ArticleJournal of paediatrics and child health2026
Management of Kaposiform Haemangioendothelioma-A Retrospective Case Series Examining the Use and Effectiveness of Vincristine.
Article in Journal of paediatrics and child health, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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5 authors.
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Abstract
aimKaposiform haemangioendothelioma is a rare vascular tumour. Management has evolved over the last two decades, with most cases receiving vincristine or sirolimus. We aim to describe our experience with managing this tumour, with a focus on the safety and efficacy of vincristine.
methodsA retrospective cohort study of all cases treated at Sydney Children's Hospital over a 19-year period was conducted. Explanatory variables included age, tumour location, imaging, the presence of Kasabach-Merrit Phenomenon, and treatment modality. The primary outcome was disease recurrence and the secondary outcome, complications.
resultsTwenty-one cases were identified with 13 (60%) presenting as neonates. The most common site was limb; and imaging included ultrasound (7/21, 33%) and MRI (12/21, 57%). Almost all had consumptive coagulopathy (20/21, 95%) with 6/21 (29%) having severe Kasabach-Merrit Phenomenon. Six tumours were excised and 16 (76%) were treated with vincristine, with three (14%) progressing to treatment with sirolimus. There were no recurrences. Complications of vincristine therapy were common but mild in most cases, with only one proven blood stream infection and one requiring central line replacement.
conclusionVincristine remains a highly effective first-line treatment for kaposiform haemangioendothelioma with an acceptable safety profile. Further well-designed multicentre trials are needed to objectively compare its effectiveness and safety with sirolimus.
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