脾脏硬化性血管瘤样结节性转化临床病理分析并文献复习

Sclerosing angiomatoid nodular transformation of the spleen:a clinicopathologic analysis and review of literature

目的探讨脾脏硬化性血管瘤样结节性转化(SANT)的临床病理特征、诊断及鉴别诊断、治疗与预后。方法分析3例SANT患者临床表现、病理组织学特征及免疫组化表型特点,并复习国内外相关文献。结果病变多位于红髓,由多个分叶状大的血管瘤样结节构成,血管瘤样结节由窦样、裂隙样或不规则的血管腔隙组成,腔隙内衬肥胖的内皮细胞,结节间多为致密的纤维结缔组织。免疫组化标记显示血管瘤样结节内存在三种类型的小血管,即毛细血管型:CD34(+)/CD31(+)/CD8(-);窦样腔隙型:CD34(-)/CD31(+)/CD8(+);小静脉型:CD34(-)/CD31(+)/CD8(-)。结论SANT是一种罕见的原发于脾脏非肿瘤性血管病变,具有独特的病理组织学特点,需要与其他肿瘤如脾错构瘤、炎性肌纤维母细胞瘤及窦岸细胞血管瘤等相鉴别。脾脏切除可获治愈,预后好。

Objective To explore the clinicopathological characteristics,diagnosis and differential diagnosis,treatment and prognosis of sclerosing angiomatoid nodular transformation(SANT)of the spleen.Methods Clinical manifestation,pathomorphological characteristics and immunohistochemical features of 3 cases of SANT were analyzed,and relevant literatures were reviewed.Results The main pathological features were that the lesions mostly located in the splenic red pulp,and presented mostly with solitary nodules,but also multifocal,and consisted of several large hemangiomatous nodules,which were composed of sinusoid,fissure,or irregular vascular spaces lined with plump endothelial cells.There was usually dense fibrous connective tissue between the nodules.The immunohistochemical staining revealed three different types of blood vessels:capillary type(CD34+/CD31+/CD8-),sinusoid lacuna type(CD34-/CD31+/CD8+),and small vein type(CD34-/CD31+/CD8-).Conclusion SANT is a rare non-neoplastic vascular lesion originating in the spleen with unique pathologic characteristics.It needs to be differentiated from other tumors such as splenic hamartoma,inflammatory myofibroblastoma,and sinusioside hemangioma.The splenectomy can be cured and the prognosis is good.