Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features: Strong Association with Lymphomas and Non-Myasthenic Autoimmune Diseases.

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License: CC BY 4.0
Serval ID
serval:BIB_89AFEDF77F3E
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features: Strong Association with Lymphomas and Non-Myasthenic Autoimmune Diseases.
Journal
Cancers
Author(s)
Porubsky S., Popovic Z.V., Badve S., Banz Y., Berezowska S., Borchert D., Brüggemann M., Gaiser T., Graeter T., Hollaus P., Huettl K.S., Kotrova M., Kreft A., Kugler C., Lötscher F., Möller B., Ott G., Preissler G., Roessner E., Rosenwald A., Ströbel P., Marx A.
ISSN
2072-6694 (Print)
ISSN-L
2072-6694
Publication state
Published
Issued date
16/01/2021
Peer-reviewed
Oui
Volume
13
Number
2
Pages
315
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Thymic hyperplasia (TH) with lymphoepithelial sialadenitis (LESA)-like features (LESA-like TH) has been described as a tumor-like, benign proliferation of thymic epithelial cells and lymphoid follicles. We aimed to determine the frequency of lymphoma and autoimmunity in LESA-like TH and performed retrospective analysis of cases with LESA-like TH and/or thymic MALT-lymphoma. Among 36 patients (21 males) with LESA-like TH (age 52 years, 32-80; lesion diameter 7.0 cm, 1-14.5; median, range), five (14%) showed associated lymphomas, including four (11%) thymic MALT lymphomas and one (3%) diffuse large B-cell lymphoma. One additional case showed a clonal B-cell-receptor rearrangement without evidence of lymphoma. Twelve (33%) patients (7 women) suffered from partially overlapping autoimmune diseases: systemic lupus erythematosus (n = 4, 11%), rheumatoid arthritis (n = 3, 8%), myasthenia gravis (n = 2, 6%), asthma (n = 2, 6%), scleroderma, Sjögren syndrome, pure red cell aplasia, Grave's disease and anti-IgLON5 syndrome (each n = 1, 3%). Among 11 primary thymic MALT lymphomas, remnants of LESA-like TH were found in two cases (18%). In summary, LESA-like TH shows a striking association with autoimmunity and predisposes to lymphomas. Thus, a hematologic and rheumatologic workup should become standard in patients diagnosed with LESA-like TH. Radiologists and clinicians should be aware of LESA-like TH as a differential diagnosis for mediastinal mass lesions in patients with autoimmune diseases.
Keywords
LESA, autoimmune disease, imaging, lymphoma, myasthenia, pathology, surgery, thymic epithelial tumor, thymitis, thymus
Pubmed
Web of science
Open Access
Yes
Create date
10/02/2021 9:32
Last modification date
02/03/2021 6:25
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