Details

Autor(en) / Beteiligte

• Porubsky, Stefan
• Popovic, Zoran V
• Badve, Sunil
• Banz, Yara
• Berezowska, Sabina
• Borchert, Dietmar
• Brüggemann, Monika
• Gaiser, Timo
• Graeter, Thomas
• Hollaus, Peter
• Huettl, Katrin S
• Kotrova, Michaela
• Kreft, Andreas
• Kugler, Christian
• Lötscher, Fabian
• Möller, Burkhard
• Ott, German
• Preissler, Gerhard
• Roessner, Eric
• Rosenwald, Andreas
• Ströbel, Philipp
• Marx, Alexander

Titel

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

Ist Teil von

• Cancers, 2021-01, Vol.13 (2), p.315

Ort / Verlag

Switzerland: MDPI AG

Erscheinungsjahr

2021

Link zum Volltext

Quelle

EZB Electronic Journals Library

Beschreibungen/Notizen

• 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 ( = 4, 11%), rheumatoid arthritis ( = 3, 8%), myasthenia gravis ( = 2, 6%), asthma ( = 2, 6%), scleroderma, Sjögren syndrome, pure red cell aplasia, Grave's disease and anti-IgLON5 syndrome (each = 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.