Beijing Da Xue Xue Bao Yi Xue Ban. 2017 Jun 18;49(3):495-500.
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.
Clinicopathological data, histological characteristics of 13 cases of LCA were retrospectively studied and immunohistochemical staining was imposed on the paraffi-nembedded specimens, and 5 cases of cavernous hemangioma, 4 cases of normal littoral cells of spleens were used as control groups, simultaneously.
All the 13 LCA patients included 7 males and 6 females, 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 FVIII RAg 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.
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.
探讨脾脏边缘细胞血管瘤(LCA)的临床病理特征、形态学特点及免疫表型,为其诊断及避免误诊提供新的依据。
回顾性分析13例LCA的临床病理资料及组织学特征,并对石蜡包埋标本进行免疫组化染色,同时以5例海绵状血管瘤、4例正常脾脏边缘细胞作为对照组。
13例LCA患者中,男性7例,女性6例,年龄39~70岁,平均54.2岁,中位年龄55岁。其中6例患者伴有腹腔恶性肿瘤、良性肿瘤或炎症状态,7例为体检时偶然发现。大体上,脾脏可见单个或多个灰红色结节,直径0.5~6.2 cm。组织学上,肿瘤由相互吻合的血管腔构成,内衬丰满、圆形至立方形的边缘细胞,这些细胞可延伸至血管腔内。部分区域可见由这些细胞覆盖的乳头状结构延伸至管腔内。管腔内还可见其他类型的组织细胞样细胞,其大小较边缘细胞大。两种细胞均无细胞学异型性。免疫组化研究显示,所有病例的边缘细胞血管内皮及组织细胞标记物如CD21、CD31、CD68、多克隆FVIII RAg及ERG均呈阳性,而CD8、CD34及WT-1呈阴性。这些结果表明LCA中边缘细胞的免疫表型与对照组不同。
LCA是一种良性病变,好发于老年人。其病因尚不明确,但部分病例因合并其他肿瘤或炎症,可能与免疫失调有关。LCA中的边缘细胞在免疫组化上表现为内皮细胞-组织细胞混合表型,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。强调组织学表现及免疫表型对诊断及鉴别诊断具有重要意义。