TY  - JOUR
A1  - Porubsky, Stefan
A1  - Popovic, Zoran V.
A1  - Badve, Sunil
A1  - Banz, Yara
A1  - Berezowska, Sabina
A1  - Borchert, Dietmar
A1  - Brüggemann, Monika
A1  - Gaiser, Timo
A1  - Graeter, Thomas
A1  - Hollaus, Peter
A1  - Huettl, Katrin S.
A1  - Kotrova, Michaela
A1  - Kreft, Andreas
A1  - Kugler, Christian
A1  - Lötscher, Fabian
A1  - Möller, Burkhard
A1  - Ott, German
A1  - Preissler, Gerhard
A1  - Roessner, Eric
A1  - Rosenwald, Andreas
A1  - Ströbel, Philipp
A1  - Marx, Alexander
T1  - Thymic hyperplasia with lymphoepithelial sialadenitis (LESA)-like features: strong association with lymphomas and non-myasthenic autoimmune diseases
JF  - Cancers
N2  - 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.
KW  - autoimmune disease
KW  - imaging
KW  - LESA
KW  - lymphoma
KW  - myasthenia
KW  - pathology
KW  - surgery
KW  - thymus
KW  - thymic epithelial tumor
KW  - thymitis
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-223049
SN  - 2072-6694
VL  - 13
IS  - 2
ER  -