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      Primary cutaneous B-cell lymphoma leg type: good response with the RADHAP 21 protocol ⋆

      case-report

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          Abstract

          Primary cutaneous lymphomas are defined as the ones that exclusively affect the skin for up to 6 months after the diagnosis. B-cell lymphomas represent 20-25% of primary cutaneous lymphomas and have, among its subtypes, the leg type, which represents 10 to 20% of cutaneous B-cell lymphomas, generally affecting elderly people and with an intermediate prognosis. This is the report of a rare case of a leg-type B-cell lymphoma with an exuberant clinical presentation affecting a young male patient.

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

          Rein Willemze, Lorenzo Cerroni, Werner Kempf … (2019)
          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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            Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases.

            Béatrice Vergier, P Courville, Caroline Robert … (2007)
            To describe clinicopathologic features and to identify prognostic factors in a large series of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT), as defined in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. Retrospective multicenter study from the French Study Group on Cutaneous Lymphomas. Nineteen departments of dermatology in 10 regions of France. Sixty patients with a PCLBCL LT included in the registry of the French Study Group on Cutaneous Lymphomas. Age, sex, outcome, therapy, B symptoms, cutaneous extent, number of lesions, location (leg vs nonleg), serum lactate dehydrogenase level, and MUM-1 and Bcl-2 expression were recorded. Disease-specific survival was used as the main end point. Prognostic factors were identified using a Cox proportional hazards model. Primary cutaneous diffuse large B-cell lymphoma, leg type is characterized by a predilection for the leg (72%), a high proportion of Bcl-2 expression (85%), an advanced age at onset (mean age, 76 years), and frequent relapses and extracutaneous dissemination. The overall 5-year disease-specific survival rate was 41%. Location on the leg and multiple skin lesions were predictive of death in multivariate analysis. Although no variable related to therapy was significantly associated with survival, patients recently treated with combinations of anthracycline-containing chemotherapies and rituximab had a more favorable short-term outcome. Primary cutaneous diffuse large B-cell lymphoma, leg type is a distinct entity with a poor prognosis, particularly in patients with multiple tumors on the legs. Despite the advanced age of many patients, the prognosis could be improved with combinations of anthracycline-containing chemotherapies and rituximab.
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              Primary cutaneous large B-cell lymphoma, leg type.

              Vibha Thomas, Robin Dobson, Robert Mennel (2011)
              Primary cutaneous large B-cell lymphoma, leg type, is a rare and aggressive neoplasm as defined by the recently updated World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. We present a case of a 74-year-old woman who presented with a cutaneous lesion on her forearm. Skin biopsy revealed pathology consistent with this entity. The patient was treated with systemic chemotherapy with rituximab combined with doxorubicin, cyclophosphamide, vincristine, and prednisone. Here, we review the available literature and summarize clinical features and management of this uncommon subtype of non-Hodgkin lymphoma.
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                Author and article information

                Contributors
                Camila Gonçalves Pinheiro
                Journal
                Journal ID (nlm-ta): An Bras Dermatol
                Journal ID (iso-abbrev): An Bras Dermatol
                Title: Anais Brasileiros de Dermatologia
                Publisher: Sociedade Brasileira de Dermatologia
                ISSN (Print): 0365-0596
                ISSN (Electronic): 1806-4841
                Publication date PMC-release: 15 January 2022
                Publication date (Print): Mar-Apr 2022
                Publication date (Electronic): 15 January 2022
                Volume: 97
                Issue: 2
                Pages: 179-183
                Affiliations
                [a ]Dermatology Service, Faculdade de Medicina Nova Esperança, João Pessoa, PB, Brazil
                [b ]Oncology Service, Hospital Napoleão Laureano, João Pessoa, PB, Brazil
                [c ]Centro de Diagnóstico Anatomopatológico, João Pessoa, PB, Brazil
                Author notes
                [* ]Corresponding author. camilagoncalves2@ 123456hotmail.com
                Article
                Publisher Item ID: S0365-0596(21)00299-3
                DOI: 10.1016/j.abd.2020.09.014
                PMC ID: 9073262
                PubMed ID: 35042641
                SO-VID: a5807d7b-184b-4a0d-b3cc-c43eb2e9d07f
                Copyright © © 2022 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U.
                License:

                This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

                History
                Date received : 19 May 2020
                Date accepted : 27 September 2020
                Categories
                Subject: Case Report

                Keywords: skin neoplasms,lymphoma,pharmacological treatment,radiotherapy,rituximab
                Data availability:
                Keywords: skin neoplasms, lymphoma, pharmacological treatment, radiotherapy, rituximab

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