良性淋巴管内皮瘤研究进展  

Research progress of benign lymphangioendothelioma

作　　者：文馨 胡宇凡 王雷 WEN Xin;HU Yufan;WANG Lei(Department of Dermatology,Xijing Hospital,School of Basic Medical Sciences,Air Force Medical University,Xi'an 710032,China;Battalion 10,No.3 Cadet Regiment,School of Basic Medical Sciences,Air Force Medical University,Xi'an 710032,China)

机构地区：[1]空军军医大学西京医院皮肤科,陕西西安710032 [2]空军军医大学基础医学院学员三大队十队,陕西西安710032

出　　处：《空军军医大学学报》2024年第3期352-355,共4页Journal of Air Force Medical University

基　　金：国家自然科学基金(82173415)。

摘　　要：良性淋巴管内皮瘤(BL)又称获得性进行性淋巴管瘤病,是一种罕见的良性淋巴管增生性疾病,目前全球共报告七十多例患者。本病临床表现通常为缓慢增大的、直径较大的红色或紫红色斑片,最大者直径可达20 cm以上。本病的诊断依赖于病理检查,其病理改变具有特征性,表现为累及真皮全层的管腔增生,在真皮内形成大量裂隙状的薄壁管腔,甚至形成胶原分割现象。增生的管腔壁主要由单层内皮细胞组成,没有明显的细胞异形性和核分裂相。增生的内皮细胞表达淋巴管标记,包括D2-40、Prox1等,同时具有Ki-67低增殖指数,可以与其他恶性血管肿瘤相鉴别。本病的发病机制尚不明确,目前也缺乏有效的治疗手段。本文系统性回顾了BL的临床和病理特征、鉴别诊断以及治疗进展。Benign lymphoangioendothelioma(BL),also known as acquired progressive lymphangiomatosis,is a rare benign lymphoproliferative disease.At present,more than 70 cases have been reported worldwide.The clinical manifestations of this disease are usually slowly enlarging,large diameter of red or purple patches,the maximum diameter of which can reach more than 20 cm.The diagnosis of the disease depends on pathological examination.The pathological changes are characteristic,manifested as luminal hyperplasia involving the whole dermis,the formation of a large number of slit-like thin-walled lumen in the dermis,and even the formation of collagen segmentation.The proliferative luminal wall is mainly composed of a single layer of endothelial cells without significant cellular atypia or mitotic figures.The proliferative endothelial cells express lymphatic markers,including D2-40,Prox1,etc.,and have a low proliferation index of Ki-67,which can be distinguished from other malignant vascular neoplasms.The pathogenesis of this disease is still unclear,and currently there is a lack of effective treatment.This paper systematically reviews the clinical and pathological features,differential diagnosis and treatment progress of BL.

关 键 词：良性淋巴管内皮瘤 胶原分割 病理学特征 鉴别诊断 

分 类 号：R733.1[医药卫生—肿瘤]