肝脏恶性上皮样血管周细胞瘤的诊断与外科治疗

目的 总结肝脏恶性上皮样血管周细胞瘤（perivascular epithelioid cell carcinoma，PEComa）的临床、影像学和病理学特征，探讨PEComa的诊断与外科治疗方法。方法 回顾性分析2008年1月1日至2018年1月1日安康市中心医院收治的6例肝脏恶性PEComa患者临床资料，均在腹部B超、上腹部增强CT检查之后行肝脏肿瘤切除术，术后经病理及免疫组化检查明确诊断并密切随访。结果 6例患者中女5例，男1例，多因上腹部疼痛就诊，且血清学检查基本正常。影像学主要表现为肝脏单发性肿瘤，形态欠规则、边界尚清晰；CT平扫期表现为低密度影或稍低密度影；增强扫描动脉期病灶轻度强化，门静脉期病灶强化明显，延迟期病灶强化程度减轻。肿瘤位于左肝4例、右肝2例，大小为（7.1 cm×6.3 cm×5.7 cm）～（21.0 cm×18.0 cm×11.9 cm）。6例均行肝部分切除术，R0切除，无手术相关并发症发生及围手术期死亡。术后病理检查示，病灶由不同比例的血管、上皮样细胞及巨核细胞组成。免疫组化显示SMA、CD34、HMB45、Vimentin表达阳性。本组6例全部获得随访，平均随访时间20.8个月（6～52个月），存活2例，死亡4例，死亡原因为肿瘤复发、转移及全身多脏器功能衰竭。结论 肝脏恶性PEComa是一种非常罕见的肝脏肿瘤，好发于中年女性，无特异性临床表现，术前增强CT检查有利于肝脏恶性PEComa的诊断和鉴别诊断，行根治性肝脏肿瘤切除术是最有效的治疗方法，一旦诊断应尽早手术，术后密切随访。

Diagnosis and surgical treatment of liver malignant perivascular epithelioid cell carcinoma

Objective To summarize the clinical, imaging and pathological features of perivascular epithelioid cell carcinoma (PEComa) and to investigate the diagnosis and surgical treatment of PEComa. Methods The clinical data of 6 patients with hepatic malignant PEComa who received surgical treatment at Ankang Central Hospital from Jan.1, 2008 to Jan.1, 2018 were retrospectively analyzed. All patients were examined by color Doppler ultrasonography, enhanced computed tomography (CT) of upper abdomen and then treated by resection of liver tumor. The diagnosis was confirmed by postoperative pathological and immunohistochemical examination and patients were followed-up closely after surgery. Results Among the 6 patients, 5 were females and 1 was male, most of whom came to hospital due to epigastric pain and their serological examination results were basically normal. The main imaging findings were solitary liver tumor with irregular shape and clear boundary. CT scan showed low density or slightly low density. In contrast enhanced CT scan, the lesions in arterial phase were slightly enhanced, the lesions in portal venous phase were significantly enhanced, and the degree of enhancement in delayed phase was reduced. The tumor was found in 4 cases of left liver and 2 cases of right liver, with the size ranging from 7.1 cm×6.3 cm×5.7 cm to 21.0 cm×18.0 cm×11.9 cm. All the 6 cases underwent partial hepatectomy and R0 resection. No operative complications or perioperative death occurred. Postoperative pathological examination showed that the lesions were composed of different proportions of blood vessels, epithelioid cells and megakaryocytes. Result of immunohistochemistry showed that SMA, CD34, HMB45 and vimentin were positive. The mean time of follow-up was 20.8 months (ranging from 6 to 52 months), during which 2 cases survived and 4 cases died. The causes of death were tumor recurrence, metastasis and multiple organ failure. Conclusion Hepatic malignant PEComa is a rare neoplasm of the liver, which is susceptive to middle-aged women. There is no specific clinical manifestation of this disease. Preoperative enhanced CT is helpful for diagnosis and differential diagnosis of liver malignant PEComa. Radical resection of liver tumor is the most effective treatment. Once the diagnosis is made, it is advisable to operate as soon as possible and follow up closely after operation.