胰腺与肝脏多灶性血管周上皮样细胞肿瘤1例及文献复习

摘要/Abstract

摘要： 血管周上皮样细胞肿瘤（PEComa）是一组具有相似免疫表型而形态各异的间叶源性肿瘤。多灶性PEComa非常罕见,认识不足对其诊断和治疗造成困扰。在某些情况下,它可能被诊断为转移性肿瘤,并采取激进的治疗手段。本文报道1例罕见的肝脏和胰脏同时出现的PEComa病例。同时,我们回顾了2000—2024年文献报道过的12例多灶性PEComa病例,分析其临床病理特征,并探讨其可能的发病机制。本例患者女,28岁。体检发现胰头和肝脏5、6段连接区两处占位性病变,临床初始怀疑为胰腺肿瘤伴肝转移。第1次手术时行肝脏肿块切除和胰腺肿块活检,两处病变均被诊断为上皮样血管平滑肌脂肪瘤——PEComa的一种最常见肿瘤类型。胰头病变接受了胰腺区段性切除术,患者术后未接受任何辅助性治疗。随访25个月后未发生肿瘤复发或转移。总之,多灶性PEComa虽然在临床上容易被诊断为转移性肿瘤,但生物学行为良好。手术切除是主要的治疗方法。散发性多灶性PEComa的发生可能与肿瘤细胞的良性血行播散有关。诊断中需注意多灶性病变与转移性病变的鉴别,治疗中不应对这类疾病进行激进治疗。在疾病的长期监测中,有少数病例可在多年后出现≥2次PEComa病变。

关键词: 血管周上皮样细胞肿瘤, 多灶性, 胰腺, 肝脏, 诊断, 治疗

关剑, 沈贵华, 黄文亭. 胰腺与肝脏多灶性血管周上皮样细胞肿瘤1例及文献复习[J]. 肝癌电子杂志, 2025, 12(1): 24-29.

Guan Jian, Shen Guihua, Huang Wenting. Multifocal perivascular epithelioid cell tumor of pancreas and liver: a case report and literature review[J]. Electronic Journal of Liver Tumor, 2025, 12(1): 24-29.

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