肝脏血管平滑肌脂肪瘤诊断与外科治疗：附19例报告

背景与目的 肝脏血管平滑肌脂肪瘤（HAML）是肝脏少见的一种间叶组织来源的良性肿瘤，影像学检查缺乏特异性，术前诊断率低，容易误诊为肝细胞癌或其他肝良性肿瘤，本文报告19例HAML患者的诊治过程，以期为临床提供参考和借鉴。方法 回顾性分析2011年1月—2019年12月收治的19例HAML患者临床资料，其中女12例，男7例；年龄28~61岁；体检发现无临床症状者14例，表现为上腹部隐痛者3例，间断腹胀者1例，腹痛伴腹泻者1例；1例合并慢性乙型病毒性肝炎及肺脓肿，1例合并乙型肝炎肝硬化代偿期。19例均不伴肾肺等其他脏器血管平滑肌脂肪瘤，均无结节性硬化症。肿瘤直径1.3~12 cm，平均直径（4.6±2.2）cm。AFP、CA19-9、CEA均正常。7例患者术前诊断肝细胞癌（36.8%），12例术前诊断肝良性肿瘤（63.2%），分别为7例诊断肝腺瘤，2例诊断炎性假瘤，1例诊断肝海绵状血管瘤，1例诊断肝局灶性结节性增生，1例术前行超声引导下肝脏穿刺活检病理证实HAML。结果 患者均行外科手术治疗，18例行肝切除治疗，1例行超声引导下肝肿瘤穿刺活检术、经皮穿刺肝肿瘤射频消融术。平均手术时间（172.7±80.4）min，术中平均出血量为（456.6±528.1）mL，平均术后住院时间（9.8±2.7）d。19例患者病理检查均证实HAML，其中7例为上皮样血管平滑肌脂肪瘤，免疫组化检查HMB-45、SMA均阳性表达。患者术后恢复良好，所有患者无肝衰竭、腹腔出血、胆汁漏等并发症发生，无死亡病例。全部病例均获随访，均未发现肿瘤复发及转移。术后患者有良好的生活质量。结论 HAML瘤属于良性肿瘤，术前诊断比较困难，一部分患者容易误诊为肝细胞癌，最终诊断依靠病理及免疫组织化学染色，外科手术切除是安全、有效的治疗选择，预后良好。

Diagnosis and surgical treatment of hepatic angiomyolipoma: a report of 19 cases

Background and Aims Hepatic angiomyolipoma (HAML) is a rare benign mesenchymal tumor of the liver, with no specific imaging features and low preoperative diagnosis rate. It is frequently misdiagnosed as hepatocellular carcinoma (HCC) or other hepatic benign tumors. This article reports the diagnosis and treatment process of 19 patients with HAML, so as to provide a reference and guidance for clinical practice.Methods The clinical data of 19 cases with HAML treated from January 2011 to December 2019 were retrospectively analyzed. Of the patients, 12 cases were females and 7 cases were males, with the age ranging from 28 to 61 years; lesion was found by health maintenance examination in 14 cases without clinical symptoms, 3 cases had abdominal pain, one case had intermittent abdominal distention, and one case had abdominal pain and diarrhea; one case was complicated with chronic hepatitis B and pulmonary abscess, one case was complicated with liver cirrhosis in compensatory period. All the 19 patients did not have angiomyolipoma of other organs such as kidney and lung, and none of them had tuberous sclerosis. The diameter of the tumors ranged from 1.3 to 12 cm, with an average diameter of (4.6±2.2) cm. The levels of AFP, CA19-9 and CEA were all within the normal range. Seven patients (36.8%) were diagnosed as HCC before operation, and 12 patients (63.2%) were considered as hepatic benign tumors that included 7 cases were diagnosed as hepatic adenoma, 2 cases were diagnosed as inflammatory pseudotumor, one case was diagnosed as hepatic cavernous hemangioma, one case was diagnosed as hepatic focal nodular hyperplasia, and one case was identified as HAML by preoperative ultrasound guided liver biopsy pathology.Results All patients underwent surgical treatment, including hepatic resection in 18 patients and ultrasound guided liver tumor biopsy and percutaneous puncture radiofrequency ablation of the liver tumor in one patient. The average operative time was (172.7±80.4) min, intraoperative blood loss was (456.6±528.1) mL, and length of hospital stay was (9.8±2.7) d. HAML was confirmed in all the 19 patients by pathological examination, in which seven cases were epithelioid angiomyolipoma. HMB-45 and SMA were positive in immunohistochemical staining. All patients recovered well after operation, and no complications such as liver failure, abdominal bleeding and bile leakage as well as death occurred. All patients were followed up, and no tumor recurrence and metastasis were found. All patients had good quality of life.Conclusions HAML belongs to benign tumor, and is difficult to diagnose before operation. Some patients were easily misdiagnosed as HCC. The final diagnosis depends on pathological and immunohistochemical analysis. Surgical resection is a safe and effective treatment option with good prognosis.