乳腺乳头状淋巴管内血管内皮细胞瘤临床病理特征分析

Clinicopathological characteristics of papillary intralymthatic angioendothelioma of the breast

目的探讨乳腺乳头状淋巴管内血管内皮细胞瘤(PILA)的临床病理特征、免疫表型、诊断与鉴别诊断及预后。方法对1例乳腺PILA行HE及免疫组化染色观察,并进行随访及文献复习。结果该病主要病理改变为薄壁囊状扩张的大小不等血管腔隙,腔内衬覆内皮细胞及鞋钉样瘤细胞。管腔内瘤细胞排列呈乳头状皱褶和玻璃样变轴心。免疫组化显示肿瘤细胞CD34、CD31、vimentin和bcl-2(+)。随访6年无复发、转移,预后良好。结论乳腺PILA是一种非常罕见的交界性血管性肿瘤,组织学形态易与多种良性、恶性血管性肿瘤混淆。经扩大手术切除后预后良好。

Objective To investigate the clinicopathologic characteristics,immunophenotype,diagnosis,differential diagnosis and prognosis of papillary intralymthatic angioendothelioma（ PILA） of breast. Methods The histopathological and immunohistochemical characteristics of breast PILA were observed and analyzed,with review of relative literatures.Results The main pathological manifestations were the expansion of thinner wall cystic cavity coverd with boots nail like vascular endothelial cells. The tumor cells in cystic cavity arranged in papillary-like folds with hyaline axis. IHC showed that the tumor cells were positive for CD34,CD31,VIM,and bcl-2. The patient was not relapsed,metastasized and favourable prognosis after follow-up of six years. Conclusion PILA of the breast is a very rare vascular intermediate type tumor and the histological appearances of the tumor can be misdiagnosed easily as various benign and malignant vascular tumor. It is important to differentiate PILA from other diseases. After expanded excision,the prognosis of PILA is well.