. 2025 Dec 13;185(1):14.

doi: 10.1007/s00431-025-06631-6.

The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

Paolo Gasparella¹, Emir Q Haxhija², Rune Andersen³, Maria Barea⁴, Eulalia Baselga⁵, Miguel Bejarano Serrano⁵, Sigurd Berger³, Annouk Anne Bisdorff⁶, Olivia Boccara⁶, Petra Borgards⁷, Maria Bom-Sucesso⁸, Laurence M Boon^{9

10}, Anca Maria Cimpean¹¹, Andrea Diociaiuti¹², Veronika Dvorakova¹³, May El Hachem¹², Sofia Frisk^{10

14}, Nader Ghaffarpour¹⁵, Annegret Holm¹⁶, Alan D Irvine¹³, Mikkel Kaltoft¹⁷, Friedrich G Kapp¹⁶, Olga Koskova¹⁸, Kristiina Kyrklund¹⁹, Miguel Madureira²⁰, Darius Palionis²¹, Przemysław Przewratil²², Bitten Schönewolf-Greulich²³, Maria-Corina Stanciulescu¹¹, Jaroslav Štěrba¹⁸, Jukka Tolonen²⁴, Birute Vaisnyte²¹, Carine van der Vleuten²⁵, Dariusz Wyrzykowski²², Leo Schultze Kool²⁶, Miikka Vikkula^{9

10}

Affiliations

¹ Department of Paediatric and Adolescent Surgery, VASCERN-VASCA European Reference Centre, Medical University of Graz, Auenbruggerplatz 34, 8036, Graz, Austria. pgasparella@yahoo.it.
² Department of Paediatric and Adolescent Surgery, VASCERN-VASCA European Reference Centre, Medical University of Graz, Auenbruggerplatz 34, 8036, Graz, Austria.
³ Department of Radiology and Nuclear Medicine, VASCERN-VASCA European Reference Centre, Oslo University Hospital, Oslo, Norway.
⁴ VASCAPA, Vascular Anomaly Patient Association, Brussels, Belgium.
⁵ Pediatric Dermatology, VASCERN-VASCA European Reference Centre, Hospital Sant Joan de Deu, Barcelona, Spain.
⁶ 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.
⁷ Bundesverband Angeborene Gefäßfehlbildungen E.V., Federal Association of Congenital Vascular Malformation, Mülheim an Der Ruhr, Germany.
⁸ Pediatric Oncology, VASCERN-VASCA European Reference Centre, Centro Hospitalar Universitário São João, Porto, Portugal.
⁹ Center for Vascular Anomalies, Division of Plastic Surgery, VASCERN-VASCA European Reference Centre, University Clinics Saint-Luc, University of Louvain, Brussels, Belgium.
¹⁰ Human Molecular Genetics, de Duve Institute, University of Louvain, Ottignies-Louvain-La-Neuve, Belgium.
¹¹ Department of Microscopic Morphology/Histology VASCERN-VASCA European Reference Centre , Victor Babes University of Medicine and Pharmacy, Timișoara, Romania.
¹² 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.
¹³ Paediatric Dermatology, Clinical Medicine, VASCERN-VASCA European Reference CentreChildren's Health IrelandTrinity College Dublin, Crumlin, Ireland.
¹⁴ Department of Clinical Genetics, VASCERN-VASCA European Reference Centre, Karolinska University Laboratory, Karolinska University Hospital, Stockholm, Sweden.
¹⁵ Department of Reconstructive Plastic and Craniofacial Surgery, VASCERN-VASCA European Reference Centre, Karolinska University Hospital, Stockholm, Sweden.
¹⁶ 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.
¹⁷ Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, VASCERN-VASCA European Reference Centre, Copenhagen University Hospital, Copenhagen, Denmark.
¹⁸ Department of Pediatric Oncology VASCERN-VASCA European Reference Centre, University Hospital Brno, Brno, Czech Republic.
¹⁹ Department of Pediatric Surgery, VASCERN-VASCA European Reference Centre, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
²⁰ Interventional Radiology, VASCERN-VASCA European Reference Centre, Centro Hospitalar Universitário São João, Porto, Portugal.
²¹ Faculty of Medicine, Vilnius University Hospital Santaros Klinikos VASCERN-VASCA European Reference Centre , Vilnius University, Vilnius, Lithuania.
²² Department of Pediatric Surgery and Oncology, Medical University of Lodz, Łódź, Poland.
²³ Department of Clinical Genetics, Rigshospitalet, VASCERN-VASCA European Reference Centre, Copenhagen University Hospital, Copenhagen, Denmark.
²⁴ Department of Internal Medicine, VASCERN-VASCA European Reference Centre, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
²⁵ 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.
²⁶ 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.

PMID: 41389323
PMCID: PMC12701870
DOI: 10.1007/s00431-025-06631-6

The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

Paolo Gasparella et al. Eur J Pediatr. 2025.

. 2025 Dec 13;185(1):14.

doi: 10.1007/s00431-025-06631-6.

Authors

Affiliations

¹ Department of Paediatric and Adolescent Surgery, VASCERN-VASCA European Reference Centre, Medical University of Graz, Auenbruggerplatz 34, 8036, Graz, Austria. pgasparella@yahoo.it.
² Department of Paediatric and Adolescent Surgery, VASCERN-VASCA European Reference Centre, Medical University of Graz, Auenbruggerplatz 34, 8036, Graz, Austria.
³ Department of Radiology and Nuclear Medicine, VASCERN-VASCA European Reference Centre, Oslo University Hospital, Oslo, Norway.
⁴ VASCAPA, Vascular Anomaly Patient Association, Brussels, Belgium.
⁵ Pediatric Dermatology, VASCERN-VASCA European Reference Centre, Hospital Sant Joan de Deu, Barcelona, Spain.
⁶ 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.
⁷ Bundesverband Angeborene Gefäßfehlbildungen E.V., Federal Association of Congenital Vascular Malformation, Mülheim an Der Ruhr, Germany.
⁸ Pediatric Oncology, VASCERN-VASCA European Reference Centre, Centro Hospitalar Universitário São João, Porto, Portugal.
⁹ Center for Vascular Anomalies, Division of Plastic Surgery, VASCERN-VASCA European Reference Centre, University Clinics Saint-Luc, University of Louvain, Brussels, Belgium.
¹⁰ Human Molecular Genetics, de Duve Institute, University of Louvain, Ottignies-Louvain-La-Neuve, Belgium.
¹¹ Department of Microscopic Morphology/Histology VASCERN-VASCA European Reference Centre , Victor Babes University of Medicine and Pharmacy, Timișoara, Romania.
¹² 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.
¹³ Paediatric Dermatology, Clinical Medicine, VASCERN-VASCA European Reference CentreChildren's Health IrelandTrinity College Dublin, Crumlin, Ireland.
¹⁴ Department of Clinical Genetics, VASCERN-VASCA European Reference Centre, Karolinska University Laboratory, Karolinska University Hospital, Stockholm, Sweden.
¹⁵ Department of Reconstructive Plastic and Craniofacial Surgery, VASCERN-VASCA European Reference Centre, Karolinska University Hospital, Stockholm, Sweden.
¹⁶ 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.
¹⁷ Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, VASCERN-VASCA European Reference Centre, Copenhagen University Hospital, Copenhagen, Denmark.
¹⁸ Department of Pediatric Oncology VASCERN-VASCA European Reference Centre, University Hospital Brno, Brno, Czech Republic.
¹⁹ Department of Pediatric Surgery, VASCERN-VASCA European Reference Centre, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
²⁰ Interventional Radiology, VASCERN-VASCA European Reference Centre, Centro Hospitalar Universitário São João, Porto, Portugal.
²¹ Faculty of Medicine, Vilnius University Hospital Santaros Klinikos VASCERN-VASCA European Reference Centre , Vilnius University, Vilnius, Lithuania.
²² Department of Pediatric Surgery and Oncology, Medical University of Lodz, Łódź, Poland.
²³ Department of Clinical Genetics, Rigshospitalet, VASCERN-VASCA European Reference Centre, Copenhagen University Hospital, Copenhagen, Denmark.
²⁴ Department of Internal Medicine, VASCERN-VASCA European Reference Centre, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
²⁵ 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.
²⁶ 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.

PMID: 41389323
PMCID: PMC12701870
DOI: 10.1007/s00431-025-06631-6

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, 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.

Keywords: Algorithm; Diagnosis; Kaposiform hemangioendothelioma; Kasabach-Merrit phenomenon; Patient pathway; Sirolimus; Treatment; Vascular tumour.

PubMed Disclaimer

Conflict of interest statement

Declarations. Ethics approval and consent to participate: Not applicable. Competing interests: The authors declare no competing interests.

Figures

**Fig. 2**
Diagnostic work-up: differential diagnosis and histological features

**Fig. 3**
Management and treatment part 1

**Fig. 4**
Management and treatment part 2

**Fig. 5**
Management and treatment: recurrence/sequelae

**Fig. 6**
A A 1-month-old infant presenting with histopathologically confirmed KHE on the back. b The same infant 3 months later, after therapy with sirolimus and corticosteroids

**Fig. 7**
A 10-year-old boy with a histopathologically confirmed tufted angioma (TA) of the right cheek

See this image and copyright information in PMC

References

1. Drolet BA, Trenor CC 3rd, Brandao LR, Chiu YE, Chun RH, Dasgupta R et al (2013) Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr 163(1):285–291 - PubMed
1. Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB et al (2013) Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 162(1):142–147 - PMC - PubMed
1. Mack JM, Crary SE (2022) How we approach coagulopathy with vascular anomalies. Pediatr Blood Cancer 69(Suppl 3):e29353 - PubMed
1. Adams DM, Trenor CC 3rd, Hammill AM, Vinks AA, Patel MN, Chaudry G et al (2016) Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics 137(2):e20153257 - PMC - PubMed
1. Lee SH, Ten Cate O, Gottlieb M, Horsley T, Shea B, Fournier K et al (2024) The use of virtual nominal groups in healthcare research: an extended scoping review. PLoS ONE 19(6):e0302437 - PMC - PubMed

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The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

Affiliations

The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

MeSH terms

Supplementary concepts

LinkOut - more resources

Full Text Sources

Medical