脾硬化性血管瘤样结节性转化临床病理观察

目的探讨脾硬化性血管瘤样结节性转化(SANT)的临床病理特征、鉴别诊断、治疗及预后。方法分析1例SANT患者的临床病理特征及免疫组化表达,并复习相关文献。结果镜下病变呈多个硬化性血管瘤样结节,结节中央为裂隙样及不规则的富含红细胞的血管样腔隙,边缘间质内见数量不等的淋巴细胞、浆细胞及组织细胞浸润。免疫组化示vimentin弥漫(+),血管瘤样结节CD31和CD34(+),CD8(-);多结节CD235α及CD 61(+);结节中靶环状排列的梭形细胞及卵圆形细胞SMA、actin、CD 68、S-100、lysozyme、EMA及CD1α不同程度(+);CK-pan、ALK、CD21、CD23均(-)。随访6个月无复发及转移。结论SANT是一种罕见具有独特的临床病理改变的良性病变,确诊需依据其特征性的病理学形态及免疫组化结果,脾切除术可以治愈。

Sclerosing angiomatoid nodular transformation of spleen:a clinicopathologie analysis

Objective To explore the clinicopathological features,differential diagnosis,treatment and prognosis of splenic sclerosing hemangiomatoid nodular transformation(SANT),so as to improve the understanding of the disease and avoid misdiagnosis.Methods The clinicopathological features and immunohistochemical expression of a patient with SANT were analyzed.The follow-up of the patient was reported and the related literature was reviewed.Results Microscopically,the lesions showed multiple sclerosing hemangioma-like nodules,with fissure-like and irregular blood vessel-like spaces rich in red blood cells in the center,and infiltration of lymphocytes,plasma cells and histiocytes in the marginal interstitium.Immunohistochemistry showed that vimentin was diffuse positive,CD31 and CD34 were positive,CD8 was negative,and CD235αand CD61 were multinodular positive;Spindle cells and oval cells in the nodules were arranged annularly with different positivity;CK-pan,ALK,CD21 and CD23 were negative;No recurrence or metastasis was found after 6 month of follow-up.Conclusion SANT is a rare benign lesion with unique clinicopathological changes.Its diagnosis depends on its characteristic pathological morphology and immunohistochemical staining.Splenectomy can be cured.