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IgG4-related disease in thymus. A very rare case of chronic fibrosis mimicking sarcoidosis

Abstract

IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated chronic fibroinflammatory condition, with unclear certain etiology. It is morphologically characterized by storiform fibrosis, dense IgG4-positive lymphoplasmacytic infiltrate, and obliterative phlebitis. It was recognized as a systemic condition as recently as 2003. IgG4-RD has been described in virtually every organ, forming sclerosing masses, and often mimicking tumors. Clinically, patients present unspecific symptoms and this condition is often recognized incidentally. The epidemiology remains poorly studied, but it has been noted that in the majority of recorded instances, patients are middle-aged men. IgG4-RD could mimic conditions other than tumors, such as infection, inflammation, or other systemic disorders. To ensure accuracy of diagnosis, an exhaustive histopathological analysis is required, together with clinical, radiological, and serological data. Thymic fibrosis in the absence of other primary thymic lesions is a very rare occurrence; in English literature only 1 case has been reported with scattered IgG4 plasma cells infiltrate and focal obliterative phlebitis. We will describe, for the first time, the case of a 49-year-old man displaying an anterior mediastinic, hilar, and intramyocardial mass simulating a sarcoidosis, with a definitive diagnosis of IgG4-related thymic fibrosis extending to the mediastinum and the heart. At the histological examination, we found many features of IgG4-RD in the thymic tissue, such as diffused storiform fibrosis, dense lymphoplasmacytic infiltrate with abundant plasma cells IgG4 positive (ratio IgG/IgG4: 40%), obliterative phlebitis, eosinophilic infiltrate, and Castleman-like lymphoid follicles. We discussed the differential diagnosis and reviewed the literature and the other cases of IgG4-related diseases that had been diagnosed in our department.

Tumori 2017; 103(Suppl. 1): e19 - e24

Article Type: CASE REPORT

DOI:10.5301/tj.5000687

Authors

Sara Simonetti, Noelia Pérez Muñoz, Josefa López Vivancos, Lluís Sanchez Sitjes, Javier Cesar Herranz Pérez, Nelson Leal Bohorquez, José Antonio Maestre Alcacer, Inessa Koptseva de García, Miguel Ángel Carrasco García

Article History

Disclosures

Financial support: No grants or funding have been received for this study.
Conflict of interest: None of the authors has financial interest related to this study to disclose.

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Authors

Affiliations

  • Department of Pathology, University General Hospital of Catalonia, International University of Catalonia, Sant Cugat del Vallés, Barcelona - Spain
  • Department of Internal Medicine, University General Hospital of Catalonia, International University of Catalonia, Sant Cugat del Vallés, Barcelona - Spain
  • Department of Radiology, University General Hospital of Catalonia, International University of Catalonia, Sant Cugat del Vallés, Barcelona - Spain
  • Department of Thoracic Surgery, University General Hospital of Catalonia, Sant Cugat del Vallés, Barcelona - Spain

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