Publication details

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 Przemyslaw SCHONEWOLF-GREULICH Bitten STANCIULESCU Maria-Corina ŠTĚRBA Jaroslav TOLONEN Jukka VAISNYTE Birute CARINE Van Der Vleuten WYRZYKOWSKI Dariusz KOOL Leo Schultze VIKKULA Miikka
Year of publication	2025
Type	Peer-reviewed scientific article
Magazine / Source	European journal of pediatrics
Citation
web	https://link.springer.com/article/10.1007/s00431-025-06631-6?utm_source=getftr&utm_medium=getftr&utm_campaign=getftr_pilot&getft_integrator=clarivate
Doi	https://doi.org/10.1007/s00431-025-06631-6
Keywords	Kaposiform hemangioendothelioma; Kasabach-Merrit phenomenon; Vascular tumour; Diagnosis; Treatment; Algorithm; Sirolimus; Patient pathway
Description	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.What is Known:center dot Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor, often associated with a life threatening coagulopathy, and can be challenging to manage.What is New:center dot The diagnostic and therapeutic pathway for KHE, developed by a multidisciplinary group of experts (VASCA-VASCERN Group), offers concrete offers concrete help in making decisions about managing this condition.