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, N.; Martínez-Madueño, F.; Caballé, B.; Badia, J.; Blanes, M.; Bujanda, D.A.; Calvo, V.; Gómez Codina, J.; Blanco, C.Q.; Espinosa, P.; Lavernia, J.; Arroyo, F.R.G.; Risueño, M.G.; Llorca, C.; Cumeras, R.; Pulla, M.P.; Gumà, J. A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL). Cancers 2024, 16, 1034. Martínez-Banaclocha, N.; Martínez-Madueño, F.; Caballé, B.; Badia, J.; Blanes, M.; Bujanda, D.A.; Calvo, V.; Gómez Codina, J.; Blanco, C.Q.; Espinosa, P.; Lavernia, J.; Arroyo, F.R.G.; Risueño, M.G.; Llorca, C.; Cumeras, R.; Pulla, M.P.; Gumà, J. A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL). Cancers 2024, 16, 1034.

Abstract

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.

Keywords

cutaneous B lymphoma; skin; surgery; radiotherapy; chemotherapy; rituximab

Subject

Medicine and Pharmacology, Oncology and Oncogenics

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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