Title The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma
Authors Gasparella, Paolo ; Haxhija, Emir Q ; Andersen, Rune ; Barea, Maria ; Baselga, Eulalia ; Serrano, Miguel Bejarano ; Berger, Sigurd ; Bisdorff, Annouk Anne ; Boccara, Olivia ; Borgards, Petra ; Bom-Sucesso, Maria ; Boon, Laurence M ; Cimpean, Anca Maria ; Diociaiuti, Andrea ; Dvorakova, Veronika ; Hachem, May El ; Frisk, Sofia ; Ghaffarpour, Nader ; Holm, Annegret ; Irvine, Alan D ; Kaltoft, Mikkel ; Kapp, Friedrich G ; Koskova, Olga ; Kyrklund, Kristiina ; Madureira, Miguel ; Palionis, Darius ; Przewratil, Przemysław ; Schönewolf-Greulich, Bitten ; Stanciulescu, Maria-Corina ; Štěrba, Jaroslav ; Tolonen, Jukka ; Vaišnytė, Birutė ; van der Vleuten, Carine ; Wyrzykowski, Dariusz ; Kool, Leo Schultze ; Vikkula, Miikka
DOI 10.1007/s00431-025-06631-6
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Is Part of European journal of pediatrics.. New York : Springer Nature. 2026, vol. 185, iss. 1, art. no. 14, p. [1-9].. ISSN 0340-6199. eISSN 1432-1076
Keywords [eng] algorithm ; diagnosis ; Kaposiform hemangioendothelioma ; Kasabach-Merrit phenomenon ; patient pathway ; sirolimus ; treatment ; vascular tumour
Abstract [eng] The European Reference Network (ERN) on Rare Multisystemic Vascular Diseases (VASCERN) is dedicated to gathering the best expertise in Europe in order to improve and provide high-level healthcare to all patients with rare vascular diseases and provide accessible cross-border healthcare to patients with rare vascular diseases. Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour with local aggressiveness, often associated with a life-threatening coagulopathy known as the Kasabach-Merritt phenomenon (KMP). The Vascular Anomalies Working Group (VASCA-WG) developed diagnostic and therapeutic pathways for the management of KHE using the nominal group technique (NGT), a well-established, structured, multi-stage, facilitated group meeting technique used to generate consensus statements. The pathways were developed following two face-to-face meetings and several web meetings to facilitate discussion. Conclusion: VASCA has produced this expert opinion, which reflects the strategies developed by experts and patient representatives on how to approach patients with Kaposiform Hemangiothelioma in a practical manner; here, we present an algorithmic flow chart of this work. (Table presented.).
Published New York : Springer Nature
Type Journal article
Language English
Publication date 2026
CC license CC license description