Ren Ning, Qin Lun-Xiu, Tang Zhao-You, Wu Zhi-Quan, Fan Jia
Liver Cancer Institute, Fudan University, Shanghai 200032, China.
World J Gastroenterol. 2003 Aug;9(8):1856-8. doi: 10.3748/wjg.v9.i8.1856.
To summarize the experience of the diagnosis and treatment of hepatic angiomyolipoma (HAML).
The clinical, imaging and pathological features, and treatment strategies of 26 patients with HAML treated at the authors' institute between October 1998 and January 2003 were retrospectively analyzed. All the patients received liver resection and were followed up till the study. Immunohistochemical assays were performed with a panel of antibodies.
There was an obvious female predominance (21:5), and most of the patients (18/26) had no symptoms. Heterogeneous high echo was found in ultrasonography and punctiform or filiform vascular distribution pattern was found in color Doppler-sonography in most of the lesions (21/26). All of the 5 lesions further enhanced with Levovist showed early and prolonged enhancement. At contrast-enhanced spiral CT, the soft-tissue components of 24 lesions were markedly enhanced in the arterial phase and 18 lesions remained enhanced in the portal venous phase. MRI was performed in 9 patients, and showed hypointensity or hyperintensity on T1-weighted images and heterogeneous hyperintensity on T2-weighted images. Histopathologically, all lesions were composed of adipose tissues, smooth muscle and blood vessels with different proportions. Most lesions showed positive immunohistochemical staining for HMB45 (26/26), A103 (24/26) and SMA (24/26). All of the 26 patients showed a benign course with no sign of recurrence.
Preoperative radiological diagnosis of HAML is possible. The demonstration of intratumoral fat and central vessels is helpful in the diagnosis. HMB45, A103 and SMA are promising markers for pathologic diagnosis of HAML, and surgical resection is effective for the treatment of HAML.
总结肝脏血管平滑肌脂肪瘤(HAML)的诊断与治疗经验。
回顾性分析1998年10月至2003年1月间在作者所在机构接受治疗的26例HAML患者的临床、影像及病理特征,以及治疗策略。所有患者均接受肝切除术并随访至本研究。采用一组抗体进行免疫组化检测。
女性明显居多(21:5),大多数患者(18/26)无症状。超声检查发现不均质高回声,多数病灶(21/26)彩色多普勒超声显示点状或丝状血管分布模式。5个经声诺维进一步增强的病灶均表现为早期及持续增强。在螺旋CT增强扫描中,24个病灶的软组织成分在动脉期明显强化,18个病灶在门静脉期仍强化。9例患者行MRI检查,T1加权像呈低信号或高信号,T2加权像呈不均质高信号。组织病理学上,所有病灶均由脂肪组织、平滑肌和血管按不同比例组成。多数病灶HMB45(26/26)、A103(24/26)和SMA(24/26)免疫组化染色呈阳性。26例患者均呈良性病程,无复发迹象。
HAML术前放射学诊断是可行的。肿瘤内脂肪及中央血管的显示有助于诊断。HMB45、A103和SMA是HAML病理诊断有前景的标志物,手术切除是治疗HAML的有效方法。