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

IgG4-related disease in thymus. A very rare case of chronic fibrosis mimicking sarcoidosis. Tumori. 2017 Oct 28;:0 Authors: Simonetti S, Pérez Muñoz N, López Vivancos J, Sanchez Sitjes L, Herranz Pérez JC, Leal Bohorquez N, Maestre Alcacer JA, de García IK, Carrasco García MÁ 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 wil...
Source: Tumori - Category: Cancer & Oncology Tags: Tumori Source Type: research