Occasional detection of thymic epithelial tumor 4 years after diagnosis of adult onset Still disease: A challenging case report and immuno-oncological considerations coming from pertinent literature review

• Published in: Medicine (Baltimore) 2016-09, Vol.95 (36), p.e4357-e4357
• Main Authors: Lococo, Filippo, Bajocchi, Gianluigi, Caruso, Andrea, Valli, Riccardo, Ricchetti, Tommaso, Sgarbi, Giorgio, Salvarani, Carlo
• Format: Article
• Language: English
• Subjects: Clinical Case Report; Humans; Male; Middle Aged; Still's Disease, Adult-Onset - complications; Thymoma - immunology; Thymus Neoplasms - immunology
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000004357

Thymoma is a T cell neoplasm arising from the thymic epithelium that due to its immunological role, frequently undercover derangements of immunity such a tumors and autoimmune diseases. Herein, we report, to the best of our knowledge, the first description of an association between thymoma and adult onset Still disease (AOSD) in a 47-year-old man. The first one was occasionally detected 4 years later the diagnosis of AOSD, and surgically removed via right lateral thoracotomy. Histology confirmed an encapsulated thymic tumor (type AB sec. WHO-classification). The AOSD was particularly resistant to the therapy, requiring a combination of immunosuppressant followed by anti-IL1R, that was the only steroids-sparing treatment capable to induce and maintain the remission. The differential diagnosis was particularly challenging because of the severe myasthenic-like symptoms that, with normal laboratory tests, were initially misinterpreted as fibromyalgia. The pathogenic link of this association could be a thymus escape of autoreactive T lymphocytes causing autoimmunity. Clinicians should be always include the possibility of a thymoma in the differential diagnosis of an unusual new onset of weakness and normal laboratories data, in particular once autoimmune disease is present in the medical history.