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Article

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

1
Institute of Pathology, University Medical Center of the Johannes Gutenberg University Mainz, Langenbeckstraße 1, 55101 Mainz, Germany
2
Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany
3
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA
4
Institute of Pathology, University of Bern, Murtenstrasse 31, 3008 Bern, Switzerland
5
Department of Surgery, Armed Forces Hospital, Scharnhorststr.13, 10115 Berlin, Germany
6
Unit for Hematological Diagnostics, Medical Department II, University Medical Center Schleswig-Holstein, Langer Segen 8-10, 24105 Kiel, Germany
7
Department of Thoracic Surgery, Klinik Löwenstein, Geißhölzle 62, 74245 Löwenstein, Germany
8
Department of Thoracic Surgery, Katholisches Klinikum Mainz, An der Goldgrube 11, 55131 Mainz, Germany
9
Department of Clinical Pathology, Robert-Bosch-Krankenhaus, and Dr. Margarete Fischer-Bosch Institute for Clinical Pharmacology, Auerbachstraße 110-112, 70376 Stuttgart, Germany
10
Department of Thoracic Surgery, LungenClinic Großhansdorf, Wöhrendamm 80, 22927 Großhansdorf, Germany
11
Department of Rheumatology and Immunology, Inselspital, Universitätsspital Bern, Freiburgstrasse, 3010 Bern, Switzerland
12
Department of Thoracic Surgery, Robert-Bosch-Krankenhaus, Klinik Schillerhöhe, Solitudestraße 18, 70839 Gerlingen, Germany
13
Academic Thoracic Center Mainz, Division of Thoracic Surgery, University Medical Center of the Johannes Gutenberg University Mainz, Langenbeckstraße 1, 55101 Mainz, Germany
14
Institute of Pathology, University of Würzburg, Josef-Schneider-Str. 2, 97080 Würzburg, Germany
15
Institute of Pathology, University Medical Center Göttingen, University of Göttingen, Robert-Koch-Straße 40, 37075 Göttingen, Germany
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Current address: Institute of Pathology, Lausanne University Hospital and University of Lausanne Rue du Bugnon 25, 1011 Lausanne, Switzerland.
§
Member of the German Center for Lung Research (Deutsches Zentrum für Lungenforschng, DZL).
Cancers 2021, 13(2), 315; https://doi.org/10.3390/cancers13020315
Received: 21 December 2020 / Revised: 11 January 2021 / Accepted: 13 January 2021 / Published: 16 January 2021
(This article belongs to the Special Issue Novel Approaches in Thymic Epithelial Tumors Diagnosis and Treatment)
Thymic epithelial tumors and lymphomas are the most frequent mediastinal mass lesions. Thymic hyperplasia with “lymphoepithelial sialadenitis (LESA)-like features” (LESA-like TH) was initially described as one form of thymic hyperplasia and was thought not to be associated with autoimmune and lymphoproliferative diseases. Our systematic analysis of patients with LESA-like TH shows that 14% have associated lymphomas and 33% partially overlapping autoimmune diseases. This implies a hematologic and rheumatologic workup in patients with LESA-like TH. In addition, LESA-like TH should enter the list of differential diagnoses of mediastinal mass lesions, in particular in patients with 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 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. View Full-Text
Keywords: autoimmune disease; imaging; LESA; lymphoma; myasthenia; pathology; surgery; thymus; thymic epithelial tumor; thymitis autoimmune disease; imaging; LESA; lymphoma; myasthenia; pathology; surgery; thymus; thymic epithelial tumor; thymitis
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MDPI and ACS Style

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. Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features: Strong Association with Lymphomas and Non-Myasthenic Autoimmune Diseases. Cancers 2021, 13, 315. https://doi.org/10.3390/cancers13020315

AMA Style

Porubsky S, Popovic ZV, Badve S, Banz Y, Berezowska S, Borchert D, Brüggemann M, Gaiser T, Graeter T, Hollaus P, Huettl KS, Kotrova M, Kreft A, Kugler C, Lötscher F, Möller B, Ott G, Preissler G, Roessner E, Rosenwald A, Ströbel P, Marx A. Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features: Strong Association with Lymphomas and Non-Myasthenic Autoimmune Diseases. Cancers. 2021; 13(2):315. https://doi.org/10.3390/cancers13020315

Chicago/Turabian Style

Porubsky, Stefan, 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. 2021. "Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features: Strong Association with Lymphomas and Non-Myasthenic Autoimmune Diseases" Cancers 13, no. 2: 315. https://doi.org/10.3390/cancers13020315

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