脾硬化性血管瘤样结节性转化临床病理分析和文献复习

Pathological analysis of sclerosing anagiomatoid nodular transformation of spleen

目的探讨脾硬化性血管瘤样结节性转化(sclerosing anagiomatoid nodular transformation,SANT)的临床病理特征、诊断及鉴别诊断。方法分析4例SANT的临床资料、组织病理学表现、免疫表型特征和术后随访情况。结果 4例SANT患者男女各2例,临床表现为无包膜肿块,大体为3～5.9 cm边界清楚的灰白色结节。镜下见特征性的血管瘤样结节,免疫组织化学显示结节内内皮细胞异质性表达,即小叶状分布毛细血管,免疫表型为CD34、CD31阳性,CD8阴性,窦岸样细胞免疫表型为CD31、CD8阳性,CD34阴性,小静脉样血管免疫表型为CD31阳性,CD8、CD34阴性,结节内梭形细胞混合表达平滑肌肌动蛋白(smooth muscle actin,SMA)和CD68。4例患者现均生存。结论 SANT是脾脏具有特征性病理改变的一种良性病变,临床上表现为无包膜肿块,诊断依靠病理组织学表现和免疫组织化学特点,行脾切除术可治愈,预后良好。

Objective To review the clinicopathological features,diagnosis and differential diagnosis of sclerosing angiomatoid nodular transformation（SANT）of spleen.Methods The clinical data,pathological findings,immunophenotypes and postoperative follow-up of 4 cases of SANT were investigated.Results There were no specific clinical manifestations in 4 cases of SANT,in which 3 cases were found in health check-up.Two were male and two female.Grossly,on the cut surface the masses were gray-white and un-encapsulated nodular measuring 3~5.9 cm in size.Microscopically,SANT was characterized by micronodular lesions consisting of vascular spaces lined by plump endothelial cells with interspersed spindle cells.Immunohistochemical results showed that the endothelial cells of vessels in angiomatoid nodules had various phenotypes： CD34＋/CD31＋/CD8-in endothelial cells of capillaries,CD34-/CD31＋/CD8＋ in lining cells of the sinusoids and CD34-/CD31＋/CD8-in endothelial cells of small veins.All patients were alive.Conclusions SANT is a benign lesion specially arising in spleen with a characteristic pathological appearance.The diagnosis relies on the pathological and immunohistochemical findings.The disease can be cured by splenectomy with a favorable prognosis.