脾脏硬化性血管瘤样结节性转化四例分析

目的探讨脾脏硬化性血管瘤样结节性转化(SANT)的临床病理表现、诊断和鉴别诊断要点及治疗方法。方法对4例SANT的临床资料、病理学表现、免疫组化特征和术后随访情况进行分析、讨论。结果4例SANT患者的临床表现无特异性。病理学检查示病灶呈灰白色结节,边界不清;镜下见多发性血管瘤样结节,结节中央可见裂隙样或窦样血管腔,血管腔隙周围散在卵圆形的组织细胞,结节周围围绕着致密的向心性分布的平滑肌或胶原纤维。免疫组化检测示部分结节内毛细血管内皮细胞呈CD34和F8阳性反应,但不表达CD8,而部分血管内皮细胞呈CD8阳性反应,但不表达CD34;血窦周围散在少量CD68阳性的组织细胞;结节周围梭形细胞呈Vimentin、SMA、Actin及Collage IV阳性反应;CD21、Desmin和NSE均阴性。术后随访,患者均未发现病变复发和转移。结论SANT是一种罕见的良性增生性病变,易与脾脏恶性肿瘤相混淆,诊断依靠病理组织学表现及免疫组化特点。行脾切除术可治愈,预后良好。

Analysis of 4 cases of sclerosing angiomatoid nodular transformation of spleen

Objective To investigate the clinicopathologic features,diagnosis,differential diagnosis and treatment of sclerosing angiomatoid nodular transformation(SANT). Methods The clinical data,pathologic characteristics,immunophenotype and postoperative follow-up of SANT were analysed. Results There were no specific findings in the clinical manifestations of the 4 cases of SANT.Grossly,the cut surface of the masses was gray-white and vague nodularity was observed.Microscopically,it was characterized by the multinodular angiomatoid appearance in a fibrosclerotic stroma.The nodules were composed of slit-like,sinusoid-like vascular spaces and were interspersed with a population of spindly or ovoid cells.It was revealed by immunohistochemistry that the expression of CD34 in some vessels' endothelial cells was positive,and CD8 was negative.While in another vessels' endothelial cells,CD8 was positive and CD34 was negative.The expression of SMA,Actin,Vimentin,Collage IV and CD68 was positive in all of the 4 cases,while that of CD21,Desmin and NSE was negative.No relapse or metastasis was found during the follow-up.Conclusion SANT is a rarely encountered benign lesion of the spleen,which should be distinguished from the malignant tumor of the spleen.The diagnosis counts on the pathologic and immunohistochemical findings.It could be cured by splenectomy with a favourable prognosis.