The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

Gasparella, Paolo;Haxhija, Emir Q.;Andersen, Rune;Barea, Maria;Vikkula, Miikka;et.al.
(2026) European Journal of Pediatrics — Vol. 185, n° 14 (2025)

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Abstract
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, struc- tured, 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 rep- resentatives on how to approach patients with Kaposiform Hemangiothelioma in a practical manner; here, we present an algorithmic flow chart of this work.
Affiliations
  • Medical University of GrazDepartment of Paediatric and Adolescent Surgery
  • Oslo University HospitalDepartment of Radiology and Nuclear Medicine
  • VASCAPAVascular Anomaly Patient Association
  • Hospital Sant Joan de DeuPediatric Dermatology
  • AP-HP, Université de Paris, Hôpital LariboisièreCentre Constitutif Des Malformations Artério Veineuses Superficielles de L’Enfant Et de L’Adulte, Neuroradiologie
  • Bundesverband Angeborene Gefäßfehlbildungen E.V.Federal Association of Congenital Vascular Malformation
  • Centro Hospitalar Universitário São JoãoPediatric Oncology
  • Centro Hospitalar Universitário São JoãoInterventional Radiology
  • Victor Babes University of Medicine and PharmacyDepartment of Microscopic Morphology/Histology
  • Bambino Gesù Children’s HospitalDermatology Unit and Genodermatosis Unit, Translational Pediatrics and Clinical Genetics Research Area
  • Children’s Health IrelandTrinity College DublinPaediatric Dermatology
  • Karolinska University HospitalDepartment of Clinical Genetics
  • Karolinska University HospitalDepartment of Reconstructive Plastic and Craniofacial Surgery
  • University of FreiburgDivision of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center, Faculty of Medicine
  • Copenhagen University HospitalDepartment of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet
  • Copenhagen University HospitalDepartment of Clinical Genetics
  • University Hospital BrnoDepartment of Pediatric Oncology
  • University of Helsinki and Helsinki University HospitalDepartment of Pediatric Surgery
  • University of Helsinki and Helsinki University HospitalDepartment of Internal Medicine
  • Vilnius UniversityFaculty of Medicine, Vilnius University Hospital Santaros Klinikos
  • Medical University of LodzDepartment of Pediatric Surgery and Oncology
  • Radboud University Medical CenterDepartment of Dermatology
  • Radboud University Medical CenterDepartment of Radiology

Citations

Gasparella, P., Haxhija, E. Q., Andersen, R., Barea, M., Baselga, E., Serrano, M. B., Berger, S., Bisdorff, A. A., Boccara, O., Borgards, P., Bom-Sucesso, M., Boon, L., Cimpean, A. M., Diociaiuti, A., Dvorakova, V., Hachem, M. E., Frisk, S., Ghaffarpour, N., Holm, A., et al. (2026). The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma. European Journal of Pediatrics, 185(14). https://doi.org/10.1007/s00431-025-06631-6 (Original work published 2025)