G Dobos1,2,3, A de Masson1,2,3, C Ram-Wolff1,2,3, M Beylot-Barry4, A Pham-Ledard4, N Ortonne5, S Ingen-Housz-Oro6, M Battistella2,3,7, M d'Incan8, J Rouanet9, F Franck9, M D Vignon-Pennamen7, N Franck10, A Carlotti11, S Boulinguez12, L Lamant13, T Petrella14, S Dalac15, P Joly16, P Courville17, J Rivet4,5, O Dereure18, F Amatore19, S Taix20, F Grange21, A Durlach22, G Quéreux23, N Josselin24, I Moulonguet1, L Mortier25, R Dubois26, E Maubec27, L Laroche27, L Michel2,3, I Templier28, S Barete29, C Nardin30, O Augereau31, B Vergier31, M Bagot1,2,3. 1. Dermatology Department, Saint-Louis Hospital, AP-HP, Paris, France. 2. INSERM U976, Human Immunology Pathophysiology and Immune Therapies, Paris, France. 3. Université de Paris, Paris, France. 4. Dermatology Department, Université de Bordeaux, Bordeaux, France. 5. Pathology Department, Henri Mondor Hospital, INSERM U955, Université Paris Est, AP-HP, Paris, France. 6. Dermatology Department, Henri Mondor Hospital, INSERM U955, Université Paris Est, AP-HP, Paris, France. 7. Pathology Department, Saint-Louis Hospital, AP-HP, Paris, France. 8. Dermatology and Cutaneous Oncology, Estaing University Hospital, Université de Clermont-Ferrand, Clermont-Ferrand, France. 9. Pathology Department, NHE Clermont et SIPATH UNILABS, Clermont-Ferrand, France. 10. Dermatology Department, Cochin Hospital, AP-HP, Paris, France. 11. Pathology Department, Cochin Hospital, AP-HP, Paris, France. 12. Dermatology Department, Cahors Hospital, Toulouse, France. 13. Pathology and Cytopathology Department, Institut Universitaire du Cancer Toulouse, Toulouse, France. 14. Pathology Department, Dijon Bourgogne University Hospital, Dijon, France. 15. Dermatology Department, Dijon Bourgogne University Hospital, Dijon, France. 16. Dermatology Department, Rouen University Hospital, INSERM U1234, Rouen Institute for Research and Innovation in Biomedicine, Rouen, France. 17. Pathology Department, Rouen University Hospital, INSERM U1234, Rouen Institute for Research and Innovation in Biomedicine, Rouen, France. 18. Dermatology Department, Montpellier University Hospital, Montpellier, France. 19. Department of Dermatology and Oncodermatology, Aix-Marseille University, AP-HM, Timone Hospital, Marseille, France. 20. Department of Pathology, Aix-Marseille University, AP-HM, Timone Hospital, Marseille, France. 21. Dermatology Department, Robert Debré University Hospital, Reims, France. 22. Pathology Department, Reims University Hospital, Reims, France. 23. Dermatology Department, Nantes University Hospital, Nantes, France. 24. Pathology Department, Nantes University Hospital, Nantes, France. 25. Dermatology Department, Claude Huriez University Hospital, INSERM U1189, Université de Lille, Lille, France. 26. Pathology Department, Claude Huriez University Hospital, Lille, France. 27. Dermatology Department, Avicenne Hospital, AP-HP, Paris, France. 28. Dermatology Department, Grenoble University Hospital, Grenoble, France. 29. Dermatology Department, Pité-Salpêtrière Hospital, AP-HP, Paris, France. 30. Dermatology Department, Minjoz Hospital, Besancon, France, INSERM U1098, University of Franche Comté, EFS Bourgogne Franche-Comté and Franche-Comté University, Besançon, France. 31. Pathology Department, Saint-André Hospital, Bordeaux University Hospital, INSERM U1053 - UMR BaRITOn, Eq 3 Oncogenèse des Lymphomes Cutanés, Université de Bordeaux, Bordeaux, France.
Abstract
BACKGROUND: Primary cutaneous lymphomas (PCLs) are a heterogeneous group of T-cell (CTCL) and B-cell (CBCL) malignancies. Little is known about their epidemiology at initial presentation in Europe and about potential changes over time. OBJECTIVES: The aim of this retrospective study was to analyse the frequency of PCLs in the French Cutaneous Lymphoma Registry (GFELC) and to describe the demography of patients. METHODS: Patients with a centrally validated diagnosis of primary PCL, diagnosed between 2005 and 2019, were included. RESULTS: The calculated incidence was unprecedently high at 1·06 per 100 000 person-years. The number of included patients increased yearly. Most PCL subtypes were more frequent in male patients, diagnosed at a median age of 60 years. The relative frequency of rare CTCL remained stable, the proportion of classical mycosis fungoides (MF) decreased, and the frequency of its variants (e.g. folliculotropic MF) increased. Similar patterns were observed for CBCL; for example, the proportion of marginal-zone CBCL increased over time. CONCLUSIONS: Changes in PCL frequencies may be explained by the emergence of new diagnostic criteria and better description of the entities in the most recent PCL classification. Moreover, we propose that an algorithm should be developed to confirm the diagnosis of PCL by central validation of the cases.
BACKGROUND:Primary cutaneous lymphomas (PCLs) are a heterogeneous group of T-cell (CTCL) and B-cell (CBCL) malignancies. Little is known about their epidemiology at initial presentation in Europe and about potential changes over time. OBJECTIVES: The aim of this retrospective study was to analyse the frequency of PCLs in the French Cutaneous Lymphoma Registry (GFELC) and to describe the demography of patients. METHODS:Patients with a centrally validated diagnosis of primary PCL, diagnosed between 2005 and 2019, were included. RESULTS: The calculated incidence was unprecedently high at 1·06 per 100 000 person-years. The number of included patients increased yearly. Most PCL subtypes were more frequent in male patients, diagnosed at a median age of 60 years. The relative frequency of rare CTCL remained stable, the proportion of classical mycosis fungoides (MF) decreased, and the frequency of its variants (e.g. folliculotropic MF) increased. Similar patterns were observed for CBCL; for example, the proportion of marginal-zone CBCL increased over time. CONCLUSIONS: Changes in PCL frequencies may be explained by the emergence of new diagnostic criteria and better description of the entities in the most recent PCL classification. Moreover, we propose that an algorithm should be developed to confirm the diagnosis of PCL by central validation of the cases.
Authors: Zhuo Ran Cai; Michael L Chen; Martin A Weinstock; Youn H Kim; Roberto A Novoa; Eleni Linos Journal: JAMA Oncol Date: 2022-09-01 Impact factor: 33.006