Moritz Felcht1,2, Claus-Detlev Klemke1,3, Jan Peter Nicolay1, Christel Weiss4, Chalid Assaf5, Marion Wobser6, Max Schlaak7, Uwe Hillen8, Rose Moritz9,10, Iliana Tantcheva-Poor7, Dorothee Nashan11, Marc Beyer12, Edgar Dippel13, Cornelia Sigrid Lissi Müller14, Michael Max Sachse15,16, Frank Meiss17, Cyrill Géraud1,2, Alexander Marx18, Sergij Goerdt1,2, Eva Geissinger19, Werner Kempf20,21. 1. Department of Dermatology, Venereology and Allergy, University Medical Centre Mannheim, Medical Faculty Mannheim, Heidelberg University and Centre of Excellence of Dermatology of Baden-Württemberg, Mannheim, Germany. 2. European Center of Angioscience, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany. 3. Hautklinik, Städtisches Klinikum Karlsruhe, Akademisches Lehrkrankenhaus der Universität Freiburg, Karlsruhe, Germany. 4. Department of Medical Statistics, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany. 5. Department of Dermatology, Hospital of Krefeld, Krefeld, Germany. 6. Department of Dermatology, Venereology and Allergy, Julius-Maximilians-University, Würzburg, Germany. 7. Department of Dermatology, University of Cologne, Cologne, Germany. 8. Department of Dermatology and Skin Tumor Center, University Hospital Essen-Duisburg, Essen-Duisburg, Germany. 9. Department of Dermatology, Venereology and Allergy, University Hospital Bochum, Bochum, Germany. 10. Department of Dermatology and Venereology, University Hospital and Martin-Luther-University of Halle-Wittenberg, Halle (Saale), Germany. 11. Department of Dermatology, Hospital of Dortmund, Dortmund, Germany. 12. Skin Cancer Center Charité, Department of Dermatology and Allergy, Charité -Universitätsmedizin Berlin, Berlin, Germany. 13. Department of Dermatology, Hospital of Ludwigshafen, Ludwigshafen, Germany. 14. Department of Dermatology, Venereology and Allergy, Saarland University Hospital, Homburg/Saar, Germany. 15. Department of Dermatology, Allergy and Phlebology, Hospital of Bremerhaven, Bremerhaven, Germany. 16. Department of Dermatology, Hospital of Bremen, Bremen, Germany. 17. Department of Dermatology and Venereology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany. 18. Department of Pathology, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany. 19. Institute of Pathology, Julius-Maximilians-University, Würzburg, Germany. 20. Kempf and Pfaltz, Histologische Diagnostik, Zurich, Switzerland. 21. Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
Abstract
BACKGROUND AND OBJECTIVES: Primary cutaneous diffuse large B-cell lymphoma, NOS (PCLBCL/NOS) is a rare PCLBCL. Only few data are available for this tumor. The aim of this study was to identify clinical and/or immunohistochemical markers (in addition to Bcl-2) that characterize PCLBCL/NOS, assist in differentiating it from PCLBCL, leg type (PCLBCL/LT) and help to assess the clinical course/prognosis. PATIENTS AND METHODS: Bcl-2- PCLBCL/NOS) cases (n = 14 were compared with Bcl-2+ PCLBCL/LT cases (n = 29). RESULTS: PCLBCL/NOS patients were younger, predominantly male and had better survival rates than patients with PCLBCL/LT. Patients with PCLBCL/NOS presented more often with larger plaques limited to one or two contiguous body regions, whereas PCLBCL/LT cases often presented with disseminated lesions. Neoplastic cells had a higher proliferation rate (Ki67) in PCLBCL/LT patients. The tumor microenvironment of PCLBCL/NOS had a more prominent CD3+ infiltrate. Overall survival data for the whole cohort (n = 37) revealed that female gender and Bcl-2 expression correlated with a worse survival rate. Bcl-6 expression and centroblastic subtype correlated with better outcomes. None of the other markers studied (e.g. GCB/non-GCB subtype) correlated with survival rate. CONCLUSIONS: PCLBCL/NOS and PCLBCL/LT differ in their clinical behavior and outcomes. Bcl-2 still seems to be the best marker for discriminating between these two subgroups. Bcl-2, female gender and Bcl-6 represent prognostic markers for PCLBCL.
BACKGROUND AND OBJECTIVES: Primary cutaneous diffuse large B-cell lymphoma, NOS (PCLBCL/NOS) is a rare PCLBCL. Only few data are available for this tumor. The aim of this study was to identify clinical and/or immunohistochemical markers (in addition to Bcl-2) that characterize PCLBCL/NOS, assist in differentiating it from PCLBCL, leg type (PCLBCL/LT) and help to assess the clinical course/prognosis. PATIENTS AND METHODS: Bcl-2- PCLBCL/NOS) cases (n = 14 were compared with Bcl-2+ PCLBCL/LT cases (n = 29). RESULTS:PCLBCL/NOSpatients were younger, predominantly male and had better survival rates than patients with PCLBCL/LT. Patients with PCLBCL/NOS presented more often with larger plaques limited to one or two contiguous body regions, whereas PCLBCL/LT cases often presented with disseminated lesions. Neoplastic cells had a higher proliferation rate (Ki67) in PCLBCL/LT patients. The tumor microenvironment of PCLBCL/NOS had a more prominent CD3+ infiltrate. Overall survival data for the whole cohort (n = 37) revealed that female gender and Bcl-2 expression correlated with a worse survival rate. Bcl-6 expression and centroblastic subtype correlated with better outcomes. None of the other markers studied (e.g. GCB/non-GCB subtype) correlated with survival rate. CONCLUSIONS:PCLBCL/NOS and PCLBCL/LT differ in their clinical behavior and outcomes. Bcl-2 still seems to be the best marker for discriminating between these two subgroups. Bcl-2, female gender and Bcl-6 represent prognostic markers for PCLBCL.