Division of Special Treatment II, Eastern Hepatobiliary Surgery Hospital, The Second Military Medical University, Shanghai 200438, China.
Med Oncol. 2013 Mar;30(1):416. doi: 10.1007/s12032-012-0416-4. Epub 2013 Jan 6.
The aim of the study was to review the main clinical, imaging and pathological features of hepatic angiomyolipoma (HAML). We retrospectively analyzed the imaging, pathological and clinical features of 178 patients who underwent surgical resection for HAML. Forty-three males and 135 females with a median age of 43.5 years (range: 17-76 years) were enrolled in the study. Routine blood tests, including those for α-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen determinant, were normal. Liver function was classified as Child-Pugh A for all patients. Twenty-one patients were positive for hepatitis B surface antigen (HBsAg), while two had liver cirrhosis. Seventy-five lesions were located in the right hepatic lobe, 42 in the left hepatic lobe, 12 in the hepatic caudal lobe, and 49 in both left and right lobe. All patients underwent examination by ultrasound sonography (US), and additional examinations by computed tomography (CT) and magnetic resonance (MR) were carried out for 115 and 81 patients, respectively. Various diseases including HAML, liver cancer, liver hemangioma, hepatic lipoma, hepatic hamartoma, focal nodular hyperplasia and liver adenoma were diagnosed before operation. CT and MRI are more conclusive than US (US vs CT: χ(2) = 17.72, P = 0.00; US vs MRI: χ(2) = 12.14, P = 0.00), but for the sensitivity of these three imaging modalities, MRI performs the best (US vs MRI: χ(2) = 5.938, P = 0.015; CT vs MRI: χ(2) = 2.544, P = 0.111). All the patients underwent liver resection (96 right hepatic lobectomy, 41 left hepatic lobectomy and nine caudal lobe hepatic lobectomy). Several sampled tissues were positive for HBsAg (21/168, 12.5 %), β-catenin (4/41, 9.8 %), CD-34 (119/165, 72.1 %), HMB-45 (162/169, 92.9 %), SMA (145/158, 91.8 %) and vimentin (20/27, 74.1 %). The diagnosis of HAML remains difficult and mainly relies on pathological findings. For the imaging modalities, we recommend MRI examination because of its better definition and sensitivity. Although HMB-45 is considered the main histopathological biomarker for HAML, SMA may be also helpful in the diagnostic process (HMB-45 vs SMA: χ(2) = 2.37, P = 0.123). When the diagnosis of HAML is suspicious or hypothesized, surgical resection remains the recommended strategy due to the difficulty in diagnosis preoperatively and the potentially invasive growth of the lesion.
回顾肝血管平滑肌脂肪瘤(HAML)的主要临床、影像和病理学特征。我们回顾性分析了 178 例行 HAML 切除术患者的影像学、病理学和临床特征。研究纳入 43 名男性和 135 名女性,中位年龄 43.5 岁(范围:17-76 岁)。常规血液检查,包括甲胎蛋白、癌胚抗原和糖抗原检测,均正常。所有患者的肝功能均为 Child-Pugh A 级。21 名患者乙型肝炎表面抗原(HBsAg)阳性,2 名患者有肝硬化。75 个病灶位于右肝叶,42 个位于左肝叶,12 个位于肝尾叶,49 个位于左右两叶。所有患者均行超声检查(US),115 例行 CT 检查,81 例行 MRI 检查。术前诊断为 HAML、肝癌、肝血管瘤、肝脂肪瘤、肝错构瘤、局灶性结节性增生和肝腺瘤等各种疾病。CT 和 MRI 比 US 更具结论性(US 与 CT:χ(2) = 17.72,P = 0.00;US 与 MRI:χ(2) = 12.14,P = 0.00),但对于这三种影像学方法的敏感性,MRI 表现最好(US 与 MRI:χ(2) = 5.938,P = 0.015;CT 与 MRI:χ(2) = 2.544,P = 0.111)。所有患者均行肝切除术(96 例右半肝切除术,41 例左半肝切除术,9 例尾叶肝切除术)。部分取样组织 HBsAg 阳性(21/168,12.5%)、β-catenin 阳性(4/41,9.8%)、CD-34 阳性(119/165,72.1%)、HMB-45 阳性(162/169,92.9%)、SMA 阳性(145/158,91.8%)和波形蛋白阳性(20/27,74.1%)。HAML 的诊断仍然具有挑战性,主要依赖于病理学发现。对于影像学方法,我们建议行 MRI 检查,因为其具有更好的定义和敏感性。虽然 HMB-45 被认为是 HAML 的主要组织病理学生物标志物,但 SMA 也有助于诊断过程(HMB-45 与 SMA:χ(2) = 2.37,P = 0.123)。当怀疑或假设 HAML 诊断时,由于术前诊断困难和病变潜在的侵袭性生长,手术切除仍然是推荐的治疗策略。