WHO-EORTC classification for cutaneous lymphomas (original) (raw)

Reclassification of 300 Primary Cutaneous B-Cell Lymphomas According to the New WHO–EORTC Classification for Cutaneous Lymphomas: Comparison With Previous Classifications and Identification of Prognostic Markers

Journal of Clinical Oncology, 2007

Purpose In the new WHO–European Organisation for Research and Treatment of Cancer (WHO-EORTC) classification for cutaneous lymphomas three major groups of primary cutaneous B-cell lymphoma (CBCL) are distinguished: primary cutaneous marginal zone B-cell lymphoma (PCMZL) and primary cutaneous follicle center lymphoma (PCFCL) with a good prognosis, and primary cutaneous large B-cell lymphoma, leg type (PCLBCL-LT), with an intermediate-level prognosis. This study aimed to assess the clinical significance of the new classification compared with previous classification schemes (EORTC 1997; WHO 2001) and to define prognostic factors within the newly defined categories. Patients and Methods In the present study clinical data and histologic sections of 300 patients with CBCL, formerly classified according to the EORTC classification, were reviewed and reclassified according to the WHO and the new WHO-EORTC classification schemes. Results After reclassification, the study comprised 71 patien...

The clinico-pathological spectrum of primary cutaneous lymphoma other than mycosis fungoides/Sezary syndrome

Virchows Archiv, 2019

The major aim of Session 1 of the 2018 European Association of Hematopathology/Society for Hematopathology Workshop was to collect examples of cutaneous lymphomas, excluding mycosis fungoides/Sezary syndrome, as defined in the current WHO classification of tumours of the haemetopoietic and lymphoid tissues. Overall 42 cases were submitted. These were considered in four main categories: primary cutaneous B cell lymphomas (12 cases), primary cutaneous T cell lymphomas/lymphoproliferations with CD8+/cytotoxic phenotype (12 cases), primary cutaneous CD30positive lymphoproliferative disorders (15 cases) and primary cutaneous T cell lymphomas/leukaemias with CD4+ phenotype (4 cases). Using these cases as examples, we were able to present the full spectrum of cutaneous lymphoproliferations (excluding mycosis fungoides/Sezary syndrome), including examples of rare, provisional and new entities as listed in the 2017 update of the WHO classification. The findings are summarized in this report with emphasis on differential diagnostic considerations and the importance of clinico-pathological correlation for final subtyping. In presenting these findings we hope to raise awareness of this enigmatic group of neoplasms and to further our understanding of these rare disease entities.

Primary cutaneous lymphoma: two-decade comparison in a population of 263 cases from a Swiss tertiary referral centre

British Journal of Dermatology, 2011

Background Epidemiological data on primary cutaneous lymphomas (PCLs) are rare and have not previously been investigated in Switzerland. Objective To analyse variations in demographics, the pattern of subtypes and staging during the two 10-year intervals, 1990-1999 and 2000-2009. Methods This was a descriptive study of 263 patients with PCL based on a retrospective review and reassessment according to the World Health Organization ⁄European Organization for Research and Treatment of Cancer classification. Results Change was observed in the pattern of cutaneous T-cell lymphoma subtypes: the frequency of Sézary syndrome decreased from 17% to 7% and the frequency of CD30+ lymphoproliferative disorders increased from 7% to 18% (overall P = 0AE04). Staging of PCL showed a higher number of cases of earlystage mycosis fungoides (P = 0AE01). In relation to the international data, the Zürich group had a higher number of patients with Sézary syndrome (11% vs. 3%) and marginal cell lymphoma (14% vs. 5-7%). In addition, comparison of the survival data showed prolonged median overall survival of Zürich patients with Sézary syndrome in the second 10-year interval (6AE5 vs. 2-4 years). Conclusion The increasing frequency of marginal cell lymphoma and CD30+ lymphoproliferative disorders might depend on an increased awareness of these diseases in the medical community, driven by progress in the classification and staging of these disease entities.

WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects

Journal of Cutaneous Pathology, 2005

The new WHO/EORTC classification for cutaneous lymphomas comprises mature T-cell and natural killer (NK)-cell neoplasms, mature B-cell neoplasms, and immature hematopoietic malignancies. It reflects the unique features of lymphoproliferative diseases of the skin, and at the same time it is as compatible as possible with the concepts underlying the WHO classification for nodal lymphomas and the EORTC classification of cutaneous lymphomas. This article reviews the histological, phenotypical, and molecular genetic features of the various nosological entities included in this new classification. These findings always have to be interpreted in the context of the clinical features and biologic behavior. Aim: To review the histological, phenotypical and molecular genetic features of the various nosological entities of the new WHO/EORTC classification for cutaneous lymphomas. Methods: Extensive review of the literature cited in Medline and own data of the authors. Results: The WHO/EORTC classification of cutaneous lymphomas comprises mature T-cell and NK-cell neoplasms, mature B-cell neoplasms and immature hematopoietic malignancies. It reflects the unique features of primary cutaneous lymphoproliferative diseases. Conclusion: This classification is as much as possible compatible with the concept of the WHO classification for nodal lymphomas and the EORTC classification of cutaneous lymphomas. The histological, phenotypical and molecular genetic features always have to be interpreted in the context of the clinical features and biologic behavior. S. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects.

Evaluation, Diagnosis, and Staging of Cutaneous Lymphoma

The unique features of the diagnosis, evaluation, classification and staging of mycosis fungoides and Sé zary syndrome. The evaluation, classification and staging of the nonMF/nonSS CTCLs and the most common subtypes of CBCLs. The response criteria for evaluation of therapeutic efficacy for all subtypes of cutaneous lymphoma.

T-cell Primary Cutaneous Lymphomas. A Clinicopathological and Immunohistochemical Study

2007

Within the large framework of the lymphoproliferative diseases, the primary cutaneous lymphomas are distinct pathologic conditions, defi ned by particular morphologic, immunologic, genetic, and clinic criteria. The study aimed to create the fi rst clinicopathological and immunohistochemical profi le of primary cutaneous lymphoma for a Romanian region. We investigated a series of 16 cases (diagnosed during a 5-year period) in accordance with the general principles of primary cutaneous lymphoma management. The methods included the clinic and morphologic exams, the latter relying on standard and immunohistochemical staining. The results revealed that all studied cases were T-type lymphomas, in terms of the WHO-EORTC classifi cation. Most of these cases were diagnosed as mycosis fungoides; the group also included cases of Sezary syndrome, as well as rare entities such as: mycosis fungoides associated with follicular mucinosis and subcutaneous panniculitis-like T-cell lymphoma. Our discussions focused on the role of the clinicopathological assessment for the primary cutaneous lymphoma diagnosis and emphasized the importance of the immunohistochemical investigation. Compared with the previous Romanian researches on this topic, presenting only isolated cases, the current study develops a new level of analysis, based on the rigorous monitoring of a relatively large geographical area, for a long time horizon.

Epidemiological changes in cutaneous lymphomas: an analysis of 8593 patients from the French Cutaneous Lymphoma Registry*

British Journal of Dermatology, 2020

Background: Primary cutaneous lymphomas (PCLs) are a heterogenous group of T-(CTCL) and B-cell (CBCL) malignancies. Little is known about their epidemiology at initial presentation in Europe and about potential changes over time. Objectives: Aim of this retrospective study was to analyse the frequency of PCLs in the French Cutaneous Lymphoma Registry (GFELC) and to describe the demography of patients. Patients and methods: Patients with a centrally validated diagnosis of primary PCL, diagnosed between 2005 and 2019, were included. Results: Calculated incidence was unprecedently high at 1.06/100,000 person-years. The number of included patients increased yearly. Most PCL subtypes were more frequent in male patients, diagnosed at a median age of 60 years. The relative frequency of rare CTCL remained stable, the proportion of classical mycosis fungoides (MF) decreased, while its variants (e.g. folliculotropic MF) increased. Similar patterns were observed for CBCL, for example the proportion of marginalzone CBCL increased over time. Conclusions: Changes in PCL frequencies may be explained by the emergence of new diagnostic criteria and better description of the entities in the most recent PCL classification. Moreover, we propose an algorithm to confirm the diagnosis of PCL by central validation of the cases.

EORTC Cutaneous Lymphoma Task Force

European Journal of Cancer, 2002

The Cutaneous Lymphoma Task Force has represented the European Organisation for Research and Treatment of Cancer (EORTC) over the last two decades and has received worldwide acceptance and the highest respect. The group has been able to bring together the world's experts in this field to try to solve the basic problems associated with primary lymphomas of the skin and to create a productive scientific research basis. The definition and the classification of the disease per se has been a major controversial problem and the development of an EORTC classification for primary cutaneous lymphoma has been one of the main goals of the group. The purpose of this paper is to highlight and to provide a historical perspective regarding the contribution of the EORTC Cutaneous Lymphoma Group to the development of consensus guidelines for securing uniform diagnosis, classification and management of the heterogeneous group of primary cutaneous lymphomas. Some future perspectives and strategies of the group are also presented.