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目的探讨血管周上皮样细胞肿瘤(PEComa)的临床病理特点及鉴别诊断。方法报道1例发生于肝的血管周上皮样细胞肿瘤并对其临床表现、组织学形态、免疫表型及生物学行为进行分析,并复习相关文献。结果血管周上皮样细胞肿瘤是起源于血管周细胞的间叶性肿瘤,组织学上由单一的上皮样细胞组成,不含厚壁血管及脂肪组织,该肿瘤黑色素标记物HMB45(+)。结论血管周上皮样细胞肿瘤是一种非常罕见的间叶组织来源的肿瘤,具有独特的形态学和免疫组化特征,生物学行为不确定。临床以手术切除为主,且建议术后长期随访。
Objective To investigate the clinicopathological characteristics and differential diagnosis of perivascular epithelial cell tumor (PEComa). Methods One case of peritumoral neoplasia of the blood vessels in the liver was reported and its clinical manifestations, histological features, immunophenotypes and biological behavior were analyzed and related literatures were reviewed. Results Perivascular epithelioid tumor was a mesenchymal tumor originated from perivascular cells. Histologically, it consisted of a single epithelium-like cell without thick-walled blood vessels and adipose tissue. The tumor melanin marker HMB45 (+). Conclusion Perivascular epithelioid cell tumor is a very rare tumor of mesenchymal origin. It has unique morphological and immunohistochemical characteristics and uncertain biological behavior. Surgical resection of the main clinical and postoperative long-term follow-up.