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头颈部血管肉瘤的明确治疗后的结果。

Outcomes after definitive treatment for head and neck angiosarcoma.

机构信息

Sydney Head and Neck Cancer Institute, Department of Head and Neck Surgery, Chris O'Brien Lifehouse, Sydney, Australia.

Westmead Hospital, Sydney, Australia.

出版信息

ANZ J Surg. 2022 Jun;92(6):1407-1414. doi: 10.1111/ans.17695. Epub 2022 May 9.

Abstract

INTRODUCTION

Head and neck angiosarcoma (HN-AS) is a rare and aggressive, representing <1% of all head and neck malignancies. It is characterized by a high rate of local recurrence and poor 5-year survival (10-54%). Australian data in this rare disease is lacking. We aim review the clinical outcomes of HN-AS from two high volume head and neck cancer units in Australia.

METHODS

Retrospective chart review.

RESULTS

A total 26 patients were identified, consist of predominantly male patients (81%) with a mean age of 77 year old. Most of the HN-AS arises from the scalp (62%). The 5-year overall survival (OS) and disease free survival (DFS) were 41% and 15%, respectively. Patients treated with upfront surgery with adjuvant therapy has better OS and DFS compared with patients receiving upfront chemoradiotherapy (median OS 3.63 vs. 0.53 years, P = 0.011 and median DFS 1.19 vs. 0.33 years, P = 0.001). There is no difference in OS or DFS for the sites of HN-AS or age of patients. Recurrences were noted in 15 patients (57.7%). For those with metastatic disease, the most common site was the lung (80%) with a median time to development of distant disease of 2.7 years (range 1.8-3.6 years). In patients who underwent salvage treatment, the median survival (post-salvage) was 1.3 years (0-2.8 years).

CONCLUSION

This is the first Australian report of treatment patterns and outcomes of patients with HN-AS. Our cohort confirms that HN-AS is a slowly progressive disease with poor survival. Patients treated with surgery and adjuvant radiotherapy in this series had better outcomes compared with those treated with chemoradiotherapy alone. The role of neoadjuvant chemotherapy is an area of ongoing research.

摘要

简介

头颈部血管肉瘤(HN-AS)是一种罕见且侵袭性强的疾病,占所有头颈部恶性肿瘤的比例<1%。其特点是局部复发率高,5 年生存率低(10-54%)。在这种罕见疾病中,澳大利亚缺乏相关数据。我们旨在回顾澳大利亚两家高容量头颈部癌症中心的 HN-AS 患者的临床结果。

方法

回顾性病历分析。

结果

共确定了 26 例患者,主要为男性(81%),平均年龄为 77 岁。大多数 HN-AS 发生于头皮(62%)。5 年总生存率(OS)和无病生存率(DFS)分别为 41%和 15%。接受初始手术联合辅助治疗的患者 OS 和 DFS 优于接受初始放化疗的患者(中位 OS 3.63 年与 0.53 年,P=0.011;中位 DFS 1.19 年与 0.33 年,P=0.001)。HN-AS 发生部位或患者年龄对 OS 或 DFS 无影响。15 例患者(57.7%)出现复发。对于发生转移的患者,最常见的部位是肺部(80%),远处转移的中位时间为 2.7 年(范围 1.8-3.6 年)。在接受挽救性治疗的患者中,中位生存(挽救后)时间为 1.3 年(0-2.8 年)。

结论

这是澳大利亚首例关于 HN-AS 患者治疗模式和结局的报告。本队列证实,HN-AS 是一种进展缓慢、生存预后差的疾病。与单独接受放化疗的患者相比,接受手术和辅助放疗的患者结局更好。新辅助化疗的作用是一个正在研究的领域。

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