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原发性肝血管肉瘤 14 年的长期生存。

Long-term 14-year survival with primary hepatic angiosarcoma.

机构信息

Hepatopancreatobiliary Surgery, Princess Alexandra Hospital, Woolloongabba, Queensland, Australia

Hepatopancreatobiliary Surgery, Princess Alexandra Hospital, Woolloongabba, Queensland, Australia.

出版信息

BMJ Case Rep. 2022 Aug 10;15(8):e250856. doi: 10.1136/bcr-2022-250856.

Abstract

Primary hepatic angiosarcomas (PHAs) are rare primary liver malignancies with poor outcomes due to their aggressive nature and the difficulty it presents in terms of diagnosis and management. However, early diagnosis and aggressive surgical resection combined with ongoing surveillance can confer prolonged survival in patients with PHA. Additionally, adjuvant radiotherapy may be of benefit in cases of involved surgical margins. Here we report the longest surviving patient with PHA in literature to date. The patient had an initial right hemihepatectomy 14 years ago and 10 years later had a segment III segmentectomy for recurrence on surveillance imaging. This was followed with adjuvant radiotherapy for involved margins. There has been no further recurrence noted on imaging to date, and the patient is continuing to thrive in the community.

摘要

原发性肝血管肉瘤(PHAs)是一种罕见的原发性肝脏恶性肿瘤,由于其侵袭性和诊断及治疗方面的困难,预后较差。然而,早期诊断和积极的手术切除并结合持续的监测可以延长 PHA 患者的生存时间。此外,对于有手术切缘受累的病例,辅助放疗可能有益。在此,我们报告了迄今为止文献中最长生存的 PHA 患者。该患者 14 年前行初始右半肝切除术,10 年后因监测影像学显示复发而行 III 段节段切除术。随后对受累切缘行辅助放疗。迄今为止,影像学检查未发现进一步复发,患者在社区中继续茁壮成长。

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