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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 clinicopathological characteristics, immunophenotype, diagnosis and differential diagnosis of breast papillary lymphatic vessel endothelial cell tumor (PILA) and its prognosis. Methods One case of breast PILA was observed by HE and immunohistochemical staining, followed up and literature review. Results The main pathological changes of the disease were thin-walled cystic dilatation ranging in size from vascular lacunar, lined endothelial cells and spike-like tumor cells. Lumens tumor cells arranged in papillary folds and vitrified axis. Immunohistochemistry showed tumor cells CD34, CD31, vimentin and bcl-2 (+). Follow-up 6 years without recurrence, metastasis, the prognosis is good. Conclusion Breast PILA is a very rare borderline vascular tumor, histological morphology easily confused with a variety of benign and malignant vascular tumors. The prognosis is good after the excised extended surgery.