Diffuse thymic fibrosis: Histologic pattern of injury or distinct entity? Journal Article


Authors: Shilo, K.; Mani, H.; Deshpande, C.; Ozbudak, I. H.; Travis, W. D.; Galvin, J. R.; Franks, T. J.
Article Title: Diffuse thymic fibrosis: Histologic pattern of injury or distinct entity?
Abstract: Thymic fibrosis in the absence of another primary thymic lesion, such as a neoplasm or cyst, is unusual. We identified 6 cases of primary extensive thymic fibrosis that developed in 3 men and 3 women, aged 28 to 60 years, mean 48 years. Patients had limited or no symptoms. Two patients had dyspnea and 1 had cough and hemoptysis. One female patient had myasthenia gravis. The lesions measured 3.5 to 17 cm in greatest dimension, mean 8 cm, and were confined to the anterior mediastinum as determined by imaging, intraoperative notes, and/or gross examination. All cases showed diffuse fibrosis with variable collagen deposition, lymphoplasmacytic infiltrates, and involution/atrophy of thymus. One case had rare IgG4-positive plasma cells and focal obliterative phlebitis. The histology showed overlap with that of IgG4-related sclerosing disease, which to our knowledge has not been documented earlier in this location. Although the etiology of the lesions is undetermined, altered immunity and/or infection may play a role. Copyright © 2010 by Lippincott Williams & Wilkins.
Keywords: adult; middle aged; metabolism; pathology; fibrosis; thymus; thymus gland; collagen; myasthenia gravis; injury; lymphatic system disease; igg4; lymphatic diseases; fibrosing mediastinitis; hyalinizing granuloma; thymic fibrosis
Journal Title: American Journal of Surgical Pathology
Volume: 34
Issue: 2
ISSN: 0147-5185
Publisher: Lippincott Williams & Wilkins  
Date Published: 2010-02-01
Start Page: 211
End Page: 215
Language: English
DOI: 10.1097/PAS.0b013e3181c91301
PUBMED: 20087160
PROVIDER: scopus
DOI/URL:
Notes: --- - "Export Date: 20 April 2011" - "CODEN: AJSPD" - "Source: Scopus"
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  1. William D Travis
    715 Travis