Cytotoxic/natural killer cell Cutaneous lymphomas: Report of the EORTC Cutaneous Lymphoma Task force workshop

• Published in: Cancer 2003-02, Vol.97 (3), p.610-627
• Main Authors: SANTUCCI, Marco, PIMPINELLI, Nicola, GRAZIA BERNENGO, Maria, CERRONI, Lorenzo, CHIMENTI, Sergio, CHOTT, Andreas, DIAZ-PEREZ, José L, DIPPEL, Edgard, DUNCAN, Lyn M, FELLER, Alfred C, GEERTS, Marie-Louise, HALLERMANN, Christian, MASSI, Daniela, KEMPF, Werner, RUSSELL-JONES, Robin, SANDER, Christian, BERTI, Emilio, KADIN, Marshall E, MEIJER, Chris J. L. M, MÜLLER-HERMELINK, Hans Konrad, PAULLI, Marco, WECHSLER, Janine, WILLEMZE, Rein, AUDRING, Heike
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; CD4-Positive T-Lymphocytes - immunology; CD56 Antigen - immunology; CD8-Positive T-Lymphocytes - immunology; Hematologic and hematopoietic diseases; Humans; Immunophenotyping; Killer Cells, Natural - immunology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma - classification; Lymphoma - immunology; Lymphoma - pathology; Lymphoma, T-Cell, Cutaneous - classification; Lymphoma, T-Cell, Cutaneous - immunology; Lymphoma, T-Cell, Cutaneous - pathology; Lymphoma, T-Cell, Peripheral - classification; Lymphoma, T-Cell, Peripheral - immunology; Lymphoma, T-Cell, Peripheral - pathology; Medical sciences; Membrane Proteins - immunology; Mycosis Fungoides - immunology; Mycosis Fungoides - pathology; Panniculitis - immunology; Panniculitis - pathology; Phenotype; Poly(A)-Binding Proteins; Proteins; RNA-Binding Proteins - immunology; Skin Neoplasms - classification; Skin Neoplasms - immunology; Skin Neoplasms - pathology; T-Cell Intracellular Antigen-1
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/cncr.11107

Cutaneous lymphomas expressing a cytotoxic or natural killer (NK) cell phenotype represent a group of lymphoproliferative disorders for which there is currently much confusion and little consensus regarding the best nomenclature and classification. This study analyzes 48 cases of primary cutaneous lymphoma expressing cytotoxic proteins and/or the NK cell marker, CD56. These cases were collected for a workshop of the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Task Force, to better clarify the clinical, morphologic, and phenotypic features of these uncommon tumors. Several categories with different clinical and pathologic features were delineated: 1) aggressive, CD8+, epidermotropic, cytotoxic T-cell lymphoma; 2) mycosis fungoides, cytotoxic immunophenotype variant; 3) subcutaneous panniculitis-like T-cell lymphoma; 4) NK/T-cell lymphoma, nasal type; 5) CD4+, NK cell lymphoma; 6) blastoid NK cell lymphoma; (7) intravascular NK-like lymphoma; and 8) cytotoxic, peripheral T-cell lymphoma. Our data show that primary cutaneous cytotoxic/NK cell lymphomas include distinct groups of diseases, clinically, histologically, and biologically. Because the finding of a cytotoxic phenotype often has prognostic significance, the routine use of cytotoxic markers in the diagnosis and classification of cutaneous lymphomas should be expanded.