探讨肝血管平滑肌脂肪瘤（HAML）诊治及疗效。

回顾性分析2011年1月至2020年1月在解放军总医院第五医学中心行手术治疗且经病理学检查明确诊断的35例HAML患者临床资料。患者均签署知情同意书，符合医学伦理学规定。其中男13例，女22例；年龄26~63岁，中位年龄48岁。病灶单发者32例，多发3例；最大直径1.1~15.3 cm，中位直径5.1 cm；位于左肝17例，位于右肝14例，同时位于左、右肝4例。分析35例患者诊治及疗效。

术前明确诊断HAML 5例，仅占14%；诊断为原发性肝癌13例，肝腺瘤4例，炎性假瘤1例，未明确诊断12例。33例患者行手术切除，切缘距病变1 cm以上或多发结节中较为孤立且直径较小者，同期行射频消融治疗。33例患者术后均经病理检查明确诊断HAML，免疫组化法染色HMB45阳性，其中混合型22例，血管型5例，肌样型4例，脂瘤型2例。另外2例术前行穿刺病理检查明确诊断后行超声引导下射频消融治疗。患者均恢复顺利。所有患者预后良好，1例肌样型术后半年复发，行介入栓塞治疗控制良好，随访2年无明显进展；其余患者随访2年以上，均无复发及远处转移。

HAML为较少见的肝脏良性病变，但临床可表现出一定的恶性倾向，其影像学检查通常缺乏特异性，容易误诊，对于临床诊断困难者，病理检查可作为确诊依据。手术治疗预后良好，条件允许者可作为首选。

To investigate the diagnosis, treatment and efficacy of hepatic angiomyolipoma (HAML).

Clinical data of 35 patients pathologically diagnosed with HAML undergoing surgical resection in the Fifth Medical Center of Chinese PLA General Hospital from January 2011 to January 2020 were retrospectively analyzed. The informed consents of all patients were obtained and the local ethical committee approval was received. Among them, 13 patients were male and 22 female, aged from 26 to 63 years, with a median age of 48 years. 32 patients were diagnosed with single lesion and 3 cases with multiple lesions. The maximum diameter was ranged from 1.1 to 15.3 cm, and the median diameter was 5.1 cm. The lesions were located in the left lobe of 17 cases, in the right lobe of 14 cases, and in bilateral lobes of 4 cases, respectively. The diagnosis, treatment and clinical efficacy of 35 patients were analyzed.

Preoperatively, 5 cases were diagnosed with HAML, accounting for only 14%. 13 patients were diagnosed with primary liver cancer, 4 cases of hepatic adenoma, 1 case of inflammatory pseudotumor, and 12 cases remained undiagnosed. 33 patients underwent surgical resection. Radiofrequency ablation was performed contemporarily in patients with surgical margin of >1 cm or multiple isolated nodules in small diameter. Postoperatively, all 33 patients were pathologically diagnosed with HAML. Positive HMB45 was detected by immunohistochemical staining, including 22 cases of mixed type, 5 cases of vascular type, 4 cases of myomatous type and 2 cases of lipomatous type. The other 2 patients were pathologically diagnosed by preoperative biopsy and subsequently received ultrasound-guided radiofrequency ablation. All patients recovered well. All patients obtained favorable prognosis. 1 case of myomatous type HAML recurred half a year after surgery, which was properly controlled by interventional embolization, and no significant progression was reported during 2-year follow-up. No recurrence or distant metastasis occurred in the remaining patients during postoperative followed-up for more than 2 years.

HAML is a rare benign liver lesion. However, it represents clinical manifestations of malignant tendency. Imaging examination lacks diagnostic specificity for HAML, which is likely to be misdiagnosed. For the undiagnosed cases, pathological examination can be taken as the diagnostic criteria. Surgical resection yields favorable clinical prognosis, which is highly recommended for eligible patients.