目的

探讨肝脏乏脂肪型血管周上皮样细胞肿瘤（PEComa）的影像学特征。

方法

回顾性分析2016年1月至2024年12月襄阳市中心医院肝胆外科经手术病理确诊的12例肝脏乏脂肪型PEComa患者的临床、病理及影像学资料，并复习相关文献。

结果

12例肝脏乏脂肪型PEComa患者均为女性，年龄（48.58±10.96）岁，肿瘤均为单发，其中4例位于肝左叶，8例位于肝右叶。临床表现为腹胀不适（2例）、腹痛（1例）及背痛（1例），8例无症状。影像学上，所有病例在动态增强扫描动脉期均呈现出显著强化，其中10例在门静脉期及延迟期强化程度逐渐减弱，符合典型“快进快出”或“快进慢出”强化模式；另外2例表现为在门静脉期及延迟期持续强化，即“持续性”强化模式。9例PEComa患者在增强扫描动脉期病灶内或周边可见增粗的血管影。病理上，肿瘤组织内缺乏成熟脂肪细胞，主要由大量上皮样细胞构成，并沿血管腔周围呈放射状排列；免疫组织化学染色显示HMB-45、Melan-A及平滑肌肌动蛋白均呈阳性表达。

结论

肝脏乏脂肪型PEComa在影像学及病理学上展现出独特的特征性表现，这些特征对于该病的早期诊断、鉴别诊断具有重要的临床价值。

Objective

To investigate the imaging characteristics of hepatic lipid-poor perivascular epithelioid cell tumor （PEComa）.

Methods

A retrospective analysis was conducted on the clinical， pathological and imaging data of 12 patients with hepatic lipid-poor PEComa diagnosed by surgical pathology in the Department of Hepatobiliary Surgery at Xiangyang Central Hospital from January 2016 to December 2024，and the relevant literatures were reviewed.

Results

All 12 patients with hepatic lipid-poor PEComa were female， with a mean age of （48.58±10.96） years old. The tumors were all solitary， among which 4 cases were located in the left lobe of the liver， and 8 cases were located in the right lobe. The clinical manifestations included abdominal distension and discomfort （2 cases）， abdominal pain （1 case） and back pain （1 case）， and while 8 cases were asymptomatic. In terms of imaging， all cases showed significant enhancement during the arterial phase of enhanced scan. Among them， the enhancement degrees of 10 cases gradually diminished in the portal vein phase and delayed phase， which was in line with the typical "fast-in and fast-out" or "fast-in and slow-out" enhancement patterns. The remaining 2 cases showed persistent enhancement during the portal vein stage and the delayed stage， and were classified as the "persistent"enhancement pattern. In the arterial phase of enhanced scanning， thickened vascular shadows could be seen in or around the lesions in 9 patients with PEComa. Pathologically， the tumor tissue lacked mature adipocytes and was mainly composed of a large number of epithelioid cells， which were radially arranged around the vascular lumen. Immunohistochemical staining showed positive expressions of HMB-45， Melan-A and smooth muscle actin.

Conclusion

Hepatic lipid-poor PEComas exhibit unique imaging and pathological characteristics， which are of significant clinical value for the early diagnosis and differential diagnosis of this disease.