The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma.

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 w...

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Detalles Bibliográficos
Autores: Gasparella P, Haxhija EQ, Andersen R, Barea M, Baselga E, Serrano MB, Berger S, Bisdorff AA, Boccara O, Borgards P, Bom-Sucesso M, Boon LM, Cimpean AM, Diociaiuti A, Dvorakova V, Hachem ME, Frisk S, Ghaffarpour N, Holm A, Irvine AD, Kaltoft M, Kapp FG, Koskova O, Kyrklund K, Madureira M, Palionis D, Przewratil P, Schönewolf-Greulich B, Stanciulescu MC, Šterba J, Tolonen J, Vaisnyte B, van der Vleuten C, Wyrzykowski D, Kool LS, Vikkula M
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2025
País:España
Institución:Fundació Sant Joan de Déu
Repositorio:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:fsjd.fundanetsuite.com:p29609
Acceso en línea:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=29609
Access Level:acceso abierto
Palabra clave:Algorithm
Diagnosis
Kaposiform hemangioendothelioma
Kasabach-Merrit phenomenon
Patient pathway
Sirolimus
Treatment
Vascular tumour
Descripción
Sumario: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: • 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: • 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.

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