肝血管平滑肌脂肪瘤的诊断与治疗

Diagnosis and treatment of hepatic angiomyolipoma

  • 摘要: 目的:总结肝血管平滑肌脂肪瘤(HAML)的诊断与治疗。
    方法:回顾性分析1990年1月至2010年12月安徽医科大学第一附属医院收治的15例HAML患者的临床资料。15例患者进行血清AFP、CEA、CA19-9等常见消化系统肿瘤标志物指标检查,根据临床特征、影像学检查(超声、CT、MRI及血管造影检查)结果选择手术方式。术后进行病理学检查。采用门诊及电话随访,随访时间截至2011年6月。
    结果:15例患者中5例患者无临床症状,所有患者无肝炎病史。术前超声、CT、MRI及血管造影检查诊断准确率分别为3/15、3/13、2/6和1/3。肿瘤位于肝左叶者4例、肝右叶者11例,肿瘤均为单发。15例患者均行手术治疗,其中13例行肝肿瘤剜除及周围部分正常肝组织切除术,2例因肿瘤直径较大行左半肝切除术。术后免疫组织化学染色检查结果示HMB45、CD34和Ki-67表达均为阳性。15例患者术后痊愈出院,13例患者获得随访,随访时间6个月至9.8年,中位随访时间3.2年。11例获得完整随访,另2例患者术后分别随访9.8年和6年后失访。所有患者随访期间均生存,无肿瘤复发及转移。
    结论:HAML无特异性临床症状,影像学检查确诊率不高,病理标本免疫组织化学染色检查HMB45阳性是特征性诊断依据,手术切除是目前治疗HAML的首选方法,患者预后较好。

     

    Abstract: Objective:To investigate the diagnosis and treatment of hepatic angiomyolipoma (HAML).
    Methods:The clinical data of 15 patients with HAML who were admitted to the First Affiliated Hospital of Anhui Medical University from January 1990 to December 2010 were retrospectively analyzed. Tumor biomarkers such as serum alphafetoprotein, carcinoembryonic antigen, CA19-9 were detected, and surgical procedures were selected according to the clinical features and results of imaging diagnosis. Pathological examination was done postoperatively. Patients were followed up via out patient examination or phone call till June 2011.
    Results:Five patients did not have clinical symptoms and all the patients did not have the history of hepatitis. The diagnostic rates of preoperative ultrasonography, computed tomography, magnetic resonance imaging and angiography were 3/15, 3/13,2/6 and 1/3. The lesions located in the left lobe in 4 patients, and lesions in the right lobe in 11 patients. All the tumors were solitary. All the patients received surgical treatment, including 13 received tumor enucleation and resection of adjacent normal hepatic tissues, 2 received left semihepatectomy because of the big size of the tumor. The results of postoperative histochemical staining showed that the expressions of HMB45, CD34 and Ki-67 were positive. All the patients were cured after operation, and 13 patients were followed up for 6 months to 9.8 years,with the median follow up time of 3.2 years. Eleven patients had complete follow up data, and 2 patients were lost to follow up at postoperative year 9.8 and 6, respectively. All patients had disease free survival, with no tumor recurrence or metastasis.
    Conclusions:HAML has no specific symptoms. Preoperative imaging diagnosis of HAML is difficult. Positive expression of HMB45 is a definitive proof for histopathological diagnosis of HAML. Surgical resection is the therapy of choice for HAML. The prognosis of patients is good.

     

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