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Cutaneous Lymphoma
Published in Debjani Sahni, Adam Lerner, Bilal Fawaz, Advanced Skin Cancer, 2022
CBCLs represent ~25% of all cutaneous lymphomas and are classified into three main types: Primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large B-cell lymphoma, leg-type (PC-DLBCL).1 PCFCL and PCMZL are low-grade, indolent malignancies with an overall good prognosis, whereas PC-DLBCL is known to behave more aggressively and have a worse overall survival rate.1 For all CBCLs, it is essential to perform a thorough staging evaluation at the time of diagnosis to rule out secondary cutaneous involvement by a systemic B-cell lymphoma as the prognosis is worse and the approach to treatment is very different in the latter scenario. Investigations include a CBC, CMP, LDH, flow cytometry of peripheral blood, CT of the chest, abdomen, pelvis, and, if feasible/indicated, PET/CT.1
Lymphomas and pseudolymphomas
Published in Aimilios Lallas, Enzo Errichetti, Dimitrios Ioannides, Dermoscopy in General Dermatology, 2018
Zoe Apalla, Aimilios Lallas, Enzo Errichetti
Primary cutaneous B-cell lymphomas (PCBCLs) represent about 25% of all PLCs. They are classified into three major categories: primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL L-T), and primary cutaneous marginal zone lymphoma (PCMZL).1
Skin
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Cutaneous B-cell lymphomas are much less common than primary cutaneous T-cell lymphomas and constitute ~20%–30% of all primary cutaneous lymphomas [2, 13, 14]. Primary cutaneous B-cell lymphomas are defined as B-cell lymphoproliferative disorders originating in the skin with no evidence of extracutaneous disease at presentation for at least 6 months after diagnosis. The WHO recognizes four main types of B-cell lymphomas in the skin: (1) primary cutaneous marginal zone lymphoma (MZL); (2) primary cutaneous follicle center cell lymphoma (PCFCCL); (3) primary cutaneous diffuse large B-cell lymphoma (DLBCL), leg type; and (4) primary cutaneous DLBCL, other [1–3, 15–26]. MZL occurs most commonly on trunk or extremities, especially the arms, followed by the head and neck area. In contrast to primary follicle center lymphoma, presentation with multifocal skin lesions is frequent. Primary cutaneous B-cell lymphomas are generally associated with a favorable prognosis, with good response rates to radiotherapy and chemotherapy. However, the relapse rates vary between 25% and 68% according to different studies [18, 27–30]. Large series of primary cutaneous B-cell lymphoma published by Zinzani et al. [31] confirmed the good overall prognosis of these tumors, with a 10-year overall survival rate of 85%, a 92% complete response rate after the first treatment, and a low tendency to extracutaneous spread. However, nearly half of the patients experienced relapse after the first treatment (46.7%) and 23.8% experienced two or more relapses during the follow-up [31]. Secondary B-cell lymphomas include mantle cell lymphoma (MCL), DLBCL, follicular lymphoma (FL), lymphomatoid granulomatosis, and precursor B-cell lymphoblastic lymphoma.
Enhancing prognostication and personalizing treatment of extranodal marginal zone lymphoma
Published in Expert Review of Hematology, 2023
Juan Pablo Alderuccio, Izidore S. Lossos
Marginal zone lymphoma (MZL) is a heterogeneous disease representing 7% of all non-Hodgkin lymphomas. The World Health Organization recognizes three subtypes according to the primary site of involvement: extranodal MZL (EMZL) of mucosa-associated lymphoid tissue (MALT), nodal MZL, and splenic MZL [1]. In the United States, EMZL is the most common subtype (61%), followed by nodal MZL (30%) and splenic MZL (9%) [2]. In the fifth edition of the World Health Organization classification of hematological malignancies, two additional separate entities will be recognized: primary cutaneous marginal zone lymphoma (PCMZL) that is now separated from the EMZL since it has clinical and pathologic features that distinguish it from other EMZL and pediatric nodal marginal zone lymphoma that is distinct from the nodal MZL observed in adults. In this review, we still include PCMZL as part of EMZL.
Primary cutaneous indolent B-cell lymphomas – a retrospective multicenter analysis and a review of literature
Published in Acta Oncologica, 2021
Magdalena Olszewska-Szopa, Marta Sobas, Kamel Laribi, Laura Bao Perez, Joanna Drozd-Sokołowska, Edyta Subocz, Monika Joks, Krzysztof Zduniak, Małgorzata Gajewska, Anna Kulikowska de Nalecz, Joanna Romejko-Jarosińska, Beata Kumiega, Anna Waszczuk-Gajda, Tomasz Wróbel, Anna Czyz
Most cutaneous lymphomas are T-cell-derived neoplasms. Primary cutaneous B-cell lymphomas (PCBCL) present in the skin with no systemic involvement at diagnosis constitute only 20–25% of skin lymphomas, at least 90% of which are primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicular lymphoma (PCFCL) and diffuse large B-cell lymphoma leg-type (DLBCL leg type). The updated WHO-EORTC classification of this group of lymphomas was published in 2018 [1,2].