**Abstract**

Malignancies of the thymus are a rare entity and are often without local symptoms. However, paraneoplastic syndromes can give symptoms varying from very mild to life-threatening. The diagnostic workup and management of these tumors warrant a multidisciplinary approach. Treatment choice is mainly decided upon by pathological World Health Organization (WHO) subtype and clinical staging. In contrast to historical belief, biopsy could be considered when indicated. For resectable tumors, surgical approach is advised, with adjuvant radiotherapy for Masaoka-Koga stage III tumors. Whether Masaoka-Koga stage II tumors should be treated with radiotherapy is controversial given different outcomes in multiple studies. In Masaoka-Koga stage III, combinations with induction chemotherapy are the standard. A surgical approach should be considered even in stage IVa disease. If distant metastases are present, the patient can be treated with systemic chemotherapy. Despite many phase II studies having been published, there is no randomized controlled phase III data regarding optimal treatment available. In addition to chemotherapy, sunitinib and octreotide have been described to be effective. Immunotherapy is seen as Pandora's box given the possibility of immune-related side effects in this immunological organ. All known data regarding immunotherapy will be discussed.

**Keywords:** thymoma, thymic carcinoma, autoimmune paraneoplastic syndromes, surgery, radiation, chemotherapy, immunotherapy, targeted therapy
