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.
原发性皮肤B细胞淋巴瘤(PCBCLs)是指发生于皮肤且无皮肤外受累证据的B细胞淋巴瘤。根据临床病理学、免疫组织化学和遗传学特征，2005年WHO/EORTC皮肤淋巴瘤分类及其2018年更新版将其分为三个主要亚型：原发性皮肤边缘区淋巴瘤(PCMZL)、原发性皮肤滤泡中心淋巴瘤(PCFCL)和原发性皮肤弥漫性大B细胞淋巴瘤-腿型(PCDLBCL-LT)。其中PCMZL和PCFCL临床呈惰性病程，而PCDLBCL-LT属于侵袭性淋巴瘤。由于发病率低且缺乏前瞻性研究，各亚型目前难以建立标准化治疗方案。本研究旨在描述西班牙淋巴瘤肿瘤学组12个中心103例皮肤B细胞淋巴瘤患者的临床病理特征。患者中位年龄53岁（40-65岁）。根据皮肤累及范围分类：62%为单部位淋巴瘤，17%为区域性淋巴瘤，20%为多灶性淋巴瘤。组织学分型：66%为PCMZL，26%为PCFCL，8%为PCDLBCL-LT。治疗方式：23%患者仅接受手术治疗，26%仅接受放疗，21%接受手术联合放疗，10%接受多药化疗，5%接受利妥昔单抗单药治疗。总体完全缓解率达96%，44%患者后续出现复发，其中多数为局部或区域性复发。全队列10年总生存率为94.5%，其中PCMZL亚组为98%，PCFCL亚组为95%，PCDLBCL-LT亚组为85.7%。除PCMZL发生率较高外，本研究数据与其他已发表系列研究具有可比性，同时观察到治疗策略存在预期中的异质性。
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