Key Points

• International collaborative organizations have dramatically expanded the knowledge base regarding thymic malignancies
• A reliable clinical diagnosis of thymic malignancy is usually possible for an experienced clinician on the basis of demographic features, radiographic characteristics and the clinical presentation
• All thymomas are malignant (but often only slowly progressive)
• Thymomas are frequently associated with a wide variety of autoimmune and neurologic syndromes (most commonly myasthenia gravis)
• Thymic carcinoma and neuroendocrine tumors of the thymus are more aggressive than thymoma
• Complete surgical resection is the main treatment intervention
• The role of postoperative radiotherapy after complete resection is unclear
• Multimodality treatment (e.g. preoperative chemo(radio)therapy, complete resection, adjuvant radiotherapy) for thymic malignancies that are locally advanced or manifest pleural dissemination yields reasonable long-term results
• Several active chemotherapy regimens have been identified
• When possible, resection of recurrence (plus chemo- or radiotherapy) results in reasonable long-term outcomes