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肝血管平滑肌脂肪瘤:诊断和治疗的临床经验。

Hepatic angiomyolipoma: a clinical experience in diagnosis and treatment.

机构信息

Hospital & Institute of Hepatobiliary Surgery, Chinese PLA General Hospital, 28 Fu-Xing Road, Haidian District, Beijing, China.

出版信息

Dig Dis Sci. 2010 Nov;55(11):3235-40. doi: 10.1007/s10620-010-1144-2. Epub 2010 Feb 18.

Abstract

BACKGROUND

Hepatic angiomyolipoma is a rare mesenchyme-derived neoplasm often misdiagnosed as hepatocellular carcinoma, and the treatment for it remains controversial.

AIMS

To develop the optimal preoperative diagnoses means and treatment modalities of hepatic angiomyolipoma.

METHODS

Retrospective analysis of the clinical features, treatment, and prognostic data of 17 hepatic AML patients admitted to Chinese People's Liberation Army Generation Hospital between 1996 and 2006.

RESULTS

Most hepatic angiomyolipoma were solitary. The overall preoperative diagnostic rate was 18% and the most common misdiagnosis was hepatocellular carcinoma (10/17, 59%). In three patients, observation was performed for 2-3 years before being admitted. The tumors increased 1-9 cm in size in all patients. All of the 17 patients finally received various liver resection procedures, and postoperative mortality and morbidity was 0 and 12% (2/17). After a median follow-up period of 73 months, the tumor recurred only in one patient 9 years post-operatively.

CONCLUSIONS

Hepatic angiomyolipoma should be suspected in liver tumor patients with normal α-fetoprotein levels and no concomitant hepatitis. Preoperative MRI combined with percutaneous fine-needle biopsy should be the diagnostic methods of choice. Small hepatic AML proved through pathologic examination (<5 cm) may be managed by observation with close follow-up, but surgery is indicated in patients suffering from large tumors or significantly larger tumors during follow-up.

摘要

背景

肝血管平滑肌脂肪瘤是一种罕见的间叶组织来源的肿瘤,常被误诊为肝细胞癌,其治疗仍存在争议。

目的

探讨肝血管平滑肌脂肪瘤的最佳术前诊断方法和治疗方式。

方法

回顾性分析 1996 年至 2006 年解放军总医院收治的 17 例肝血管平滑肌脂肪瘤患者的临床特征、治疗及预后资料。

结果

大多数肝血管平滑肌脂肪瘤为单发。术前总体诊断率为 18%,最常见的误诊为肝细胞癌(10/17,59%)。有 3 例患者观察 2-3 年才入院。所有患者肿瘤均增大 1-9cm。17 例患者最终均接受了各种肝切除术,术后死亡率和并发症发生率均为 0 和 12%(2/17)。中位随访 73 个月后,仅 1 例患者术后 9 年肿瘤复发。

结论

对于 AFP 水平正常且无合并肝炎的肝肿瘤患者,应怀疑肝血管平滑肌脂肪瘤。术前 MRI 结合经皮细针活检应为首选诊断方法。通过病理检查证实为小肝血管平滑肌脂肪瘤(<5cm)者可密切随访观察,但对于肿瘤较大或随访中肿瘤明显增大的患者,手术是必要的。

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