Thymomas and thymic carcinomas are rare diseases of the anterior mediastinum. Although some thymomas are quite indolent and able to be resected in a curative fashion, the treatment of metastatic disease remains a challenge, especially for the more aggressive thymic carcinoma histology. Based on the results of single-arm trials, combination chemotherapy is the standard of care in the first-line, and anthracycline-based treatments should be used if patients are reasonably fit. Several single-agent cytotoxic chemotherapy agents have some effectiveness in subsequent lines of therapy, especially pemetrexed and, in octreotide scan-positive patients, octreotide. Prospective trials of new agents are difficult to conduct given the rarity of thymoma, but various targeted therapies do show promise. Greater international research collaboration, as well as modern techniques in molecular and genomic characterization, should help to advance the treatment of thymic malignancies in the near future.
Treatment updates in advanced thymoma and thymic carcinoma