血管周围上皮样细胞瘤(PEComa),一种不常见的间充质肿瘤,来自特殊的血管周围上皮样细胞,表现出明显的平滑肌和黑素细胞分化特征,具有不可预测的行为。PEComa往往更常见于子宫和肾脏;它在肝脏中的发生极为罕见。我们介绍了一例29岁的肝PEComa女性,并用MRI评估了肿瘤,整合的18F-氟代脱氧葡萄糖(FDG),和68Ga-成纤维细胞活化蛋白抑制剂(FAPI)PET/CT扫描。该患者有口服避孕药间歇使用数年的病史。来自外部机构的体格检查中的腹部超声显示肝脏中有肿块。对比增强的腹部MRI显示弥散加权成像(DWI)上的弥散受限,肝脏病变的对比增强和冲洗模式迅速,提示肝腺瘤(HA)或肝细胞癌(HCC)。使用18F-FDG和68Ga-FAPIPET/CT扫描进行进一步评估。肝损害是非FDG狂热,而在68Ga-FAPIPET/CT上观察到示踪剂摄取增加。随后,行腹腔镜肝V段部分切除术。免疫组织化学分析显示HMB45,Melan-A,和SMA虽然显示AFP阴性结果,磷脂酰肌醇蛋白聚糖-3,肝细胞,和精氨酸酶-1。结果指示基于这些发现的肝PEComa诊断。我们还回顾了目前关于临床特征的文献,病理特征,以及肝PEComa诊断中的挑战。
Perivascular epithelioid cell tumor (PEComa), an uncommon mesenchymal neoplasm, arises from specialized perivascular epithelioid cells exhibiting distinct features of smooth muscle and melanocytic differentiation with unpredictable behavior. PEComa tends to occur more commonly in the uterus and kidneys; its occurrence in the liver is exceedingly rare. We presented a
case of a 29-year-old woman with hepatic PEComa and evaluated the tumor with MRI, integrated 18F-fluorodeoxyglucose (FDG), and 68Ga-fibroblast activation protein inhibitor (FAPI) PET/CT scans at presentation. The patient had a history of intermittent utilization of oral contraceptive drugs for several years. An abdominal ultrasound in a physical examination from an outside institution revealed a mass in the liver. A contrast-enhanced abdominal MRI revealed restricted diffusion on diffusion-weighted imaging (DWI) and rapid contrast enhancement and washout patterns in the hepatic lesion, suggesting hepatic adenoma (HA) or hepatocellular carcinoma (HCC). Further assessment was carried out using 18F-FDG and 68Ga-FAPI PET/CT scans. The hepatic lesion was non-FDG avid, whereas increased tracer uptake was observed on the 68Ga-FAPI PET/CT. Subsequently, laparoscopic partial resection of liver segment V was performed. Immunohistochemical analyses demonstrated positive staining for HMB45, Melan-A, and SMA while showing negative results for AFP, glypican-3, hepatocyte, and arginase-1. The results were indicative of a hepatic PEComa diagnosis based on these findings. We also review the current literature on the clinical characteristics, pathological features, and challenges in the diagnosis of hepatic PEComa.