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

Autor: Fabian Lötscher, German Ott, Timo Gaiser, Peter Hollaus, Philipp Ströbel, Stefan Porubsky, Burkhard Möller, Zoran V. Popovic, Michaela Kotrova, Monika Brüggemann, Christian Kugler, Andreas Kreft, Yara Banz, Andreas Rosenwald, Sunil Badve, Dietmar Borchert, Sabina Berezowska, Gerhard Preissler, Katrin S. Huettl, Alexander Marx, Eric Dominic Roessner, Thomas Graeter
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Cancers, vol. 13, no. 2, pp. 315
Cancers
Volume 13
Issue 2
Cancers, Vol 13, Iss 315, p 315 (2021)
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; ... (2021). Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features: Strong Association with Lymphomas and Non-Myasthenic Autoimmune Diseases. Cancers, 13(2) MDPI AG 10.3390/cancers13020315
Popis: 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&ndash
80
lesion diameter 7.0 cm, 1&ndash
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, Sjö
gren syndrome, pure red cell aplasia, Grave&rsquo
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.
Databáze: OpenAIRE