The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma.
Por:
Gasparella P, Haxhija EQ, Andersen R, Barea M, Baselga E, Miguel Bejarano Serrano, 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 and Vikkula M
Publicada:
13 dic 2025
Ahead of Print:
13 dic 2025
Resumen:
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.
Filiaciones:
Gasparella P:
Department of Paediatric and Adolescent Surgery, VASCERN-VASCA European Reference Centre, Medical University of Graz, Auenbruggerplatz 34, 8036, Graz, Austria
Haxhija EQ:
Department of Paediatric and Adolescent Surgery, VASCERN-VASCA European Reference Centre, Medical University of Graz, Auenbruggerplatz 34, 8036, Graz, Austria
Andersen R:
Department of Radiology and Nuclear Medicine, VASCERN-VASCA European Reference Centre, Oslo University Hospital, Oslo, Norway
Barea M:
VASCAPA, Vascular Anomaly Patient Association, Brussels, Belgium
Baselga E:
Pediatric Dermatology, VASCERN-VASCA European Reference Centre, Hospital Sant Joan de Deu, Barcelona, Spain
Miguel Bejarano Serrano:
Pediatric Dermatology, VASCERN-VASCA European Reference Centre, Hospital Sant Joan de Deu, Barcelona, Spain
Berger S:
Department of Radiology and Nuclear Medicine, VASCERN-VASCA European Reference Centre, Oslo University Hospital, Oslo, Norway
Bisdorff AA:
VASCERN-VASCA European Reference Centre, AP-HP, Université de Paris, Hôpital Lariboisière, Centre Constitutif Des Malformations Artério Veineuses Superficielles de L'Enfant Et de L'Adulte, NeuroradiologieParis, France
Boccara O:
VASCERN-VASCA European Reference Centre, AP-HP, Université de Paris, Hôpital Lariboisière, Centre Constitutif Des Malformations Artério Veineuses Superficielles de L'Enfant Et de L'Adulte, NeuroradiologieParis, France
Borgards P:
Bundesverband Angeborene Gefäßfehlbildungen E.V., Federal Association of Congenital Vascular Malformation, Mülheim an Der Ruhr, Germany
Bom-Sucesso M:
Pediatric Oncology, VASCERN-VASCA European Reference Centre, Centro Hospitalar Universitário São João, Porto, Portugal
Boon LM:
Center for Vascular Anomalies, Division of Plastic Surgery, VASCERN-VASCA European Reference Centre, University Clinics Saint-Luc, University of Louvain, Brussels, Belgium
Human Molecular Genetics, de Duve Institute, University of Louvain, Ottignies-Louvain-La-Neuve, Belgium
Cimpean AM:
Department of Microscopic Morphology/Histology VASCERN-VASCA European Reference Centre , Victor Babes University of Medicine and Pharmacy, Timi?oara, Romania
Diociaiuti A:
Dermatology Unit and Genodermatosis Unit, Translational Pediatrics and Clinical Genetics Research Area, VASCERN-VASCA European Reference Centre, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
Dvorakova V:
Paediatric Dermatology, Clinical Medicine, VASCERN-VASCA European Reference CentreChildren's Health IrelandTrinity College Dublin, Crumlin, Ireland
Hachem ME:
Dermatology Unit and Genodermatosis Unit, Translational Pediatrics and Clinical Genetics Research Area, VASCERN-VASCA European Reference Centre, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
Frisk S:
Human Molecular Genetics, de Duve Institute, University of Louvain, Ottignies-Louvain-La-Neuve, Belgium
Department of Clinical Genetics, VASCERN-VASCA European Reference Centre, Karolinska University Laboratory, Karolinska University Hospital, Stockholm, Sweden
Ghaffarpour N:
Department of Reconstructive Plastic and Craniofacial Surgery, VASCERN-VASCA European Reference Centre, Karolinska University Hospital, Stockholm, Sweden
Holm A:
Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center, Faculty of Medicine, VASCERN-VASCA European Reference CentreUniversity of FreiburgUniversity of Freiburg, Freiburg, Germany
Irvine AD:
Paediatric Dermatology, Clinical Medicine, VASCERN-VASCA European Reference CentreChildren's Health IrelandTrinity College Dublin, Crumlin, Ireland
Kaltoft M:
Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, VASCERN-VASCA European Reference Centre, Copenhagen University Hospital, Copenhagen, Denmark
Kapp FG:
Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center, Faculty of Medicine, VASCERN-VASCA European Reference CentreUniversity of FreiburgUniversity of Freiburg, Freiburg, Germany
Koskova O:
Department of Pediatric Oncology VASCERN-VASCA European Reference Centre, University Hospital Brno, Brno, Czech Republic
Kyrklund K:
Department of Pediatric Surgery, VASCERN-VASCA European Reference Centre, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
Madureira M:
Interventional Radiology, VASCERN-VASCA European Reference Centre, Centro Hospitalar Universitário São João, Porto, Portugal
Palionis D:
Faculty of Medicine, Vilnius University Hospital Santaros Klinikos VASCERN-VASCA European Reference Centre , Vilnius University, Vilnius, Lithuania
Przewratil P:
Department of Pediatric Surgery and Oncology, Medical University of Lodz, Lódz, Poland
Schönewolf-Greulich B:
Department of Clinical Genetics, Rigshospitalet, VASCERN-VASCA European Reference Centre, Copenhagen University Hospital, Copenhagen, Denmark
Stanciulescu MC:
Department of Microscopic Morphology/Histology VASCERN-VASCA European Reference Centre , Victor Babes University of Medicine and Pharmacy, Timi?oara, Romania
Šterba J:
Department of Pediatric Oncology VASCERN-VASCA European Reference Centre, University Hospital Brno, Brno, Czech Republic
Tolonen J:
Department of Internal Medicine, VASCERN-VASCA European Reference Centre, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
Vaisnyte B:
Faculty of Medicine, Vilnius University Hospital Santaros Klinikos VASCERN-VASCA European Reference Centre , Vilnius University, Vilnius, Lithuania
van der Vleuten C:
Department of Dermatology, Radboudumc Expertise Center for Haemangiomas and Congenital Vascular Malformations Nijmegen (Hecovan), Radboud University Medical Center, The Netherlands
VASCERN-VASCA European Reference Centre, Nijmegen, Netherlands
Wyrzykowski D:
Department of Pediatric Surgery and Oncology, Medical University of Lodz, Lódz, Poland
Kool LS:
Department of Radiology, VASCERN-VASCA European Reference Centre, Radboudumc Expertise Center for Haemangiomas and Congenital Vascular Malformations Nijmegen (Hecovan), Radboud University Medical Center, Nijmegen, The Netherlands
Vikkula M:
Center for Vascular Anomalies, Division of Plastic Surgery, VASCERN-VASCA European Reference Centre, University Clinics Saint-Luc, University of Louvain, Brussels, Belgium
Human Molecular Genetics, de Duve Institute, University of Louvain, Ottignies-Louvain-La-Neuve, Belgium
Green Submitted, hybrid
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