{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T20:21:47Z","timestamp":1792182107767,"version":"4.4.0"},"reference-count":58,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2020,10,11]],"date-time":"2020-10-11T00:00:00Z","timestamp":1602374400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100007075","name":"European Society for Medical Oncology","doi-asserted-by":"publisher","award":["Translational Research Fellowship 2019"],"award-info":[{"award-number":["Translational Research Fellowship 2019"]}],"id":[{"id":"10.13039\/501100007075","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of rare diseases. Many studies have reported on local epidemiology or geographic clustering, however we lack information from a global perspective. A systematic review and meta-analysis was conducted in Medline and the Cochrane Library based on a previously registered protocol and according to the preferred reporting of items for systematic reviews and meta-analyses (PRISMA). We selected publications that enrolled at least 100 patients with primary cutaneous lymphomas according to the current classifications. The relative frequencies (proportions) of subtypes were compared between studies and geographic regions in a meta-analysis. In total, 26 studies met our inclusion criteria, reporting on altogether 16,953 patients. Within primary cutaneous lymphomas, CTCL appeared to be 15% more frequent in Asian populations. Mycosis fungoides (MF) accounted for 62% of CTCL, with an important heterogeneity in frequencies between studies and continents. The proportion of S\u00e9zary syndrome (SS) was 3%, stable worldwide. Rare CTCL, such as NK\/T-cell lymphoma or subcutaneous panniculitis-like lymphoma, were more frequent in Asian studies. This global meta-analysis of CTCL confirmed the predominance of CTCL among primary cutaneous lymphomas (83% on average) in the three analyzed continents, most of which were MF cases. It revealed the same proportions of SS across continents, and the heterogeneity of MF frequencies, suggesting the possible role of environmental factors in the pathophysiology of the latter. Registration number: CRD42020148295 (PROSPERO).<\/jats:p>","DOI":"10.3390\/cancers12102921","type":"journal-article","created":{"date-parts":[[2020,10,15]],"date-time":"2020-10-15T21:42:21Z","timestamp":1602798141000},"page":"2921","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":135,"title":["Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients"],"prefix":"10.3390","volume":"12","author":[{"given":"Gabor","family":"Dobos","sequence":"first","affiliation":[{"name":"Dermatology Department, Saint-Louis Hospital, AP-HP, 75010 Paris, France"},{"name":"INSERM U976 Human Immunology, Pathophysiology and Immune Therapies, Institut de Recherche Saint-Louis, 75010 Paris, France"},{"name":"Department of Medicine, Universit\u00e9 de Paris, 75010 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6104-622X","authenticated-orcid":false,"given":"Anne","family":"Pohrt","sequence":"additional","affiliation":[{"name":"Institute of Biometry and Clinical Epidemiology, Charit\u00e9-Universit\u00e4ttsmedizin, 10117 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Ram-Wolff","sequence":"additional","affiliation":[{"name":"Dermatology Department, Saint-Louis Hospital, AP-HP, 75010 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9leste","family":"Lebb\u00e9","sequence":"additional","affiliation":[{"name":"Dermatology Department, Saint-Louis Hospital, AP-HP, 75010 Paris, France"},{"name":"INSERM U976 Human Immunology, Pathophysiology and Immune Therapies, Institut de Recherche Saint-Louis, 75010 Paris, France"},{"name":"Department of Medicine, Universit\u00e9 de Paris, 75010 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-David","family":"Bouaziz","sequence":"additional","affiliation":[{"name":"Dermatology Department, Saint-Louis Hospital, AP-HP, 75010 Paris, France"},{"name":"INSERM U976 Human Immunology, Pathophysiology and Immune Therapies, Institut de Recherche Saint-Louis, 75010 Paris, France"},{"name":"Department of Medicine, Universit\u00e9 de Paris, 75010 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxime","family":"Battistella","sequence":"additional","affiliation":[{"name":"INSERM U976 Human Immunology, Pathophysiology and Immune Therapies, Institut de Recherche Saint-Louis, 75010 Paris, France"},{"name":"Department of Medicine, Universit\u00e9 de Paris, 75010 Paris, France"},{"name":"Pathology Department, Saint-Louis Hospital, AP-HP, 75010 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martine","family":"Bagot","sequence":"additional","affiliation":[{"name":"Dermatology Department, Saint-Louis Hospital, AP-HP, 75010 Paris, France"},{"name":"INSERM U976 Human Immunology, Pathophysiology and Immune Therapies, Institut de Recherche Saint-Louis, 75010 Paris, France"},{"name":"Department of Medicine, Universit\u00e9 de Paris, 75010 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7828-6211","authenticated-orcid":false,"given":"Ad\u00e8le","family":"de Masson","sequence":"additional","affiliation":[{"name":"Dermatology Department, Saint-Louis Hospital, AP-HP, 75010 Paris, France"},{"name":"INSERM U976 Human Immunology, Pathophysiology and Immune Therapies, Institut de Recherche Saint-Louis, 75010 Paris, France"},{"name":"Department of Medicine, Universit\u00e9 de Paris, 75010 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,10,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1182\/blood-2018-11-881268","article-title":"The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas","volume":"133","author":"Willemze","year":"2019","journal-title":"Blood"},{"key":"ref_2","first-page":"1174","article-title":"Clustering of mycosis fungoides in the County of Vasternorrland","volume":"74","author":"Gip","year":"1977","journal-title":"Lakartidningen"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"3550","DOI":"10.1002\/cncr.30758","article-title":"Comprehensive analysis of cutaneous T-cell lymphoma (CTCL) incidence and mortality in Canada reveals changing trends and geographic clustering for this malignancy","volume":"123","author":"Ghazawi","year":"2017","journal-title":"Cancer"},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Moreau, J.F., Buchanich, J.M., Geskin, J.Z., Akilov, O.E., and Geskin, L.J. 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