摘要
目的:探讨脾窦岸细胞血管瘤(littoral cell angioma,LCA)的临床病理特征、形态学特点和免疫表型,为正确的诊断、避免误诊提供依据。方法:回顾性研究13例LCA患者的临床病理资料、组织学特点并将LCA的石蜡包埋标本进行免疫组织化学染色,同时将5例海绵状血管瘤和4例正常脾窦岸细胞作为对照组。结果:13例LCA患者包括7名男性和6名女性,年龄从39~70岁,平均54.2岁,中位年龄55岁,这些肿瘤患者中,6例伴有腹腔内恶性、良性肿瘤或者炎症,7例由体格检查被偶然发现。肿瘤标本大体观发现脾内含有直径为0.5~6.2 cm孤立性或者多发性灰白色结节。肿瘤组织学特征为由相互吻合的血管腔隙构成,血管腔隙内排列丰富的、从圆形到立方状窦岸细胞,并延伸到血管腔,通常可以看到在肿瘤的一些区域中,覆盖于乳头状结构中的窦岸细胞延伸到血管腔内,在血管腔中还可以辨认出另外一种组织细胞样细胞,大小比窦岸细胞大,两种细胞均未显示细胞学非典型性。免疫组织化学显示所有病例中的窦岸细胞对血管内皮细胞和组织细胞标记物(如CD21、CD31、CD68、多克隆FⅧRAg和ERG)呈阳性表达,而这些细胞对CD8、CD34和WT-1呈阴性表达,表明LCA中的窦岸细胞的免疫表型与对照组不同。结论:LCA是一种良性病变,多发生在老年人,它的病因尚不清楚,然而,由于在一些病例中伴随的其他肿瘤或炎症,因此免疫失调可能和该肿瘤存在关联,LCA中的窦岸细胞在免疫组织化学中呈内皮细胞-组织细胞混合性表型,因此这些细胞可能具有介于内皮细胞和组织细胞之间的特征,强调组织学检查和免疫表型对诊断和鉴别诊断至关重要。
Objective: To investigate the clinicopathological features,morphological characteristics,immunophenotypes of littoral cell angioma( LCA) in spleen,and to provide new evidence for making diagnosis and avoiding misdiagnosis. Methods: Clinicopathological data,histological characteristics of 13 cases of LCA were retrospectively studied and immunohistochemical staining was imposed on the paraffinembedded specimens,and 5 cases of cavernous hemangioma,4 cases of normal littoral cells of spleens were used as control groups,simultaneously. Results: All the 13 LCA patients included 7 males and 6females,aged from 39 to 70 years with an average of 54. 2 years and a median age of 55 years. Among these tumor patients,6 cases were accompanied by malignances,benign tumors or inflammation states at abdominal cavities,and 7 cases were accidentally discovered by physical examinations. Grossly,spleens contained solitary or multiple gray red nodules,which ranged from 0. 5 to 6. 2 cm in diameter. Histologically,tumors were composed by anastomosing vascular spaces which were lining by plump,rounded to cuboidal littoral cells that extended into vascular lumens. Usually,papillary frameworks that were covered by these cells were also seen extending into the lumens in some areas. Other types of histiocytoid cells were identified in lumens and the sizes were larger than the littoral cells. Both types of cells absented cytologic atypia. Immunohistochemical study demonstrated that the littoral cells in all cases were positive for vascular endothelial and histiocyte markers,such as CD21,CD31,CD68,polyclone FⅧRAg and ERG,while these cells were negative for CD8,CD34,and WT-1. These findings manifested that immunophenotype of littoral cell in LCA distinctive from that in controls. Conclusion: LCA is a benign lesion,which frequently occurs in the elderly. Its etiology remains confusion,however,immune dysregulation may associate with it because of the concomitance with other tumor or inflammation in some cases. The littoral cells in LCA show a hybrid endothelial-histiocytic phenotype on immunohistochemistry,therefore these cells may have features that intermediate between those of endotheliocytes and histiocytes. Emphasizing the histological findings and immunophenotypes is significant for diagnosis and differential diagnosis.
出处
《北京大学学报(医学版)》
CAS
CSCD
北大核心
2017年第3期495-500,共6页
Journal of Peking University:Health Sciences
关键词
窦岸细胞血管瘤
免疫表型分型
病理学
临床
诊断
诊断
鉴别
littoral cell angioma,immunophenotyping,Pathology,clinical
Diagnosis
Diagnosis differential