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Thymomas are fascinating tumors because of their multifaceted clinical presentation, including an unrivaled frequency of associated paraneoplastic autoimmune diseases and an astounding histologic heterogeneity. Thus, thymomas challenge the clinician, pathologist, and immunologist alike with complex diagnostic and therapeutic problems in almost all fields of medicine, radiology, nuclear medicine, surgery, radio-oncology, and pathology. This review highlights recent progress in these fields, including the new World Health Organization’s classification of thymomas, and reports on the beginning discovery of the genetic basis of thymoma oncogenesis and the cellular and molecular immunopathology of thymoma-associated paraneoplastic autoimmunity.

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