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肝血管肉瘤:44 例多机构、国际经验。

Hepatic Angiosarcoma: A Multi-institutional, International Experience with 44 Cases.

机构信息

Department of Surgery, Division of Hepatobiliary and Pancreatic Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

Departments of Surgery and Pathology, Mayo Clinic Florida, Jacksonville, FL, USA.

出版信息

Ann Surg Oncol. 2019 Feb;26(2):576-582. doi: 10.1245/s10434-018-7062-9. Epub 2018 Nov 19.

Abstract

BACKGROUND

Hepatic angiosarcoma is a rare primary liver tumor. The aim of this current study was to evaluate the presentation and treatment outcomes in a modern cohort.

METHODS

This was a retrospective, multi-institutional, observational study of patients with histopathologic diagnoses of primary hepatic angiosarcoma from four institutions. Clinicopathologic characteristics, treatments, and patient outcomes were examined.

RESULTS

Forty-four patients with hepatic angiosarcoma were identified. Patients were predominantly Caucasian and presented at a median age of 63.7 years; 81.4% of patients had bilobar disease and 37.2% had metastatic disease at the time of presentation. Only 10 patients underwent surgical resection. Median overall survival for the entire cohort was 5.8 months (interquartile range 1.9-16.4), and 1-, 3-, and 5-year actual survival was 30.0%, 8.1%, and 5.6%, respectively. There were only two 5-year survivors, both of whom presented with localized disease and underwent curative resection.

CONCLUSION

The prognosis for hepatic angiosarcoma remains quite poor. Surgical resection for localized disease results in the best outcomes. Unfortunately, current imaging modalities are often non- diagnostic, and most patients are unresectable at the time of presentation.

摘要

背景

肝血管肉瘤是一种罕见的原发性肝脏肿瘤。本研究旨在评估现代队列中肝血管肉瘤的表现和治疗结果。

方法

这是一项回顾性的、多机构的、观察性研究,研究对象是来自四个机构的组织病理学诊断为原发性肝血管肉瘤的患者。研究检查了临床病理特征、治疗和患者结局。

结果

共确定了 44 例肝血管肉瘤患者。患者主要为白种人,中位年龄为 63.7 岁;81.4%的患者存在双叶疾病,37.2%的患者在就诊时存在转移疾病。仅有 10 例患者接受了手术切除。全队列的中位总生存期为 5.8 个月(四分位距 1.9-16.4),1、3 和 5 年实际生存率分别为 30.0%、8.1%和 5.6%。只有两名 5 年幸存者,均为局限性疾病且接受了根治性切除。

结论

肝血管肉瘤的预后仍然很差。局限性疾病的手术切除可获得最佳结局。不幸的是,目前的成像方式通常无法确诊,而且大多数患者在就诊时无法切除。

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