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Cancer (WHO–EORTC) ’ Most Common Group of Extranodal Non-Hodgkin S Classification (Table 1)[1]

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Cancer (WHO–EORTC) ’ Most Common Group of Extranodal Non-Hodgkin S Classification (Table 1)[1] Annals of Oncology 24 (Supplement 6): vi149–vi154, 2013 clinical practice guidelines doi:10.1093/annonc/mdt242 Published online 17 July 2013 Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up† R. Willemze1, E. Hodak2, P. L. Zinzani3, L. Specht4 & M. Ladetto5, on behalf of the ESMO Guidelines Working Group* 1Department of Dermatology, Leiden University Medical Centre, Leiden, The Netherlands; 2Department of Dermatology, Rabin Medical Centre, Beilinson Hospital, Petach Tikva, Israel; 3Institute of Haematology and Medical Oncology, University of Bologna, Bologna, Italy; 4Department of Oncology and Haematology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; 5Department of Molecular Biotechnology and Health Sciences, University of Torino, Torino, Italy, These Clinical Practice Guidelines are endorsed by the Japanese Society of Medical Oncology (JSMO) epidemiology skin or peripheral blood may be a valuable adjunct in selected cases. However, clinical and histopathological features are, in fi Primary cutaneous lymphomas (PCLs) are de ned as non- most cases, the most important deciding factors for therapeutic ’ Hodgkin slymphomasthatpresentintheskinwithno planning. PCLs should be classified according to the criteria of evidence of extracutaneous disease at the time of diagnosis. the World Health Organisation–European Organisation for After the gastrointestinal lymphomas, PCLs are the second Research and Treatment of Cancer (WHO–EORTC) ’ most common group of extranodal non-Hodgkin s classification (Table 1)[1]. lymphomas with an estimated annual incidence of 1/100 000 in Western countries. PCLs must be distinguished from nodal or systemic malignant lymphomas involving the staging skin secondarily, which often have another clinical In all cases, adequate staging should be carried out to exclude behaviour, have a different prognosis and require a different the presence of extracutaneous disease. Staging includes fi guidelines therapeutic approach. In recent lymphoma classi cations, complete physical examination, complete and differential clinical practice PCLs are therefore included as separate entities. Within the blood cell count and serum biochemistry and appropriate group of PCLs, distinct types of cutaneous T-cell lymphoma imaging studies (computed tomography scans ± [18F]2-fluoro- (CTCL) and cutaneous B-cell lymphoma (CBCL) can be 2-deoxy-D-glucose–positron emission tomography scans in all distinguished [1, 2]. In the western world, CTCL constitutes but stage IA), although they are not required in patients with ∼ – 75% 80% of all PCLs, with mycosis fungoides (MF) as the lymphomatoid papulosis (LyP) [5, 6]. Flow cytometry of the ∼ – most common type of CTCL, and CBCL 20% 25% [1]. peripheral blood should only be carried out in selected cases, However, different distributions have been observed in other but is mandatory in patients with (suspected) Sézary syndrome parts of the world. In southeast Asian countries, CTCLs (SS). Bone marrow biopsy and aspiration should be carried out other than MF, in particular Epstein-Barr virus-associated in cutaneous lymphomas with an intermediate or aggressive natural killer/T-cell lymphomas, are much more common clinical behaviour, but is not required in cutaneous than in Western countries, while CBCLs are much more lymphomas with an indolent clinical behaviour (MF, uncommon [3, 4]. cutaneous anaplastic large-cell lymphoma and cutaneous marginal zone lymphoma), unless indicated by other staging diagnosis assessments [5, 6]. The significanceofbonemarrow examination in primary cutaneous follicle centre lymphoma fi The diagnosis and classi cation of PCLs should always be based (PCFCL) is controversial [6, 7]. Prognosis is extremely variable on a combination of clinical, histological and depending on the type of PCL and the stage of disease. For immunophenotypical data. Demonstration of clonal T-cell clinical staging of MF and SS, the revised International Society receptor or immunoglobulin gene rearrangements in lesional for Cutaneous Lymphomas/European Organization of Research and Treatment of Cancer (ISCL/EORTC) TNMB (tumour–node–metastasis-blood) staging system should be *Correspondence to: ESMO Guidelines Working Group, ESMO Head Office, Via L. Taddei 4, CH-6962 Viganello-Lugano, Switzerland; used (Tables 2 and 3)[5].ForPCLotherthanMF/SS,a E-mail: [email protected] separate ISCL/EORTC TNM classification system has been published [6]. This staging system is primarily intended to †Approved by the ESMO Guidelines Working Group: December 2006, last update June 2013. This publication supersedes the previously published version. Ann Oncol 2010; 21 document the extent of disease and cannot be used as a (Suppl. 5): v177–v180. prognostic guide. © The Author 2013. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For permissions, please email: [email protected]. clinical practice guidelines Annals of Oncology Table 1. World Health Organisation–European Organisation for Table 2. Revised TNMB classification of mycosis fungoides (MF) and Research and Treatment of Cancer (WHO–EORTC) classification Sézary syndrome (SS) Cutaneous T-cell lymphoma (CTCL) T (skin) Mycosis fungoides (MF) T1 Limited patch/plaque (involving <10% of total skin surface) ≥ Variants of MF T2 Generalised patch/plaque (involving 10% of total skin surface) Folliculotropic MF T3 Tumour(s) Pagetoid reticulosis T4 Erythroderma Granulomatous slack skin N (lymph node) Sézary syndrome (SS) N0 No clinically abnormal peripheral lymph nodes Primary cutaneous CD30-positive lymphoproliferative disorders N1 Clinically abnormal peripheral lymph nodes; histologically Primary cutaneous anaplastic large-cell lymphoma uninvolved Lymphomatoid papulosis N2 Clinically abnormal peripheral lymph nodes; histologically involved Subcutaneous panniculitis-like T-cell lymphoma (nodal architecture uneffaced) Extranodal natural killer (NK)/T-cell lymphoma, nasal-type N3 Clinically abnormal peripheral lymph nodes; histologically involved Primary cutaneous peripheral T-cell lymphoma-not otherwise specified (nodal architecture (partially) effaced) Aggressive epidermotropic CD8+ CTCLa Nx Clinically abnormal peripheral lymph nodes; no histological Cutaneous γ/δ T-cell lymphomaa confirmation CD4+ small/medium-sized pleomorphic CTCLa Cutaneous B-cell lymphoma (CBCL) M (viscera) Primary cutaneous marginal zone B-cell lymphoma M0 No visceral involvement Primary cutaneous follicle centre lymphoma M1 Visceral involvement Primary cutaneous diffuse large B-cell lymphoma, leg type B (blood) aProvisional entities. B0 No circulating atypical (Sézary) cells (or <5% of lymphocytes) ≥ Republished with permission of the American Society of Hematology, B1 Low blood tumour burden ( 5% of lymphocytes are Sézary cells, from Willemze R, Jaffe ES, Burg G et al., WHO/EORTC classification but not B2) ≥ for cutaneous lymphomas Blood 2005; 105: 3768–3785; permission B2 High blood tumour burden ( 1000/µl Sézary cells and positive conveyed through Copyright Clearance Center, Inc. clone) Republished with permission of American Society of Hematology, from Olsen E, Vonderheid E, Pimpinelli N et al., Revisions to the staging and therapy classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous The choice of treatment depends on the type of PCL and the Lymphoma Task Force of the European Organization of Research and stage of disease. Due to their heterogeneity and rarity, controlled Treatment of Cancer (EORTC), Blood 2007; 110: 1713–1722; permission clinical trials in PCLs are almost non-existent, with a few conveyed through Copyright Clearance Center, Inc. exceptions mainly concerning recently marketed drugs. Recommendations are therefore largely based on (retrospective) cohort studies and expert opinions discussed during consensus meetings of the EORTC Cutaneous Lymphoma Group, the International Society for Cutaneous Lymphomas (ISCL), the United States Cutaneous Lymphoma Consortium (USCLC) and Table 3. Revised clinical staging system for mycosis fungoides (MF) and the International Lymphoma Radiation Oncology Group. Sézary syndrome (SS) Consensus recommendations for clinical end points and response criteria in MF/SS and in PCLs other than MF/SS have Clinical stage recently been published (Olsen EA, Willemze R, Wood GS et al. IA T1 N0 M0 B0-1 Clinical endpoints and response criteria in primary cutaneous IB T2 N0 M0 B0-1 lymphomas other than mycosis fungoides and Sézary IIA T1–2 N1-2 M0 B0-1 syndrome: a Consensus Statement of the International Society IIB T3 N0-2 M0 B0-1 for Cutaneous Lymphomas (ISCL), the United States III T4 N0-2 M0 B0-1 Cutaneous Lymphoma Consortium (USCLC) and the IVA1 T1-4 N0-2 M0 B2 Cutaneous Lymphoma Task Force of the European IVA2 T1-4 N3 M0 B0-2 Organization for the Research and Treatment of Cancer IVB T1-4 N0-3 M1 B0-2 (EORTC). Blood. In preparation.) [8]. Republished with permission of American Society of Hematology, from Olsen E, Vonderheid E, Pimpinelli N et al., Revisions to the staging and mycosis fungoides and variants classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Since early aggressive chemotherapy is associated with Lymphoma Task Force of the European Organization of Research and considerable side-effects but does not improve survival, a stage- Treatment
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