Zhou Jiangyuan, Qiu Tong, Zhang Zixin, Lan Yuru, Huo Ran, Xiang Bo, Chen Siyuan, Qiu Li, Xia Chunchao, Xu Xuewen, Li Jing, Ma Yangyang, Yao Wei, Wang Zuopeng, Dong Changxian, Qin Zhongping, Tai Maozhong, Guo Lei, He Xin, Gu Song, Li Li, Hou Fang, Cai Yu, Wang Huaijie, Wang Jinhu, Jiang Xian, Zheng Jiawei, Li Kai, Ji Yi
Division of Oncology, Department of Pediatric Surgery, and Rare Diseases Center, West China Hospital, Sichuan University, China.
Department of Burn and Plastic Surgery, Shandong Provincial Hospital, Cheeloo College of Medicine, Jinan, China.
Int J Cancer. 2025 May 15;156(10):1986-1994. doi: 10.1002/ijc.35344. Epub 2025 Jan 20.
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that typically presents in infancy or early childhood. As awareness of KHE increases, it is imperative that the management of KHE be updated to reflect the latest evidence-based guidelines. The aim of this study was to integrate the literature and Chinese expert opinions to provide updated recommendations that will guide the diagnosis, treatment, and prognosis of patients with KHE. According to this consensus statement, 28 nationally peer-recognized experts in vascular anomalies and an expert in evidence-based medicine were assembled and formed three consensus subgroups. A series of key themes and questions were developed for each group, including recommendations for diagnosis, treatment, and prognosis. A systematic search was conducted for English-language articles published in PubMed and other relevant studies identified by the expert panel. A diagnosis of KHE necessitates the integration of clinical, imaging, and histologic features. The treatment of KHE should be tailored to the specific characteristics of each patient, including the size of the lesion, the presence of symptoms, the location, and the overall condition of the patient. In addition to focusing on the disease itself, it is also important to consider the complications of KHE and their impact on prognosis. The recommendations presented herein are intended to assist in the guidance of clinical practice and decision-making in patients with KHE, with the objective of improving patient outcomes.
卡波西样血管内皮瘤（KHE）是一种罕见的血管肿瘤，通常在婴儿期或幼儿期出现。随着对KHE认识的增加，更新KHE的管理以反映最新的循证指南势在必行。本研究的目的是整合文献和中国专家意见，以提供最新建议，指导KHE患者的诊断、治疗和预后。根据本共识声明，召集了28位全国同行认可的血管异常专家和1位循证医学专家，组成了三个共识小组。为每个小组制定了一系列关键主题和问题，包括诊断、治疗和预后的建议。对PubMed上发表的英文文章以及专家小组确定的其他相关研究进行了系统检索。KHE的诊断需要综合临床、影像学和组织学特征。KHE的治疗应根据每个患者的具体特征进行调整，包括病变大小、症状的存在、位置以及患者的整体状况。除了关注疾病本身，考虑KHE的并发症及其对预后的影响也很重要。本文提出的建议旨在协助指导KHE患者的临床实践和决策，以改善患者的治疗效果。
