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EORTC Classification for Primary Cutaneous Lymphomas: A Proposal From the Cutaneous Lymphoma Study Group of the European Organization for Research and Treatment of Cancer

R. Willemze, H. Kerl, W. Sterry, E. Berti, L. Cerroni, S. Chimenti, J.L. Diaz-Peréz, M.L. Geerts, M. Goos, R. Knobler, E. Ralfkiaer, M. Santucci, N. Smith, J. Wechsler, W.A. van Vloten, and C.J.L.M. Meijer

From the Departments of Dermatology and Pathology, Free University Hospital, Amsterdam, The Netherlands; the Department of Dermatology, University of Graz, Austria; the Department of Dermatology, Charité, Humboldt University, Berlin, Germany; the Department of Dermatology, University of Milan, Italy; the Department of Dermatology, University of L'Aquila, Italy; the Department of Dermatology, Cruces Hospital, Bilbao, Spain; the Department of Dermatology, University of Gent, Belgium; the Department of Dermatology, University of Essen, Germany; the Department of Dermatology, University of Vienna, Austria; the Department of Pathology, Herlev Hospital, University of Copenhagen, Denmark; the Department of Pathology, University of Florence, Italy; the Department of Dermatology, St John's Hospital, London, UK; the Department of Pathology, Hospital Henri Mondor, Paris, France; the Department of Dermatology, University of Utrecht, The Netherlands.

Primary cutaneous lymphomas represent a heterogeneous group of T- and B-cell lymphomas that show considerable variation in histology, phenotype, and prognosis. Recently, the European Organization for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Project Group has reached consensus on a new classification for this group of diseases. The EORTC classification for primary cutaneous lymphomas is based on a combination of clinical, histologic, and immunophenotypic criteria, and thus contains well-defined disease entities rather than histologic subgroups. In addition, this new classification contains a number of provisional entities, which may display characteristic histologic features, but are not yet well defined clinically. These provisional entities account for less than 5% of all primary cutaneous lymphomas. In this report the basic principles of this new classification, as well as the characteristic features of the different disease entities, are described. In addition, survival data of 626 patients with primary cutaneous lymphomas derived from the registry of the Dutch Cutaneous Lymphoma Working Group, illustrating the clinical validity of this new classification, are presented.

Blood, Vol. 90 No. 1 (July 1), 1997: pp. 354-371
© 1997 by The American Society of Hematology.


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Arch DermatolHome page
L. Cerroni, R. Fink-Puches, B. Back, and H. Kerl
Follicular Mucinosis: A Critical Reappraisal of Clinicopathologic Features and Association With Mycosis Fungoides and Sezary Syndrome
Arch Dermatol, February 1, 2002; 138(2): 182 - 189.
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Arch DermatolHome page
R. van Doorn, E. Scheffer, R. Willemze, and for the Dutch Cutaneous Lymphoma Group
Follicular Mycosis Fungoides, a Distinct Disease Entity With or Without Associated Follicular Mucinosis: A Clinicopathologic and Follow-up Study of 51 Patients
Arch Dermatol, February 1, 2002; 138(2): 191 - 198.
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Arch DermatolHome page
R. Dummer, J. Kamarashev, W. Kempf, A. C. Haffner, M. Hess-Schmid, and G. Burg
Junctional CD8+ Cutaneous Lymphomas With Nonaggressive Clinical Behavior: A CD8+ Variant of Mycosis Fungoides?
Arch Dermatol, February 1, 2002; 138(2): 199 - 203.
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BloodHome page
R. Fink-Puches, P. Zenahlik, B. Back, J. Smolle, H. Kerl, and L. Cerroni
Primary cutaneous lymphomas: applicability of current classification schemes (European Organization for Research and Treatment of Cancer, World Health Organization) based on clinicopathologic features observed in a large group of patients
Blood, February 1, 2002; 99(3): 800 - 805.
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BloodHome page
M. Urosevic, J. Willers, B. Mueller, W. Kempf, G. Burg, and R. Dummer
HLA-G protein up-regulation in primary cutaneous lymphomas is associated with interleukin-10 expression in large cell T-cell lymphomas and indolent B-cell lymphomas
Blood, January 15, 2002; 99(2): 609 - 617.
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ASH Education BookHome page
J. M. Connors, E. D. Hsi, and F. M. Foss
Lymphoma of the Skin
Hematology, January 1, 2002; 2002(1): 263 - 282.
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JCOHome page
M. H. Vermeer, R. van Doorn, D. Dukers, M. W. Bekkenk, C. J.L.M. Meijer, and R. Willemze
CD8+ T Cells in Cutaneous T-Cell Lymphoma: Expression of Cytotoxic Proteins, Fas Ligand, and Killing Inhibitory Receptors and Their Relationship With Clinical Behavior
J. Clin. Oncol., December 1, 2001; 19(23): 4322 - 4329.
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Arch DermatolHome page
N. V. Kamath, A. C. Gilliam, M. Nihal, T. P. Spiro, and G. S. Wood
Primary Cutaneous Large B-Cell Lymphoma of the Leg Relapsing as Cutaneous Intravascular Large B-Cell Lymphoma
Arch Dermatol, December 1, 2001; 137(12): 1657 - 1658.
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NEJMHome page
K. D. Berg, N. K. Brinster, K. M. Huhn, M. G. Goggins, R. J. Jones, A. Makary, K. M. Murphy, C. A. Griffin, L. S. Rosenblum-Vos, M. J. Borowitz, et al.
Transmission of a T-Cell Lymphoma by Allogeneic Bone Marrow Transplantation
N. Engl. J. Med., November 15, 2001; 345(20): 1458 - 1463.
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Arch DermatolHome page
G. S. Wood
T-Cell Receptor and Immunoglobulin Gene Rearrangements in Diagnosing Skin Disease
Arch Dermatol, November 1, 2001; 137(11): 1503 - 1506.
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BloodHome page
R. L. Piekarz, R. Robey, V. Sandor, S. Bakke, W. H. Wilson, L. Dahmoush, D. M. Kingma, M. L. Turner, R. Altemus, and S. E. Bates
Inhibitor of histone deacetylation, depsipeptide (FR901228), in the treatment of peripheral and cutaneous T-cell lymphoma: a case report
Blood, November 1, 2001; 98(9): 2865 - 2868.
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JCOHome page
F. Grange, M. W. Bekkenk, J. Wechsler, C. J.L.M. Meijer, L. Cerroni, M. Bernengo, J. Bosq, G. Hedelin, R. Fink Puches, W. A. van Vloten, et al.
Prognostic Factors in Primary Cutaneous Large B-Cell Lymphomas: A European Multicenter Study
J. Clin. Oncol., August 15, 2001; 19(16): 3602 - 3610.
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Arch DermatolHome page
M. H. Vermeer, C. P. Tensen, P. M. van der Stoop, H. W. van Oostveen, M. Lund, R. J. Scheper, and R. Willemze
Absence of TH2 Cytokine Messenger RNA Expression in CD30-Negative Primary Cutaneous Large T-Cell Lymphomas
Arch Dermatol, July 1, 2001; 137(7): 901 - 905.
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Arch DermatolHome page
P. Rubegni, G. De Aloe, M. Di Renzo, G. Pompella, A. L. Pasqui, A. Auteri, L. Andreassi, and M. Fimiani
Cytokine Production Profile of Peripheral Blood Mononuclear Cells in Patients With Large-Plaque Parapsoriasis
Arch Dermatol, July 1, 2001; 137(7): 966 - 967.
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Am. J. Pathol.Home page
J. Kamarashev, G. Burg, M. C. Mingari, W. Kempf, G. Hofbauer, and R. Dummer
Differential Expression of Cytotoxic Molecules and Killer Cell Inhibitory Receptors in CD8+ and CD56+ Cutaneous Lymphomas
Am. J. Pathol., May 1, 2001; 158(5): 1593 - 1598.
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Arch DermatolHome page
R. Bergman, P. J. Kurtin, L. E. Gibson, P. R. Hull, T. K. Kimlinger, and A. L. Schroeter
Clinicopathologic, Immunophenotypic, and Molecular Characterization of Primary Cutaneous Follicular B-Cell Lymphoma
Arch Dermatol, April 1, 2001; 137(4): 432 - 439.
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Arch DermatolHome page
L. Cerroni and H. Kerl
Primary Cutaneous Follicular Lymphoma
Arch Dermatol, April 1, 2001; 137(4): 489 - 490.
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J. Clin. Pathol.Home page
D F Dukers, M H Vermeer, L H Jaspars, C A Sander, M J Flaig, W Vos, R Willemze, and C J L M Meijer
Expression of killer cell inhibitory receptors is restricted to true NK cell lymphomas and a subset of intestinal enteropathy-type T cell lymphomas with a cytotoxic phenotype
J. Clin. Pathol., March 1, 2001; 54(3): 224 - 228.
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BloodHome page
M. Bagot, A. Moretta, S. Sivori, R. Biassoni, C. Cantoni, C. Bottino, L. Boumsell, and A. Bensussan
CD4+ cutaneous T-cell lymphoma cells express the p140-killer cell immunoglobulin-like receptor
Blood, March 1, 2001; 97(5): 1388 - 1391.
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Nucleic Acids ResHome page
T. E. Hansen-Hagge, U. Trefzer, A. S. zu Reventlow, K. Kaltoft, and W. Sterry
Identification of sample-specific sequences in mammalian cDNA and genomic DNA by the novel ligation-mediated subtraction (Limes)
Nucleic Acids Res., February 15, 2001; 29(4): e20 - e20.
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BloodHome page
J. J. Scarisbrick, S. Whittaker, A. V. Evans, E. A. Fraser-Andrews, F. J. Child, A. Dean, and R. Russell-Jones
Prognostic significance of tumor burden in the blood of patients with erythrodermic primary cutaneous T-cell lymphoma
Blood, February 1, 2001; 97(3): 624 - 630.
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JCOHome page
A. H. Sarris, I. Braunschweig, L. J. Medeiros, M. Duvic, C. S. Ha, M. A. Rodriguez, F. B. Hagemeister, P. McLaughlin, J. Romaguera, J. Cox, et al.
Primary Cutaneous Non-Hodgkin's Lymphoma of Ann Arbor Stage I: Preferential Cutaneous Relapses but High Cure Rate With Doxorubicin-Based Therapy
J. Clin. Oncol., January 15, 2001; 19(2): 398 - 405.
[Abstract] [Full Text] [PDF]


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JRSMHome page
D. Slater
Histopathological aspects of cutaneous lymphoma
J R Soc Med, January 7, 2001; 94(7): 337 - 340.
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Cancer Res.Home page
M. Storz, K. Zepter, J. Kamarashev, R. Dummer, G. Burg, and A. C. Häffner
Coexpression of CD40 and CD40 Ligand in Cutaneous T-Cell Lymphoma (Mycosis Fungoides)
Cancer Res., January 1, 2001; 61(2): 452 - 454.
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ASH Education BookHome page
J. P. Greer, M. C. Kinney, and T. P. Loughran Jr.
T Cell and NK Cell Lymphoproliferative Disorders
Hematology, January 1, 2001; 2001(1): 259 - 281.
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BloodHome page
H. Stein, H.-D. Foss, H. Durkop, T. Marafioti, G. Delsol, K. Pulford, S. Pileri, and B. Falini
CD30+ anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features
Blood, December 1, 2000; 96(12): 3681 - 3695.
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BloodHome page
M.-H. Delfau-Larue, L. Laroche, J. Wechsler, E. Lepage, C. Lahet, M. Asso-Bonnet, M. Bagot, and J.-P. Farcet
Diagnostic value of dominant T-cell clones in peripheral blood in 363 patients presenting consecutively with a clinical suspicion of cutaneous lymphoma
Blood, November 1, 2000; 96(9): 2987 - 2992.
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JCOHome page
R. S. Siegel, T. Pandolfino, J. Guitart, S. Rosen, and T. M. Kuzel
Primary Cutaneous T-Cell Lymphoma: Review and Current Concepts
J. Clin. Oncol., August 15, 2000; 18(15): 2908 - 2925.
[Abstract] [Full Text] [PDF]


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Arch DermatolHome page
J. R. Toro, M. Beaty, L. Sorbara, M. L. Turner, J. White, D. W. Kingma, M. Raffeld, and E. S. Jaffe
{gamma}{delta} T-Cell Lymphoma of the Skin: A Clinical, Microscopic, and Molecular Study
Arch Dermatol, August 1, 2000; 136(8): 1024 - 1032.
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Arch DermatolHome page
M. E. Kadin
Cutaneous {gamma}{delta} T-Cell Lymphomas--How and Why Should They Be Recognized?
Arch Dermatol, August 1, 2000; 136(8): 1052 - 1054.
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BloodHome page
E. Poszepczynska, M. Bagot, H. Echchakir, D. Martinvalet, M. Ramez, D. Charue, L. Boumsell, and A. Bensussan
Functional characterization of an IL-7-dependent CD4+CD8alpha alpha + Th3-type malignant cell line derived from a patient with a cutaneous T-cell lymphoma
Blood, August 1, 2000; 96(3): 1056 - 1063.
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BloodHome page
M. W. Bekkenk, F. A. M. J. Geelen, P. C. v. V. Vader, F. Heule, M.-L. Geerts, W. A. van Vloten, C. J. L. M. Meijer, and R. Willemze
Primary and secondary cutaneous CD30+ lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment
Blood, June 15, 2000; 95(12): 3653 - 3661.
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BloodHome page
L. Cerroni, E. Arzberger, B. Putz, G. Hofler, D. Metze, C. A. Sander, C. Rose, P. Wolf, A. Rutten, J. M. McNiff, et al.
Primary cutaneous follicle center cell lymphoma with follicular growth pattern
Blood, June 15, 2000; 95(12): 3922 - 3928.
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J. Clin. Pathol.Home page
R. L t. Berge, J. J Oudejans, G.-J. Ossenkoppele, K. Pulford, R. Willemze, B. Falini, A. Chott, and C. J L M Meijer
ALK expression in extranodal anaplastic large cell lymphoma favours systemic disease with (primary) nodal involvement and a good prognosis and occurs before dissemination
J. Clin. Pathol., June 1, 2000; 53(6): 445 - 450.
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JCOHome page
T. L. Pandolfino, R. S. Siegel, T. M. Kuzel, S. T. Rosen, and J. Guitart
Primary Cutaneous B-Cell Lymphoma: Review and Current Concepts
J. Clin. Oncol., May 10, 2000; 18(10): 2152 - 2168.
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Arch DermatolHome page
R. van Doorn, C. W. Van Haselen, P. C. van Voorst Vader, M.-L. Geerts, F. Heule, M. de Rie, P. M. Steijlen, S. K. Dekker, W. A. van Vloten, and R. Willemze
Mycosis Fungoides: Disease Evolution and Prognosis of 309 Dutch Patients
Arch Dermatol, April 1, 2000; 136(4): 504 - 510.
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Arch DermatolHome page
M. Ming and P. E. LeBoit
Can Dermatopathologists Reliably Make the Diagnosis of Mycosis Fungoides?: If Not, Who Can?
Arch Dermatol, April 1, 2000; 136(4): 543 - 546.
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NEJMHome page
J. Guitart, F. Roufosse, L. Schandene, E. Cogan, R. Suzuki, M. Seto, S. Nakaura, H.-U. Simon, and R. Dummer
Idiopathic Eosinophilia
N. Engl. J. Med., March 2, 2000; 342(9): 659 - 661.
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Arch DermatolHome page
L. Heinzerling, R. Dummer, W. Kempf, M. H. Schmid, and G. Burg
Intralesional Therapy With Anti-CD20 Monoclonal Antibody Rituximab in Primary Cutaneous B-Cell Lymphoma
Arch Dermatol, March 1, 2000; 136(3): 374 - 378.
[Abstract] [Full Text] [PDF]



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