Consensus statement for the diagnosis, treatment, and prognosis of kaposiform hemangioendothelioma

Jiangyuan Zhou¹, Tong Qiu¹, Zixin Zhang¹, Yuru Lan¹, Ran Huo², Bo Xiang¹, Siyuan Chen³, Li Qiu⁴, Chunchao Xia⁵, Xuewen Xu⁶, Jing Li⁷, Yangyang Ma⁸, Wei Yao⁹, Zuopeng Wang⁹, Changxian Dong¹⁰, Zhongping Qin¹¹, Maozhong Tai¹², Lei Guo¹³, Xin He¹⁴, Song Gu¹⁵, Li Li¹⁶, Fang Hou¹⁷, Yu Cai¹⁸, Huaijie Wang¹⁹, Jinhu Wang²⁰, Xian Jiang²¹, Jiawei Zheng²², Kai Li⁹, Yi Ji¹

Affiliations

¹ Division of Oncology, Department of Pediatric Surgery, and Rare Diseases Center, West China Hospital, Sichuan University, China.
² Department of Burn and Plastic Surgery, Shandong Provincial Hospital, Cheeloo College of Medicine, Jinan, China.
³ Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, China.
⁴ Department of Ultrasound, West China Hospital, Sichuan University, Chengdu, China.
⁵ Department of Radiology, West China Hospital, Sichuan University, Chengdu, China.
⁶ Department of Burn and Plastic Surgery, West China Hospital, Sichuan University, Chengdu, China.
⁷ Department of Evidence-Based Medicine and Clinical Epidemiology, West China Hospital, Sichuan University, Chengdu, China.
⁸ Department of Pathology, Children's Hospital of Fudan University, Shanghai, China.
⁹ Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China.
¹⁰ Department of Hemangioma Surgery, Henan Provincial People's Hospital, The People's Hospital of Zhengzhou University, Zhengzhou, China.
¹¹ Special Department of Vascular Anomalies, Tumor Hospital of Linyi City, Linyi, China.
¹² Special Department of Hemangioma, Tumor Hospital of Linyi City, Linyi, China.
¹³ Department of Vascular Anomalies and Interventional Radiology, Qilu Children's Hospital of Shandong University, Jinan, China.
¹⁴ Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
¹⁵ Department of Pediatric Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
¹⁶ Department of Dermatology, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing, China.
¹⁷ Department of Pediatric Surgery, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu, China.
¹⁸ Department of Oral and Maxillofacial Surgery, School & Hospital of Stomatology, Wuhan University, Wuhan, China.
¹⁹ Department of Pediatric Surgery and Vascular Anomalies, Xi'an International Medical Center Hospital, Xi'an, China.
²⁰ Department of Pediatric Surgery, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.
²¹ Department of Dermatology, West China Hospital, Sichuan University, China.
²² Department of Oral and Maxillofacial Surgery, Shanghai Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

PMID: 39831682
DOI: 10.1002/ijc.35344

Consensus statement for the diagnosis, treatment, and prognosis of kaposiform hemangioendothelioma

Jiangyuan Zhou et al. Int J Cancer. 2025.

. 2025 May 15;156(10):1986-1994.

doi: 10.1002/ijc.35344. Epub 2025 Jan 20.

Authors

Affiliations

¹ Division of Oncology, Department of Pediatric Surgery, and Rare Diseases Center, West China Hospital, Sichuan University, China.
² Department of Burn and Plastic Surgery, Shandong Provincial Hospital, Cheeloo College of Medicine, Jinan, China.
³ Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, China.
⁴ Department of Ultrasound, West China Hospital, Sichuan University, Chengdu, China.
⁵ Department of Radiology, West China Hospital, Sichuan University, Chengdu, China.
⁶ Department of Burn and Plastic Surgery, West China Hospital, Sichuan University, Chengdu, China.
⁷ Department of Evidence-Based Medicine and Clinical Epidemiology, West China Hospital, Sichuan University, Chengdu, China.
⁸ Department of Pathology, Children's Hospital of Fudan University, Shanghai, China.
⁹ Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China.
¹⁰ Department of Hemangioma Surgery, Henan Provincial People's Hospital, The People's Hospital of Zhengzhou University, Zhengzhou, China.
¹¹ Special Department of Vascular Anomalies, Tumor Hospital of Linyi City, Linyi, China.
¹² Special Department of Hemangioma, Tumor Hospital of Linyi City, Linyi, China.
¹³ Department of Vascular Anomalies and Interventional Radiology, Qilu Children's Hospital of Shandong University, Jinan, China.
¹⁴ Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
¹⁵ Department of Pediatric Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
¹⁶ Department of Dermatology, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing, China.
¹⁷ Department of Pediatric Surgery, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu, China.
¹⁸ Department of Oral and Maxillofacial Surgery, School & Hospital of Stomatology, Wuhan University, Wuhan, China.
¹⁹ Department of Pediatric Surgery and Vascular Anomalies, Xi'an International Medical Center Hospital, Xi'an, China.
²⁰ Department of Pediatric Surgery, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.
²¹ Department of Dermatology, West China Hospital, Sichuan University, China.
²² Department of Oral and Maxillofacial Surgery, Shanghai Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

PMID: 39831682
DOI: 10.1002/ijc.35344

Abstract

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.

Keywords: consensus statement; diagnosis; kaposiform hemangioendothelioma; prognosis; treatment.

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References

REFERENCES

1. Drolet BA, Trenor CC 3rd, Brandão LR, et al. Consensus‐derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr. 2013;163(1):285‐291.
1. Croteau SE, Liang MG, Kozakewich HP, et al. Kaposiform hemangioendothelioma: atypical features and risks of Kasabach‐Merritt phenomenon in 107 referrals. J Pediatr. 2013;162(1):142‐147.
1. Lim YH, Bacchiocchi A, Qiu J, et al. GNA14 somatic mutation causes congenital and sporadic vascular tumors by MAPK activation. Am J Hum Genet. 2016;99(2):443‐450.
1. Carli D, Kalantari S, Manicone R, et al. Kaposiform hemangioendothelioma further broadens the phenotype of PIK3CA‐related overgrowth spectrum. Clin Genet. 2021;100(5):624‐627.
1. Dadras SS, Skrzypek A, Nguyen L, et al. Prox‐1 promotes invasion of kaposiform hemangioendotheliomas. J Invest Dermatol. 2008;128(12):2798‐2806.

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Consensus statement for the diagnosis, treatment, and prognosis of kaposiform hemangioendothelioma

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Consensus statement for the diagnosis, treatment, and prognosis of kaposiform hemangioendothelioma

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