Alistair Robson1, Chalid Assaf2, Martine Bagot3, Gunter Burg4, Eduardo Calonje1, Christine Castillo5, Lorenzo Cerroni6, Nicola Chimenti7, Pierre Dechelotte8, Frederic Franck8, Maria Geerts9, Sylke Gellrich2, John Goodlad10, Werner Kempf4, Robert Knobler11, Cesare Massone6, Chris Meijer12, Pablo Ortiz13, Tony Petrella14, Nicola Pimpinelli15, Joclim Roewert2, Robin Russell-Jones1, Marco Santucci16, Mattias Steinhoff2, Wolfram Sterry2, Janine Wechsler17, Sean Whittaker1, Rein Willemze18, Emilio Berti19. 1. St John's Institute of Dermatology, London, UK. 2. Department of Dermatology, Charité-University Medicine, Berlin, Germany. 3. Department of Pathology, Universite Paris, Paris, France. 4. Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland. 5. CHRU, Lille, France. 6. Department of Dermatology Medical, University of Graz, Graz, Austria. 7. Department of Dermatology, University of L'Aquila, Rome, Italy. 8. Department of Pathology, University of Clermont-Ferrand, Clermont-Ferrand, France. 9. Department of Dermatology, Ghent University Hospital, Gent, Belgium. 10. Department of Pathology, Western General Hospital, Edinburgh, UK. 11. Department of Dermatology, University of Vienna, Vienna, Austria. 12. Department of Pathology, VU University Medical Center, Amsterdam, the Netherlands. 13. Hospital Universitario, Universidad Complutense, Madrid, Spain. 14. Departmentof Pathology, Dijon's University Hospital, Dijon, France. 15. Division of Dermatology, University of Florence Medical School, Florence, Italy. 16. Division of Pathological Anatomy, University of Florence, Florence, Italy. 17. Department of Pathology Henri-Mondor Hospital, University Paris-Val-de-Marne, Paris, France. 18. Department of Dermatology, Leiden University, Leiden, the Netherlands. 19. Department of Dermatology, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico and Università degli Studi di Milano-Bicocca, Milan, Italy.
Abstract
AIMS: Aggressive epidermotropic cutaneous CD8(+) lymphoma is currently afforded provisional status in the WHO classification of lymphomas. An EORTC Workshop was convened to describe in detail the features of this putative neoplasm and evaluate its nosological status with respect to other cutaneous CD8(+) lymphomas. METHODS AND RESULTS: Sixty-one CD8(+) cases were analysed at the workshop; clinical details, often with photographs, histological sections, immunohistochemical results, treatment and patient outcome were discussed and recorded. Eighteen cases had distinct features and conformed to the diagnosis of aggressive epidermotropic cutaneous CD8(+) lymphoma. The patients typically present with widespread plaques and tumours, often ulcerated and haemorrhagic, and histologically have striking pagetoid epidermotrophism. A CD8(+) /CD45RA(+) /CD45RO(-) /CD2(-) /CD5(-) /CD56(-) phenotype, with one or more cytotoxic markers, was found in seven of 18 patients, with a very similar phenotype in the remainder. The tumours seldom involve lymph nodes, but mucosal and central nervous system involvement are not uncommon. The prognosis is poor, with a median survival of 12 months. Examples of CD8(+) mycosis fungoides, lymphomatoid papulosis and Woringer-Kolopp disease presented the typical features well documented in the CD4(+) forms of those diseases. CONCLUSIONS: Aggressive epidermotropic cutaneous CD8(+) lymphoma is a distinct lymphoma that warrants inclusion as a distinct entity in future revisions of lymphoma classifications.
AIMS: Aggressive epidermotropic cutaneous CD8(+) lymphoma is currently afforded provisional status in the WHO classification of lymphomas. An EORTC Workshop was convened to describe in detail the features of this putative neoplasm and evaluate its nosological status with respect to other cutaneous CD8(+) lymphomas. METHODS AND RESULTS: Sixty-one CD8(+) cases were analysed at the workshop; clinical details, often with photographs, histological sections, immunohistochemical results, treatment and patient outcome were discussed and recorded. Eighteen cases had distinct features and conformed to the diagnosis of aggressive epidermotropic cutaneous CD8(+) lymphoma. The patients typically present with widespread plaques and tumours, often ulcerated and haemorrhagic, and histologically have striking pagetoid epidermotrophism. A CD8(+) /CD45RA(+) /CD45RO(-) /CD2(-) /CD5(-) /CD56(-) phenotype, with one or more cytotoxic markers, was found in seven of 18 patients, with a very similar phenotype in the remainder. The tumours seldom involve lymph nodes, but mucosal and central nervous system involvement are not uncommon. The prognosis is poor, with a median survival of 12 months. Examples of CD8(+) mycosis fungoides, lymphomatoid papulosis and Woringer-Kolopp disease presented the typical features well documented in the CD4(+) forms of those diseases. CONCLUSIONS: Aggressive epidermotropic cutaneous CD8(+) lymphoma is a distinct lymphoma that warrants inclusion as a distinct entity in future revisions of lymphoma classifications.
Authors: Joan Guitart; M Estela Martinez-Escala; Antonio Subtil; Madeleine Duvic; Melissa P Pulitzer; Elise A Olsen; Ellen Kim; Alain H Rook; Sara S Samimi; Gary S Wood; Michael Girardi; Jacqueline Junkins-Hopkins; Doina S Ivan; M Angelica Selim; Kimberly A Sable; Pooja Virmani; Laura B Pincus; Michael T Tetzlaff; Jinah Kim; Youn H Kim Journal: Mod Pathol Date: 2017-01-27 Impact factor: 7.842
Authors: Kerasia-Maria Plachouri; Carsten Weishaupt; Dieter Metze; Georg Evers; Wolfgang E Berdel; Werner Kempf; Cord Sunderkötter; Matthias Stelljes Journal: JAAD Case Rep Date: 2017-04-14