Kou Yan-Qi, Yang Yu-Ping, Ye Wei-Xiang, Yuan Wei-Nan, Du Shen-Shen, Nie Biao
Department of Gastroenterology, The First Affiliated Hospital of Jinan University, Guangzhou 510630, Guangdong Province, China.
Department of Gastrointestinal Endoscopy, The First Affiliated Hospital of Jinan University, Guangzhou 510630, Guangdong Province, China.
World J Clin Cases. 2023 Jan 16;11(2):426-433. doi: 10.12998/wjcc.v11.i2.426.
Hepatic perivascular epithelioid cell neoplasms (PEComas) are rare. Diagnostic and treatment experience with hepatic PEComa remains insufficient.
Three hepatic PEComa cases are reported in this paper: One case of primary malignant hepatic PEComa, one case of benign hepatic PEComa, and one case of hepatic PEComa with an ovarian mature cystic teratoma. During preoperative imaging and pathological assessment of intraoperative frozen samples, patients were diagnosed with hepatocellular carcinoma (HCC), while postoperative pathology and immunohistochemistry subsequently revealed hepatic PEComa. Patients with hepatic PEComa which is misdiagnosed as HCC often require a wider surgical resection. It is easy to mistake them for distant metastases of hepatic PEComa and misdiagnosed as HCC, especially when it's combined with tumors in other organs. Three patients eventually underwent partial hepatectomy. After 1-4 years of follow-up, none of the patients experienced recurrence or metastases.
A clear preoperative diagnosis of hepatic PEComa can reduce the scope of resection and prevent unnecessary injuries during surgery.
肝血管周上皮样细胞肿瘤（PEComas）较为罕见。肝PEComa的诊断和治疗经验仍不足。
本文报告3例肝PEComa病例：1例原发性恶性肝PEComa、1例良性肝PEComa以及1例合并卵巢成熟囊性畸胎瘤的肝PEComa。在术前影像学检查及术中冰冻样本的病理评估中，患者被诊断为肝细胞癌（HCC），而术后病理及免疫组化随后显示为肝PEComa。被误诊为HCC的肝PEComa患者通常需要更广泛的手术切除。容易将它们误认为肝PEComa的远处转移并误诊为HCC，尤其是当它与其他器官的肿瘤合并时。3例患者最终均接受了肝部分切除术。经过1至4年的随访，所有患者均未出现复发或转移。
术前明确诊断肝PEComa可缩小切除范围并避免手术中不必要的损伤。
