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[肝脏恶性上皮样血管内皮瘤。手术治疗策略的结果]

[Malignant epithelioid hemangioendothelioma of the liver. Results of surgical treatment strategies].

作者信息

Langrehr J M, Petersen I, Pfitzmann R, Lopez-Hänninen E

机构信息

Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Charité -- Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin.

出版信息

Chirurg. 2005 Dec;76(12):1161-7. doi: 10.1007/s00104-005-1070-6.

Abstract

BACKGROUND

Malignant epithelioid hemangioendothelioma is a rare vascular tumor described mostly in soft tissue, lungs, or liver. The outcome after a wide variety of therapeutic measures, ranging from extended surgical therapy to no therapy, is reported to be variable. Therefore, we reviewed our experience with resective therapy for this rare liver tumor, including orthotopic liver transplantation.

MATERIAL AND METHODS

During a period of 5 years, seven patients with the histological diagnosis of hepatic epithelia hemangioendothelioma were seen. In five of them, the liver pathology was detected at random, and all patients were prospectively followed. The therapeutic measures and course of disease are given as case reports.

RESULTS

Three patients received formal liver resection and two received liver grafts, one partial and one whole. One further patient is scheduled for transplantation and one is undergoing alternative therapy, with the tumor remaining stable. All resected patients recovered quickly and are alive and well 2 months to 4 years later without signs of tumor recidivism.

CONCLUSION

Anatomic liver resection, or in the case of diffuse tumor spread, orthotopic liver transplantation, show favorable long-term results for the treatment of hepatic epithelioid hemangioendothelioma. Therefore, surgical therapy is proposed as the treatment of choice for this entity.

摘要

背景

恶性上皮样血管内皮瘤是一种罕见的血管肿瘤,主要见于软组织、肺或肝脏。据报道,从扩大手术治疗到不治疗等多种治疗措施后的结果各不相同。因此,我们回顾了我们对这种罕见肝脏肿瘤的切除治疗经验,包括原位肝移植。

材料与方法

在5年期间,共诊治了7例经组织学诊断为肝上皮样血管内皮瘤的患者。其中5例肝脏病理为偶然发现,所有患者均进行了前瞻性随访。治疗措施和病程以病例报告形式给出。

结果

3例患者接受了正规肝切除术,2例接受了肝移植,1例为部分肝移植,1例为全肝移植。另有1例患者计划进行移植,1例正在接受替代治疗,肿瘤保持稳定。所有接受切除手术的患者恢复迅速,在2个月至4年后均存活良好,无肿瘤复发迹象。

结论

解剖性肝切除术,或在肿瘤弥漫性扩散的情况下进行原位肝移植,对肝上皮样血管内皮瘤的治疗显示出良好的长期效果。因此,建议手术治疗作为该疾病的首选治疗方法。

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