目的探讨肝脏血管平滑肌脂肪瘤(HAML)的临床病理学特点、诊断及鉴别诊断。方法对15例肝脏血管平滑肌脂肪瘤的临床及病理学资料进行回顾性临床病理分析。结果 15例HAML中女性13例,男性2例,男女比例1∶6.5,年龄19~59岁(平均42.2岁,中位年龄45岁)。6例患者有间断性腹部胀痛、恶心、反酸等不同的临床症状;1例合并慢性乙型病毒性肝炎,9例无明显症状。影像学检查均表现为肝脏内孤立性占位,其中6例考虑为血管瘤,6例为错构瘤,3例为肝癌。15例均行肿块切除术,12例行术中冰冻切片诊断,与石蜡切片诊断符合率97.5%。肿瘤位于肝右叶11例(其中1例合并肾AML并肝脏复发,2例合并肝海绵状血管瘤,1例合并胃低分化腺癌),左叶2例,肝左右叶多发2例(其中1例合并肾AML,1例首发于右叶,2年后左叶发生并合并肝海绵状血管瘤)。肿瘤组织内可见不同比例的平滑肌、脂肪组织和血管成分,并可见多灶性髓外造血,瘤周肝组织无肝硬化改变。15例中经典型1例,血管瘤样型1例,脂肪瘤样型3例,平滑肌瘤样型10例(上皮样细胞型9例、梭形细胞型1例),其中上皮样平滑肌细胞可表现为多种形态:透明细胞型4例,嗜酸细胞型4例,多形细胞型1例。均有核异型性(15/15):中度13例,重度2例;细胞密度:低密度3例,中密度7例,高密度5例。恶性HAML 3例,其肿瘤组织与周围肝组织部分分界不清,呈浸润性生长。免疫组化标记HMB45、Melan-A和SMA阳性,S-100阴性。结论肝脏血管平滑肌脂肪瘤诊断困难,确诊主要依靠病理组织学改变及免疫组化染色;患者预后大多良好,术后需长期随访。 Objective To investigate the clinicopathological features, diagnosis and differential diagnosis of hepatic angiomyolipoma (HAML). Methods The clinical and pathological data of 15 cases of hepatic angiomyolipoma were retrospectively analyzed clinically and pathologically. Results Of the 15 HAML patients, 13 were women and 2 were males. The male-to-female ratio was 1: 6.5, ranging in age from 19 to 59 years (mean 42.2 years, median 45 years). 6 patients had intermittent abdominal pain, nausea, acid reflux and other clinical symptoms; 1 case of chronic hepatitis B, 9 cases without obvious symptoms. Imaging examination showed lone occupancy in the liver, of which 6 were considered as hemangiomas, 6 as hamartoma and 3 as liver cancer. Fifteen patients underwent radical resection, and 12 patients underwent intraoperative frozen section diagnosis, which was consistent with paraffin section diagnosis of 97.5%. The tumors were located in the right lobe of the liver in 11 cases (1 with renal AML and liver recurrence, 2 with hepatomegaly and 1 with gastric poorly differentiated adenocarcinoma), 2 in the left lobe and 2 in the left and right lobes 1 with renal AML, 1 with first onset in the right lobe, 2 years after the left lobe and with hepatic cavernous hemangiomas. Tumor tissue showed different proportions of smooth muscle, adipose tissue and vascular components, and multifocal extramedullary hematopoiesis, peritumor liver cirrhosis without change. Among the 15 cases, 1 was classic, 1 was a hemangioma, 3 was a lipoma, 10 was a leiomyoma (9 epithelioid and 1 spindle), in which epithelial smooth muscle cells Can be manifested in a variety of forms: 4 cases of clear cell type, 4 cases of eosinophilic cell type in 1 cases. All had nuclear atypia (15/15): moderate in 13 cases, severe in 2 cases; cell density: low density in 3 cases, medium density in 7 cases, high density in 5 cases. Malignant HAML 3 cases, the tumor tissue and surrounding liver tissue part of the boundary is unclear, was invasive growth. Immunohistochemical markers HMB45, Melan-A and SMA positive, S-100 negative. Conclusions Hepatic angiomyolipoma is difficult to diagnose. The diagnosis depends mainly on histopathological changes and immunohistochemical staining. The prognosis of patients is mostly good and needs long-term follow-up after operation.