Arblade S, Vilgrain V, Terris B, Zins M, Vullierme M P, Fléjou J F, Belghiti J, Menu Y
Service de Radiologie, Hôpital Beaujon, Clichy.
Gastroenterol Clin Biol. 1996;20(11):1022-6.
Hepatic angiomyolipoma is a rare, benign, mesenchymal tumor. We report 3 cases of atypical angiomyolipomas simulating benign hepatic tumors in 3 women free of tuberous sclerosis (Bourneville's disease). Hepatic angiomyolipomas were solitary in 2 cases and multiple in one. At imaging, the 6 lesions were large (7 cm in mean diameter), mainly hypoechoic and heterogeneous. At CT, lesions did not contain fat and were hypervascular. At MR imaging, lesions were hypointense in 4 cases and isointense in one case on T1-weighted sequences, hyperintense and isointense on T2-weighted sequences in four and in one case respectively. Percutaneous biopsy, performed in 2 cases, did not provide a correct diagnosis. Histopathologic examination of the resected specimen showed angiomyolipomas with very low or lacking fat in all 3 patients. All lesions were positive with the HMB-45 antibody. In conclusion, hepatic angiomyolipoma with a low fat component may mimic other hepatic tumors. Diagnosis may be improved by using HMB-45 reactivity on the biopsy specimen.
肝血管平滑肌脂肪瘤是一种罕见的良性间叶组织肿瘤。我们报告3例非典型血管平滑肌脂肪瘤,在3名无结节性硬化症(博恩维尔病)的女性中表现为良性肝肿瘤。肝血管平滑肌脂肪瘤2例为单发,1例为多发。影像学检查显示,6个病灶较大(平均直径7cm),主要为低回声且不均匀。CT检查时,病灶不含脂肪且血供丰富。磁共振成像检查时,4例病灶在T1加权序列上呈低信号,1例呈等信号;4例在T2加权序列上呈高信号,1例呈等信号。2例行经皮活检,均未得出正确诊断。所有3例患者切除标本的组织病理学检查均显示血管平滑肌脂肪瘤脂肪含量极低或无脂肪。所有病灶HMB - 45抗体均呈阳性。总之,脂肪成分少的肝血管平滑肌脂肪瘤可能会与其他肝肿瘤相混淆。对活检标本进行HMB - 45免疫反应检测可能有助于提高诊断准确性。