脾脏硬化性血管瘤样结节性转化的临床病理研究

Clinicopathological study on sclerosing angiomatoid nodular transformation of spleen

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。方法观察、分析中山大学附属第五医院3例SANT患者的临床影像学资料、病理学特征并复习相关文献。结果低倍镜下显示,脾脏组织中多见结节样结构,类似于肉芽肿的表现,部分结节分界不清。结节内可见窦样、裂隙状或不规则形血管腔结构,以及较肥胖的内皮细胞,结节之间可见增生的纤维组织,部分伴胶原化,伴散在的淋巴细胞、浆细胞浸润,并可见含铁血黄素沉积。免疫组织化学染色显示3种不同类型的血管:CD34^+/CD31^+/CD8^-的毛细血管、CD34^-/CD31^+/CD8^-的小静脉和CD34^-/CD31^+/CD8^+的窦隙。结节间梭形细胞SMA、CD68标记阳性,S-100标记阴性。结论 SANT是一种罕见的脾脏原发的良性瘤样病变,有特征性的组织学及免疫组织化学表现,需要与脾脏的其他良恶性肿瘤及瘤样病变相鉴别。

Objective To explore the clinicopathological characteristics,diagnosis and differential diagnosis of sclerosing angiomatoid nodular transformation(SANT)of the spleen.Methods The clinical imaging data and pathological features in 3 cases of SANT in the Fifth Affiliated Hospital of SunYat-Sen University were observed and analyzed,and the related literature was reviewed.Results At low power microscope,multiple nodular structures were seen in the spleen tissue,which were similar to the manifestations of granuloma,and some nodules had unclear boundary.Sinusoid,fissure-like or irregular vascular cavity structure could be seen in the nodules,and relatively fat endothelial cells could be seen.The hyperplastic fibrous tissue could be seen in the nodules,the part of which was accompanied by collagenation,scattered lymphocytes and plasma cells infiltration,moreover hemosiderosis could be seen.The immunohistochemical staining revealed three different types of blood vessels:CD34^+/CD31^+/CD8^-capillaries,small veins of CD34^-/CD31^+/CD8^-and sinusoids of CD34^-/CD31^+/CD8^+.The spindle cell SMA and CD68 marker between the nodules was positive and the the S-100 marker was negative.Conclusion SANT of the spleen is a rare benign tumor-like lesion of the spleen,which has characteristic histological and immunohistochemical manifestations and needs to be differentiated from other benign and malignant tumors and tumor-like lesions in the spleen.