肝血管周上皮样细胞瘤的诊断与外科治疗

Diagnosis and surgical treatment of hepatic perivascular epithelioid cell carcinoma

目的 总结肝血管周上皮样细胞瘤（PEComa）的临床特征,探讨该病的诊断与治疗方法.方法 回顾性分析2008年1月至2012年1月南京医科大学第一附属医院收治的16例肝PEComa患者的临床资料,总结该病的发病情况、临床特点、影像学特征、手术及病理检查情况.患者行部分肝切除术后定期复查腹部B超、肝功能和肿瘤标志物,必要时复查腹上区CT,了解肿瘤有无复发、转移.随访时间截至2012年12月.结果 16例患者中发病以中年女性患者居多,8例无自觉症状,其他患者表现为右季肋区不适、肝区隐痛或胀痛.肿瘤均为单发,位于肝右叶11例、肝左叶4例、肝尾状叶1例.术前B超检查无明显特异性征象,CT检查示肿瘤呈不均匀低密度表现.16例患者均行部分肝切除术,术后病理检查示均为R0切除,无手术并发症发生及患者死亡.免疫组织化学检测示16例患者上皮样肿瘤细胞中恶性黑色素瘤HMB-45、Melan-A和血管平滑肌肌动蛋白均呈阳性.16例患者均获随访,平均随访时间为27.9个月（9.0 ～46.0个月）,随访期内无肿瘤复发和死亡.结论 肝PEComa好发于中年女性患者,无特异性临床表现,术前CT检查有利于肝PEComa的鉴别诊断,行部分肝切除术治疗近期疗效良好.

Objective To investigate the clinical features of hepatic perivascular epithelioid cell carcinoma （PEComa） and the experiments in the surgical treatment of PEComa. Methods The clinical data of 16 patients with hepatic PEComa who received surgical treatment at the First Affiliated Hospital of Nanjing Medical University from January 2008 to January 2012 were retrospectively analyzed. The incidence, clinical manifestations, imagiong characteristics, surgical outcomes and pathological manifestations of this disease were analyzed. B sonography, hepatic function test, tumor markers test and epigastric computed tomography （CT） were applied to detect tumor recurrence and metastasis. The follow-up was ended in December 2012. Result Middle aged and female patients took large part of the patients. Of the 16 patients, 8 had no subjective symptoms, and other patients had discomfort in the right upper quadrant, pain or tenderness of the liver. All the tumors were solitary, and most of them located at the right liver （11 tumors were in the right liver, 4 in the left liver, 1 in the caudate lobe）. No specific features were detected by preoperative B sonography, while inhomogeneous low density in the tumor region was detected on by CT. All the patients received partial liver resection without morbidity and morality. The results of immunohistochemistry showed that the expressions of HMB-45, Melan-A and vascular smooth muscle actin were positive. The mean time of follow-up was 27.9 months （ range, 9.0-46.0 months ） , no tumor recurrence and death was observed during the follow-up. Conclusions Middle aged females are susceptive to hepatic PEComa, and patients have no specific clinical presentations. Preoperative CT examination is helpful for differential diagnosis of PEComa, and partieal hepatectomy can achieve satisfactory short-term clinical outcomes.