脾窦岸细胞血管瘤2例临床病理观察

目的探讨脾窦岸细胞血管瘤(LCA)的临床病理及相关性、免疫表型特征、诊断与鉴别诊断要点。方法对2例脾LCA进行临床资料、影像学特征及病理形态学分析,进行多种免疫组化标记,并复习相关文献。结果 2例脾LCA有相似的临床表现(脾大、脾功能亢进),CT示脾实质内多发及单发性低密度影,增强扫描有对比增强表现。2例大体病理均为脾不同程度增大,例1脾实质内有多个暗红色结节;例2为单发结节,并伴多发性肾囊肿病、肝囊肿。2例镜下改变均为瘤组织由大小不等的窦样腔隙构成,腔壁向腔内形成乳头状结构,少数呈囊状扩张,内衬单层内皮样细胞。这些细胞有两种类型,一类内衬细胞呈高柱状,具有巨噬细胞/组织细胞样形态,体积较大,部分游离在腔内呈稀疏片状;另一类内衬细胞形似正常窦岸细胞,体积较小;两类细胞无明显异型。2例LCA大细胞CD68/kp1、CD163和溶菌酶(+),少数大细胞CD31(+),CD34(-);2例小细胞CD31、多克隆第Ⅷ因子相关抗原和CD21(+),CD8弱(+)/(-),CD34(-),组织细胞抗原均(-)。大、小细胞S-100、单克隆FⅧAg均(-)。结论 LCA是一种较为罕见的良性病变,特征性的病理形态改变及CD31、多克隆FⅧ抗原和组织细胞抗原的检测有利于诊断,应密切随访,以除外肿瘤复发及伴发恶性肿瘤可能。

Littoral cell angioma of spleen:a clinicopathologic analysis of two cases

Objective To study the clinicopathologic features,immunophenotype,and the main points of diagnosis of splenic littoral cell angioma.Methods The clinical features,radiologic findings and histopathologic changes were analyzed in 2 cases of splenic littoral cell angioma.Immunohistochemical study was carried out on paraffin-embedded tissues,and relevant literatures were reviewd.Results All the 2 cases had similar clinical manifestations and radiologic findings,namely splenomegaly and hypersplenism.CT showed mul...