脾脏硬化性血管瘤样结节性转化的临床病理特征

The clinicopathological characteristics of sclerosing angiomatoid nodular transformation of spleen

目的探讨脾脏硬化性血管瘤样结节性转化(sclerosing angiomatoid nodular transformation,SANT)的临床病理特征、诊断和发生机制。方法对5例脾脏SANT进行常规HE及免疫组化(SP法)观察并结合文献进行讨论。结果眼观病变呈单发或多发性结节,边界清楚,切面斑彩状。显微镜下特征性的表现是多发性血管瘤样结节形成,其周围被增生的纤维组织包绕。结节中央为裂隙样、不规则形或略扩张的血管腔,内衬肿胀的内皮细胞。血管腔隙之间为梭形细胞和卵圆形细胞。结节周围的纤维化组织中有淋巴细胞、浆细胞和组织细胞浸润。免疫组化显示结节内毛细血管内皮细胞CD34和CD31(+),CD8(-);窦内皮细胞CD8和CD31(+),CD34(-);结节内的小静脉CD31(+),CD34和CD8(-);结节周围梭形细胞vimentin、SMA(+),desmin(-);其内的炎细胞CD68(+)。其中1例伴发腹腔多发性钙化性纤维性肿瘤。结论脾脏SANT是一种少见的特殊的脾脏血管病变,有其特征性的临床病理特征,可以伴发腹腔多发性钙化性纤维性肿瘤,预后良好,应注意与脾脏其他类型肿瘤及瘤样病变鉴别。

Purpose To investigate the clinicopathological characteristics,differential diagnosis and pathogenesis of sclerosing angiomatoid nodular transformation of spleen（SANT）.Methods 5 cases of SANTs of spleen were studied by using routine hematoxylin-eosin staining（HE） and immunohistochemical staining.Results Macroscopically,grey white and grey brown multinodular lesion could be easily found and had a well-demarcated boundary.Microscopically,all of cases were characterized by multiple angiomatoid nodules of various sizes in a fibrosclerotic stroma.In the center of the angiomatoid nodules,vascular spaces with its diverse formed such as slit-like,irregular-shaped as well as slightly dilated vascular spaces lined by plump endothelial cells and interspersed with a population of spindly cells or ovoid cells.Immunohistochemically,the lining cells of the small capillaries expressed CD34,CD31,but not CD8.The endothelial cells of sinusoid expressed CD8 and CD31,but not CD34 while those of small veins only expressed CD31,but not CD34 and CD8.The spindle cells were vimentin（＋）,SMA（＋） and desmin（-）.The infiltrating inflammtery cells within the stroma were CD68（＋）.Conclusions Sclerosing angiomatoid nodular transformation is a rarely encountered benign lesion of the spleen,which has its own clinicpathological features.It should be distinguished from other angiomatoid tumors and tumor-like lesions.