[Translated article] Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years

Primary cutaneous lymphomas (PCL) are non-Hodgkin lymphomas, presenting in the skin. They are classified into cutaneous B-cell (CBCL), or cutaneous T-cell lymphomas (CTCL), the latter being the most common. The most common CTCL subtypes are mycosis fungoides (MF) and Sézary syndrome (SS).1 All cases should be discussed at a specialist multidisciplinary team (MDT) meeting.2 This report is the first published review of PCL MDT case discussion in the UK. Cases from the supra-regional specialist cutaneous lymphoma MDT in Glasgow, between 2008 and 2019, were reviewed. Our objectives were: assess frequency of PCL subtype; review documentation of CTCL staging; review management of MF/SS. Of 356 cases, 103 (29%) were CBCL. The majority (n=200, 56%) were CTCL. MF/SS was the final diagnosis in 120 (34%). Staging was documented in 44% (n=53) MF/SS cases. Management largely followed guidelines, topical corticosteroids (TCS) were the most common treatment (n=93, 87%) (Figure 1).2 Frequency of PCL subtype is largely comparable to previously published data (Table 1). Documentation of CTCL staging is low, but higher than other reports.3 Our work begins to address the gap in real-world data on CTCL. Going forward, a standardised approach to data collection would inform clinical practice.

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Primary cutaneous lymphomas: exploration of 10-year data from supraregional mutlidisciplinary team meetings in Glasgow, UK

McCusker

Ngu

2023

Clinical and Experimental Dermatology

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A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL)

Martínez-Banaclocha,

Martínez-Madueño,

Caballé

et al. 2024

Cancers

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Primary cutaneous B-cell lymphomas (PCBCLs) are B-cell lymphomas that can occur in the skin without evidence of extracutaneous involvement. The 2005 WHO/EORTC classification of cutaneous lymphomas and its 2018 update have distinguished three main categories based on clinicopathological, immunohistochemical, and genetic characteristics: primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle centre lymphoma (PCFCL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT). PCMZL and PCFCL are clinically indolent, while PCDLBCL-LT is an aggressive lymphoma. Due to its low incidence and lack of prospective studies, it is difficult to establish a standard treatment for each subgroup. The objective of our study was to describe the clinical and pathological characteristics of 103 patients with cutaneous B-cell lymphoma from 12 centres belonging to the Spanish Lymphoma Oncology Group. The median age was 53 years (40–65). According to skin extension, 62% had single-site lymphoma, 17% had regional lymphoma, and 20% had multifocal lymphoma. Histology: 66% had PCMZL, 26% had PCFCL, and 8% had PCDLBCL-LT. Twenty-three percent of the patients were treated exclusively with surgery, 26% with radiotherapy only, 21% with surgery plus radiotherapy, 10% with polychemotherapy, and 5% with rituximab monotherapy. Overall, 96% of patients achieved a complete response, and 44% subsequently relapsed, most of them relapsing either locally or regionally. The 10-year OS was 94.5% for the entire cohort, 98% for the PCMZL cohort, 95% for the PCFCL cohort, and 85.7% for the PCDLBCL-LT cohort. Our data are comparable to those of other published series, except for the high frequency of PCMZL. The expected heterogeneity in therapeutic management has been observed.

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[Translated article] Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years

Cited by 2 publications

References 19 publications

Primary cutaneous lymphomas: exploration of 10-year data from supraregional mutlidisciplinary team meetings in Glasgow, UK

Primary cutaneous lymphomas: exploration of 10-year data from supraregional mutlidisciplinary team meetings in Glasgow, UK

A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL)

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