Aggressive epidermotropic cutaneous CD8+ lymphoma: a cutaneous lymphoma with distinct clinical and pathological features. Report of an EORTC Cutaneous Lymphoma Task Force Workshop
Corresponding Author
Alistair Robson
St John's Institute of Dermatology, London, UK
Address for correspondence: Dr A Robson, Department of Dermatopathology, 2nd Floor, Block C, South Wing, St John's Institute of Dermatology, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH London, UK. e-mail: [email protected]Search for more papers by this authorChalid Assaf
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorMartine Bagot
Department of Pathology, Universite Paris, Paris, France
Search for more papers by this authorGunter Burg
Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland
Search for more papers by this authorLorenzo Cerroni
Department of Dermatology Medical, University of Graz, Graz, Austria
Search for more papers by this authorNicola Chimenti
Department of Dermatology, University of L'Aquila, Rome, Italy
Search for more papers by this authorPierre Dechelotte
Department of Pathology, University of Clermont-Ferrand, Clermont-Ferrand, France
Search for more papers by this authorFrederic Franck
Department of Pathology, University of Clermont-Ferrand, Clermont-Ferrand, France
Search for more papers by this authorMaria Geerts
Department of Dermatology, Ghent University Hospital, Gent, Belgium
Search for more papers by this authorSylke Gellrich
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorJohn Goodlad
Department of Pathology, Western General Hospital, Edinburgh, UK
Search for more papers by this authorWerner Kempf
Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland
Search for more papers by this authorRobert Knobler
Department of Dermatology, University of Vienna, Vienna, Austria
Search for more papers by this authorCesare Massone
Department of Dermatology Medical, University of Graz, Graz, Austria
Search for more papers by this authorChris Meijer
Department of Pathology, VU University Medical Center, Amsterdam, the Netherlands
Search for more papers by this authorPablo Ortiz
Hospital Universitario, Universidad Complutense, Madrid, Spain
Search for more papers by this authorTony Petrella
Departmentof Pathology, Dijon's University Hospital, Dijon, France
Search for more papers by this authorNicola Pimpinelli
Division of Dermatology, University of Florence Medical School, Florence, Italy
Search for more papers by this authorJoclim Roewert
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorRobin Russell-Jones
St John's Institute of Dermatology, London, UK
Search for more papers by this authorMarco Santucci
Division of Pathological Anatomy, University of Florence, Florence, Italy
Search for more papers by this authorMattias Steinhoff
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorWolfram Sterry
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorJanine Wechsler
Department of Pathology Henri-Mondor Hospital, University Paris-Val-de-Marne, Paris, France
Search for more papers by this authorRein Willemze
Department of Dermatology, Leiden University, Leiden, the Netherlands
Search for more papers by this authorEmilio Berti
Department of Dermatology, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico and Università degli Studi di Milano-Bicocca, Milan, Italy
Search for more papers by this authorCorresponding Author
Alistair Robson
St John's Institute of Dermatology, London, UK
Address for correspondence: Dr A Robson, Department of Dermatopathology, 2nd Floor, Block C, South Wing, St John's Institute of Dermatology, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH London, UK. e-mail: [email protected]Search for more papers by this authorChalid Assaf
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorMartine Bagot
Department of Pathology, Universite Paris, Paris, France
Search for more papers by this authorGunter Burg
Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland
Search for more papers by this authorLorenzo Cerroni
Department of Dermatology Medical, University of Graz, Graz, Austria
Search for more papers by this authorNicola Chimenti
Department of Dermatology, University of L'Aquila, Rome, Italy
Search for more papers by this authorPierre Dechelotte
Department of Pathology, University of Clermont-Ferrand, Clermont-Ferrand, France
Search for more papers by this authorFrederic Franck
Department of Pathology, University of Clermont-Ferrand, Clermont-Ferrand, France
Search for more papers by this authorMaria Geerts
Department of Dermatology, Ghent University Hospital, Gent, Belgium
Search for more papers by this authorSylke Gellrich
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorJohn Goodlad
Department of Pathology, Western General Hospital, Edinburgh, UK
Search for more papers by this authorWerner Kempf
Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland
Search for more papers by this authorRobert Knobler
Department of Dermatology, University of Vienna, Vienna, Austria
Search for more papers by this authorCesare Massone
Department of Dermatology Medical, University of Graz, Graz, Austria
Search for more papers by this authorChris Meijer
Department of Pathology, VU University Medical Center, Amsterdam, the Netherlands
Search for more papers by this authorPablo Ortiz
Hospital Universitario, Universidad Complutense, Madrid, Spain
Search for more papers by this authorTony Petrella
Departmentof Pathology, Dijon's University Hospital, Dijon, France
Search for more papers by this authorNicola Pimpinelli
Division of Dermatology, University of Florence Medical School, Florence, Italy
Search for more papers by this authorJoclim Roewert
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorRobin Russell-Jones
St John's Institute of Dermatology, London, UK
Search for more papers by this authorMarco Santucci
Division of Pathological Anatomy, University of Florence, Florence, Italy
Search for more papers by this authorMattias Steinhoff
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorWolfram Sterry
Department of Dermatology, Charité-University Medicine, Berlin, Germany
Search for more papers by this authorJanine Wechsler
Department of Pathology Henri-Mondor Hospital, University Paris-Val-de-Marne, Paris, France
Search for more papers by this authorRein Willemze
Department of Dermatology, Leiden University, Leiden, the Netherlands
Search for more papers by this authorEmilio Berti
Department of Dermatology, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico and Università degli Studi di Milano-Bicocca, Milan, Italy
Search for more papers by this authorAbstract
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.
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