{"title":"Auckland Sarcoma Unit Experience of Epithelioid Haemangioendothelioma.","authors":"Joshua Mitchell, Claire Paul, Andrew Johnston","doi":"10.1111/ans.70337","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Epithelioid haemangioendothelioma (EHE) is a rare vascular sarcoma of endothelial origin, characterized by heterogeneous clinical presentations with limited consensus on optimal management. This study aims to describe the incidence, clinical features, treatment, and outcomes of EHE within the Auckland regional sarcoma unit.</p><p><strong>Methods: </strong>A retrospective review was conducted of 20 patients diagnosed with histological confirmed EHE between 1999 and 2025, managed by the Auckland Sarcoma Unit, which receives referrals across the North Island of New Zealand. Data was extracted from electronic medical records, and incomplete records were excluded. Variables analyzed included incidence, demographics, tumor location, metastatic patterns, treatment modalities, and mortality.</p><p><strong>Results: </strong>Twenty cases of EHE were identified in the North Island of New Zealand (1999-2025), yielding an estimated annual incidence of 0.19 per 1,000,000. The mean age at diagnosis was 57 years, with 65% female. The lower limb (50%) and liver (20%) were the most common primary sites; bony primaries accounted for 20% of cases. 50% of patients presented with metastatic disease. Metastases occurred predominantly in the lungs (60%), liver (30%), and bones (15%). Surgical resection was the main treatment for localized disease (82%), while metastatic cases were most often managed with active surveillance (56%). No patients received systemic chemotherapy. Recurrence occurred in three patients who presented with primary disease (27%). Overall mortality was 45%, higher in patients with metastasis at diagnosis (70% vs. 22%). Cumulative mortality at 1, 3, and 5 years was 15%, 25%, and 35%, respectively.</p><p><strong>Conclusion: </strong>This is the largest reported case series of EHE in New Zealand, highlighting its rarity, variable clinical course, and poor prognosis. The findings underscore the need for improved data collection and international collaboration to inform management strategies for EHE.</p>","PeriodicalId":8158,"journal":{"name":"ANZ Journal of Surgery","volume":" ","pages":""},"PeriodicalIF":1.6000,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"ANZ Journal of Surgery","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1111/ans.70337","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"SURGERY","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Epithelioid haemangioendothelioma (EHE) is a rare vascular sarcoma of endothelial origin, characterized by heterogeneous clinical presentations with limited consensus on optimal management. This study aims to describe the incidence, clinical features, treatment, and outcomes of EHE within the Auckland regional sarcoma unit.
Methods: A retrospective review was conducted of 20 patients diagnosed with histological confirmed EHE between 1999 and 2025, managed by the Auckland Sarcoma Unit, which receives referrals across the North Island of New Zealand. Data was extracted from electronic medical records, and incomplete records were excluded. Variables analyzed included incidence, demographics, tumor location, metastatic patterns, treatment modalities, and mortality.
Results: Twenty cases of EHE were identified in the North Island of New Zealand (1999-2025), yielding an estimated annual incidence of 0.19 per 1,000,000. The mean age at diagnosis was 57 years, with 65% female. The lower limb (50%) and liver (20%) were the most common primary sites; bony primaries accounted for 20% of cases. 50% of patients presented with metastatic disease. Metastases occurred predominantly in the lungs (60%), liver (30%), and bones (15%). Surgical resection was the main treatment for localized disease (82%), while metastatic cases were most often managed with active surveillance (56%). No patients received systemic chemotherapy. Recurrence occurred in three patients who presented with primary disease (27%). Overall mortality was 45%, higher in patients with metastasis at diagnosis (70% vs. 22%). Cumulative mortality at 1, 3, and 5 years was 15%, 25%, and 35%, respectively.
Conclusion: This is the largest reported case series of EHE in New Zealand, highlighting its rarity, variable clinical course, and poor prognosis. The findings underscore the need for improved data collection and international collaboration to inform management strategies for EHE.
背景：上皮样血管内皮瘤（EHE）是一种罕见的内皮源性血管肉瘤，其临床表现具有异质性，对最佳治疗方法的共识有限。本研究旨在描述奥克兰地区肉瘤单位内EHE的发病率、临床特征、治疗和结果。方法：回顾性分析了1999年至2025年间，由奥克兰肉瘤单位管理的20例经组织学证实的EHE患者，该单位接受新西兰北岛的转诊。数据从电子病历中提取，排除不完整的病历。分析的变量包括发病率、人口统计学、肿瘤位置、转移模式、治疗方式和死亡率。结果：在新西兰北岛（1999-2025）发现了20例EHE病例，估计年发病率为0.19 / 1,000,000。确诊时的平均年龄为57岁，其中65%为女性。下肢（50%）和肝脏（20%）是最常见的原发部位；骨性原发占20%。50%的患者表现为转移性疾病。转移主要发生在肺部（60%）、肝脏（30%）和骨骼（15%）。手术切除是局部疾病的主要治疗方法（82%），而转移性病例最常采用主动监测（56%）。没有患者接受全身化疗。3例出现原发疾病的患者出现复发（27%）。总死亡率为45%，诊断时有转移的患者死亡率更高（70% vs 22%）。1年、3年和5年的累积死亡率分别为15%、25%和35%。结论：这是新西兰报道的最大的EHE病例系列，突出了其罕见性，临床病程多变，预后差。研究结果强调需要改进数据收集和国际合作，为EHE管理战略提供信息。
期刊介绍：
ANZ Journal of Surgery is published by Wiley on behalf of the Royal Australasian College of Surgeons to provide a medium for the publication of peer-reviewed original contributions related to clinical practice and/or research in all fields of surgery and related disciplines. It also provides a programme of continuing education for surgeons. All articles are peer-reviewed by at least two researchers expert in the field of the submitted paper.
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