在晚期，皮肤T细胞淋巴瘤（CTCL）可以转移到皮肤外区域。转移的CTCL表现出独特的临床病理特征。本研究在一个单一机构收集了20年间的35例原发性伴有皮肤外转移的CTCL病例。分析了人口统计学特征、CD30表达、大细胞转变、转移部位、T细胞受体克隆性研究和生存数据等临床病理特征。研究确定了包括真菌病样肉芽肿（MF）、塞扎里综合征（SS）、皮肤非典型大细胞淋巴瘤（C-ALCL）和原发性皮肤外周T细胞淋巴瘤，未明确分类（pcPTCL-NOS）在内的不同CTCL实体。有限的数据显示，转移可以独立于大细胞转变和/或CD30表达。淋巴结是最常见的转移部位，其次是骨髓。口咽转移很可能伴随内脏器官或脑转移（P = .049）。MF的转移时间间隔比SS长（P = .038）。只有淋巴结转移的患者比其他部位有转移的患者有更好的生存率（P = .012）。据我们所知，目前很少有研究以转移作为单一人群分析不同CTCL实体的临床病理特征。本研究为了解转移性CTCL的独特特征提供了宝贵的见解。版权所有 © 2023 Elsevier Inc. 保留所有权利。

In advanced stages, Cutaneous T-cell lymphomas (CTCL) can metastasize to extracutaneous regions. CTCL with metastasis exhibits unique clinicopathologic characteristics.This study collected 35 cases of primary CTCL with extracutaneous metastasis from a single institution over a period of 20 years. Clinicopathologic features including demographics, CD30 expression, large cell transformation, metastatic sites, T-cell receptor clonality studies and survival data were analyzed.The study identified various CTCL entities including mycosis fungoides (MF), Sezary syndrome (SS), cutaneous anaplastic large cell lymphoma (C-ALCL), and primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS). Limited data showed that metastasis can be independent of large cell transformation and/or CD30 expression. Lymph nodes were the most common site of metastasis, followed by the bone marrow. Oropharyngeal metastasis is likely to accompany visceral organ or brain metastasis (P = .049). MF had a longer interval to metastasis than SS (P = .038). Patients with lymph node only metastasis have better survival than patients with metastasis to other sites (P = .012).To the best of our knowledge, there are limited studies analyzing the clinicopathologic features of different CTCL entities with metastasis as a single population. This research provides valuable insights into the unique characteristics of metastatic CTCL.Copyright © 2023 Elsevier Inc. All rights reserved.