy, and systemic therapy. For early stage tumors, surgery is the treatment of choice. Long-term survival depends on histologic type, presence of invasion, and quality of surgical resection. For locally advanced thymomas and thymic carcinomas, multimodal treatment includes neoadjuvant chemotherapy and adjuvant radiation. Patients with metastatic tumors are mainly treated with palliative chemotherapy. The optimal chemotherapy combination has not yet been established, although platinum-based drug regimes show reasonable response rates. For patients with poor performance status, the somatostatin (SST) analog octreotide with high affinity for SST receptors could be a potential treatment alternative. Small trials showed tumor responses, especially in combination with prednisolone. In the last decade, an effort has been made to establish targeted agents in the treatment of thymomas and thymic carcinomas. Nevertheless, up to now these agents have had only limited activity in this rare malignancy.