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Thymic hyperplasia with lymphoepithelial sialadenitis (LESA)-like features: Strong association with lymphomas and non-myasthenic autoimmune diseases

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 re...

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Main Authors: Porubský, Štefan (Author) , Popovic, Zoran V. (Author) , Badve, Sunil (Author) , Banz, Yara (Author) , Berezowska, Sabina (Author) , Borchert, Dietmar (Author) , Brüggemann, Monika (Author) , Gaiser, Timo (Author) , Graeter, Thomas (Author) , Hollaus, Peter (Author) , Huettl, Katrin S. (Author) , Kotrova, Michaela (Author) , Kreft, Andreas (Author) , Kugler, Christian (Author) , Lötscher, Fabian (Author) , Möller, Burkhard (Author) , Ott, German (Author) , Preißler, Gerhard (Author) , Rößner, Eric (Author) , Rosenwald, Andreas (Author) , Ströbel, Philipp (Author) , Marx, Alexander (Author)
Format: Article (Journal)
Language:English
Published: 16 January 2021
In: Cancers
Year: 2021, Volume: 13, Issue: 2, Pages: 1-13
ISSN:2072-6694
DOI:10.3390/cancers13020315
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.3390/cancers13020315
Verlag, lizenzpflichtig, Volltext: https://www.mdpi.com/2072-6694/13/2/315
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Author Notes:Stefan Porubsky, Zoran V. Popovic, Sunil Badve, Yara Banz, Sabina Berezowska, Dietmar Borchert, Monika Brüggemann, Timo Gaiser, Thomas Graeter, Peter Hollaus, Katrin S. Huettl, Michaela Kotrova, Andreas Kreft, Christian Kugler, Fabian Lötscher, Burkhard Möller, German Ott, Gerhard Preissler, Eric Roessner, Andreas Rosenwald, Philipp Ströbel and Alexander Marx

MARC

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520 |a 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. 
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