The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas

Abstract Primary cutaneous lymphomas are a heterogeneous group of T- and B-cell lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. The 2005 World Health Organization–European Organization for Research and Treatment of Cancer (WHO-EORTC) consensus classification has served as a golden standard for the diagnosis and classification of these conditions. In September 2018, an updated version of the WHO-EORTC was published in the fourth edition of the WHO Classification of Skin Tumours Blue Book. In this classification, primary cutaneous acral CD8+ T-cell lymphoma and Epstein-Barr virus positive (EBV+) mucocutaneous ulcer are included as new provisional entities, and a new section on cutaneous forms of chronic active EBV disease has been added. The term “primary cutaneous CD4+ small/medium T-cell lymphoma” was modified to “primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder” because of its indolent clinical behavior and uncertain malignant potential. Modifications have also been made in the sections on lymphomatoid papulosis, increasing the spectrum of histologic and genetic types, and primary cutaneous marginal zone lymphomas recognizing 2 different subtypes. Herein, the characteristic features of these new and modified entities as well as the results of recent molecular studies with diagnostic, prognostic, and/or therapeutic significance for the different types of primary cutaneous lymphomas are reviewed. An update of the frequency and survival of the different types of primary cutaneous lymphomas is provided.

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Subcutaneous Panniculitis-like T-Cell Lymphoma: Redefinition of Diagnostic Criteria in the Recent World Health Organization–European Organization for Research and Treatment of Cancer Classification for Cutaneous Lymphomas

Archives of Pathology & Laboratory Medicine ◽

10.5858/133.2.303 ◽

2009 ◽

Vol 133 (2) ◽

pp. 303-308 ◽

Cited By ~ 6

Author(s):

Zahida Parveen ◽

Karen Thompson

Keyword(s):

T Cell ◽

World Health Organization ◽

Diagnostic Criteria ◽

Cell Lymphoma ◽

Cancer Classification ◽

T Cell Lymphoma ◽

World Health ◽

European Organization ◽

Cutaneous Lymphomas ◽

Health Organization

Abstract Subcutaneous panniculitis-like T-cell lymphoma is a primary T-cell lymphoma that preferentially involves the subcutaneous tissue. Although subcutaneous panniculitis-like T-cell lymphoma has been recognized as a distinctive entity in the category of peripheral T-cell lymphoma in the World Health Organization classification, its diagnostic criteria has been redefined by the recent World Health Organization–European Organization for Research and Treatment of Cancer classification for primary cutaneous lymphomas. Subcutaneous panniculitis-like T-cell lymphoma is now restricted to primary cutaneous T-cell lymphoma expressing αβ T-cell receptor phenotype. These lymphomas are usually CD3+, CD4−, CD8+, and CD56−, and usually have an indolent clinical course. The clinicopathologic features, differential diagnosis, immunophenotypic characteristics, and molecular features of subcutaneous panniculitis-like T-cell lymphoma are presented in light of the recent World Health Organization–European Organization for Research and Treatment of Cancer classification.

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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 ◽

10.1182/blood.v99.3.800 ◽

2002 ◽

Vol 99 (3) ◽

pp. 800-805 ◽

Cited By ~ 170

Author(s):

Regina Fink-Puches ◽

Paulus Zenahlik ◽

Barbara Bäck ◽

Josef Smolle ◽

Helmut Kerl ◽

...

Keyword(s):

T Cell ◽

B Cell ◽

Who Classification ◽

Cell Lymphoma ◽

World Health ◽

European Organization ◽

Diagnostic Categories ◽

Cutaneous Lymphomas ◽

Health Organization

Abstract Classification of primary cutaneous lymphomas (PCLs) is the subject of ongoing controversy. Based on a series of 556 patients, the applicability of the European Organization for Research and Treatment of Cancer (EORTC) classification for PCLs was assessed and compared to the proposed World Health Organization (WHO) classification of hematologic malignancies. The large majority of patients could be properly classified according to the scheme proposed by the EORTC. Comparison of estimated 5-year survival for specific diagnostic categories of PCLs demonstrated nearly complete concordance of the present results with those of the EORTC study for most of the indolent cutaneous T-cell lymphomas and cutaneous B-cell lymphomas, whereas differences were found for mycosis fungoides-associated follicular mucinosis and Sezary syndrome. A few patients with newly described entities (CD8+ epidermotropic cytotoxic T-cell lymphoma, primary cutaneous natural killer/T-cell lymphoma) could not be classified according to the EORTC scheme. Comparison of the EORTC with the WHO classification showed that the EORTC scheme allows a more precise categorization of the patients, especially for cutaneous B-cell lymphoma. In conclusion, the study confirmed that the EORTC classification allows a better management of patients with PCL. Small amendments to that classification should be carried out to account for recently described entities and to unify some of the diagnostic categories.

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Update on Gastrointestinal Lymphomas

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2018-0275-ra ◽

2018 ◽

Vol 142 (11) ◽

pp. 1347-1351 ◽

Cited By ~ 7

Author(s):

Steven C. Weindorf ◽

Lauren B. Smith ◽

Scott R. Owens

Keyword(s):

T Cell ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

World Health ◽

Barr Virus ◽

Not Otherwise Specified ◽

Gastrointestinal Lymphomas ◽

Epstein Barr ◽

Health Organization

Herein we review the following selection of gastrointestinal lymphomas: monomorphic epitheliotropic intestinal T-cell lymphoma; indolent T-cell lymphoproliferative disorder of the gastrointestinal tract; intestinal T-cell lymphoma, not otherwise specified; duodenal-type follicular lymphoma; and Epstein-Barr virus–positive mucocutaneous ulcer. Definitions reflect the 2016 revision of the World Health Organization classification of lymphoid neoplasms. Clinical, morphologic, and immunophenotypic characteristics of each entity are emphasized.

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Primary Cutaneous Acral CD8+ T-Cell Lymphoma

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2017-0230-ra ◽

2017 ◽

Vol 141 (11) ◽

pp. 1469-1475 ◽

Cited By ~ 8

Author(s):

Vivian M. Hathuc ◽

Alexandra C. Hristov ◽

Lauren B. Smith

Keyword(s):

T Cell ◽

Cell Lymphoma ◽

Correct Diagnosis ◽

Cd8 T Cell ◽

T Cell Lymphoma ◽

World Health ◽

T Cell Lymphomas ◽

Pathologic Features ◽

Health Organization

Primary cutaneous acral CD8+ T-cell lymphoma is a new provisional entity in the 2016 revision of the World Health Organization classification of lymphoid neoplasms. This is a challenging diagnosis because of its rarity, as well as its morphologic and immunophenotypic overlap with other CD8+ cytotoxic lymphoid proliferations. Appropriate classification of this entity is crucial because of its indolent clinical behavior compared with other CD8+ T-cell lymphomas. Knowledge of the clinical setting, sites of involvement, and morphologic features can aid in correct diagnosis. Here, we review the clinical and pathologic features of primary cutaneous acral CD8+ T-cell lymphoma with an emphasis on the differential diagnosis among other C8+ T-cell lymphomas.

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Recent Advances in Digestive Tract Tumors: Updates From the 5th Edition of the World Health Organization “Blue Book”

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2020-0047-ra ◽

2020 ◽

Author(s):

Raul S. Gonzalez ◽

Anwar Raza ◽

Robert Propst ◽

Oyedele Adeyi ◽

Justin Bateman ◽

...

Keyword(s):

T Cell ◽

World Health Organization ◽

Digestive System ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

World Health ◽

World Health Organization Classification ◽

The World ◽

Health Organization

Context.— The World Health Organization Classification of Tumors: Digestive System Tumors, 5th edition, was published in 2019 and shows several impactful changes as compared with the 4th edition published in 2010. Changes include a revised nomenclature of serrated lesions and revamping the classification of neuroendocrine neoplasms. Appendiceal goblet cell adenocarcinoma is heavily revised, and intrahepatic cholangiocarcinoma is split into 2 subtypes. New subtypes of colorectal carcinoma and hepatocellular carcinoma are described. Precursor lesions are emphasized with their own entries, and both dysplastic and invasive lesions are generally recommended to be graded using a 2-tier system. Hematolymphoid tumors, mesenchymal tumors, and genetic tumor syndromes each have their own sections in the 5th edition. New hematolymphoid lesions include monomorphic epitheliotropic intestinal T-cell lymphoma; duodenal-type follicular lymphoma; intestinal T-cell lymphoma, not otherwise specified; and indolent T-cell lymphoproliferative disorder of the gastrointestinal tract. This paper will provide an in-depth look at the changes in the 5th edition as compared with the 4th edition. Objective.— To provide a comprehensive, in-depth update on the World Health Organization classification of digestive tumors, including changes to nomenclature, updated diagnostic criteria, and newly described entities. Data Sources.— The 5th edition of the World Health Organization Classification of Tumours: Digestive System Tumours, as well as the 4th edition. Conclusions.— The World Health Organization has made many key changes in its newest update on tumors of the digestive system. Pathologists should be aware of these changes and incorporate them into their practice as able or necessary.

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Potentials of brentuximab vedotin in the treatment of relapse/refractory cutaneous T-cell lymphomas: literature review and authors’ observation

Vestnik dermatologii i venerologii ◽

10.25208/0042-4609-2019-95-2-42-49 ◽

2019 ◽

Vol 95 (2) ◽

pp. 42-49

Author(s):

L. G. Gorenkova ◽

S. K. Kravchenko ◽

I. E. Belousova

Keyword(s):

T Cell ◽

Cell Lymphoma ◽

Brentuximab Vedotin ◽

T Cell Lymphoma ◽

Lymphoproliferative Diseases ◽

Peripheral T Cell Lymphoma ◽

Lymphomatoid Papulosis ◽

Not Otherwise Specified ◽

T Cell Lymphomas ◽

Cutaneous Lymphomas

Primary cutaneous T-cell lymphomas encompass a heterogeneous group of T-cell lymphoproliferative disorders developing primarily in the skin and characterized by a number of specific diagnostic, clinical, and therapeutic features. Mycosis fungoides accounts for more than half of all cutaneous lymphoma cases, while CD30+ lymphoproliferative diseases of the skin (primary cutaneous anaplastic large-cell lymphoma and lymphomatoid papulosis) constitute one-fourth of them and the remaining cases are rare tumour types, including primary cutaneous peripheral T-cell lymphoma, unspecified/not otherwise specified.Activation antigen СD30 is a cell membrane glycoprotein of the tumour necrosis factor family. More than 75 % of primary cutaneous CD30-positive lymphoma cells express CD30; it may be detected in other diseases as well, but to a lesser extent.Most patients with cutaneous CD30+ lymphoproliferative diseases have indolent disease and a favourable prognosis; resistant disease is observed in approximately 30 % of sufferers, and fatal outcomes occur in 8 % of cases [1].Systemic immunomodulatory therapy or chemotherapy is often used in advanced disease. Monoclonal antibodies were recently introduced into clinical practice for the treatment of cutaneous lymphomas. One of these agents is brentuximab vedotin, a CD30-monoclonal antibody conjugated to monomethyl auristatin E.We present two case reports: one of frequently recurring lymphomatoid papulosis and the other of refractory primary cutaneous peripheral T-cell lymphoma, unspecified/not otherwise specified. Targeted therapy with brentuximab vedotin, either alone or in combination with chemotherapy, resulted in a sustained, long-lasting remission in both cases.

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Primary Cutaneous Lymphomas in Thailand: A 10-Year Retrospective Study

BioMed Research International ◽

10.1155/2021/4057661 ◽

2021 ◽

Vol 2021 ◽

pp. 1-8

Author(s):

Chutipon Pruksaeakanan ◽

Phurichaya Teyateeti ◽

Poramin Patthamalai ◽

Janista Thumrongtharadol ◽

Manasmon Chairatchaneeboon

Keyword(s):

T Cell ◽

B Cell ◽

Cell Lymphoma ◽

Early Stage ◽

Tertiary Care ◽

B Cell Lymphoma ◽

T Cell Lymphoma ◽

Early Stage Disease ◽

Lymphomatoid Papulosis ◽

Cutaneous Lymphomas

Background. Primary cutaneous lymphomas (PCLs) refer to cutaneous lymphomas that primarily develop in the skin with no evidence of extracutaneous disease at the time of diagnosis. The epidemiological and clinical data of PCLs in Thailand are lacking. Objectives. To evaluate the frequency, demographic data, and clinical characteristics of different subtypes of PCLs in a tertiary care university hospital. Methods. In total, 137 patients with PCLs diagnosed in our hospital in 2008–2017 were retrospectively reviewed. Results. Of the 137 patients, 57 (41.6%) were male and 80 (58.4%) were female ( M : F = 1 : 1.4 ). The median age at diagnosis was 40 years. Most patients (134, 97.8%) had cutaneous T-cell lymphomas (CTCLs). Three patients (2.2%) had cutaneous B-cell lymphomas (CBCLs). The most common subtype was mycosis fungoides (MF) (67.9%), followed by subcutaneous panniculitis-like T-cell lymphoma (SPTCL) (21.2%), primary cutaneous anaplastic large cell lymphoma (pcALCL) (3.6%), lymphomatoid papulosis (LyP) (1.5%), primary cutaneous gamma/delta T-cell lymphoma (pcGDTCL) (1.5%), Sézary syndrome (SS) (0.7%), extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) (0.7%), primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS) (0.7%), primary cutaneous diffuse large B-cell lymphoma, leg type (pcDLBCL-LT) (1.5%), and primary cutaneous follicle center lymphoma (pcFCL) (0.7%). Most patients with MF presented with early-stage disease (84.0%), with hypopigmented MF the most common variant (42.6%). Conclusions. Compared to earlier Caucasian and Asian studies, the present study revealed a higher proportion of CTCL patients with a younger age at onset and a female predominance. MF was the most common CTCL subtype, followed by SPTCL. More than 80% of MF patients were diagnosed at an early stage.

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