Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop.
BACKGROUND
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Santucci
Marco
M
Department of Human Pathology and Oncology, University of Florence Medical School, Florence, Italy. Marco.Santucci@UNIFI.IT
Pimpinelli
Nicola
N
Massi
Daniela
D
Kadin
Marshall E
ME
Meijer
Chris J L M
CJ
Müller-Hermelink
Hans Konrad
HK
Paulli
Marco
M
Wechsler
Janine
J
Willemze
Rein
R
Audring
Heike
H
Bernengo
Maria Grazia
MG
Cerroni
Lorenzo
L
Chimenti
Sergio
S
Chott
Andreas
A
Díaz-Pérez
José L
JL
Dippel
Edgard
E
Duncan
Lyn M
LM
Feller
Alfred C
AC
Geerts
Marie-Louise
ML
Hallermann
Christian
C
Kempf
Werner
W
Russell-Jones
Robin
R
Sander
Christian
C
Berti
Emilio
E
D015496:CD4-Positive T-Lymphocytes / Q000276:immunology; D019002:CD56 Antigen / Q000276:immunology*; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology; D008223:Lymphoma / Q000145:classification* / Q000276:immunology / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000145:classification / Q000276:immunology / Q000473:pathology; D016411:Lymphoma, T-Cell, Peripheral / Q000145:classification / Q000276:immunology / Q000473:pathology; D008565:Membrane Proteins / Q000276:immunology*; D009182:Mycosis Fungoides / Q000276:immunology / Q000473:pathology; D015434:Panniculitis / Q000276:immunology / Q000473:pathology; D010641:Phenotype; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D016601:RNA-Binding Proteins / Q000276:immunology*; D012878:Skin Neoplasms / Q000145:classification* / Q000276:immunology / Q000473:pathology; D000074019:T-Cell Intracellular Antigen-1
D019002:CD56 Antigen; D008565:Membrane Proteins; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D016601:RNA-Binding Proteins; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human