The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

Paolo Gasparella; Emir Q Haxhija; Rune Andersen; Maria Barea; Eulalia Baselga; Miguel Bejarano Serrano; Sigurd Berger; Annouk Anne Bisdorff; Olivia Boccara; Petra Borgards; Maria Bom-Sucesso; Laurence M Boon; Anca Maria Cimpean; Andrea Diociaiuti; Veronika Dvorakova; May El Hachem; Sofia Frisk; Nader Ghaffarpour; Annegret Holm; Alan D Irvine; Mikkel Kaltoft; Friedrich G Kapp; Olga Koskova; Kristiina Kyrklund; Miguel Madureira; Darius Palionis; Przemysław Przewratil; Bitten Schönewolf-Greulich; Maria-Corina Stanciulescu; Jaroslav Štěrba; Jukka Tolonen; Birute Vaisnyte; Carine van der Vleuten; Dariusz Wyrzykowski; Leo Schultze Kool; Miikka Vikkula

doi:10.1007/s00431-025-06631-6

The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

Paolo Gasparella^*, Emir Q Haxhija, Rune Andersen, Maria Barea, Eulalia Baselga, Miguel Bejarano Serrano, Sigurd Berger, Annouk Anne Bisdorff, Olivia Boccara, Petra Borgards, Maria Bom-Sucesso, Laurence M Boon, Anca Maria Cimpean, Andrea Diociaiuti, Veronika Dvorakova, May El Hachem, Sofia Frisk, Nader Ghaffarpour, Annegret Holm, Alan D IrvineMikkel Kaltoft, Friedrich G Kapp, Olga Koskova, Kristiina Kyrklund, Miguel Madureira, Darius Palionis, Przemysław Przewratil, Bitten Schönewolf-Greulich, Maria-Corina Stanciulescu, Jaroslav Štěrba, Jukka Tolonen, Birute Vaisnyte, Carine van der Vleuten, Dariusz Wyrzykowski, Leo Schultze Kool, Miikka Vikkula

^*Corresponding author for this work

Abstract

UNLABELLED: 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.

Original language	English
Article number	14
Journal	European Journal of Pediatrics
Volume	185
Issue number	1
Pages (from-to)	14
ISSN	0340-6199
DOIs	https://doi.org/10.1007/s00431-025-06631-6
Publication status	Published - 13 Dec 2025

Keywords

Algorithm
Diagnosis
Kaposiform hemangioendothelioma
Kasabach-Merrit phenomenon
Patient pathway
Sirolimus
Treatment
Vascular tumour

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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. M., Cimpean, A. M., Diociaiuti, A., Dvorakova, V., Hachem, M. E., Frisk, S., Ghaffarpour, N., Holm, A., ... Vikkula, M. (2025). The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma. European Journal of Pediatrics, 185(1), 14. Article 14. https://doi.org/10.1007/s00431-025-06631-6

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, M-C, Štěrba, J, Tolonen, J, Vaisnyte, B, van der Vleuten, C, Wyrzykowski, D, Kool, LS & Vikkula, M 2025, 'The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma', European Journal of Pediatrics, vol. 185, no. 1, 14, pp. 14. https://doi.org/10.1007/s00431-025-06631-6

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title = "The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma",

abstract = "UNLABELLED: 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",

author = "Paolo Gasparella and Haxhija, \{Emir Q\} and Rune Andersen and Maria Barea and Eulalia Baselga and Serrano, \{Miguel Bejarano\} and Sigurd Berger and Bisdorff, \{Annouk Anne\} and Olivia Boccara and Petra Borgards and Maria Bom-Sucesso and Boon, \{Laurence M\} and Cimpean, \{Anca Maria\} and Andrea Diociaiuti and Veronika Dvorakova and Hachem, \{May El\} and Sofia Frisk and Nader Ghaffarpour and Annegret Holm and Irvine, \{Alan D\} and Mikkel Kaltoft and Kapp, \{Friedrich G\} and Olga Koskova and Kristiina Kyrklund and Miguel Madureira and Darius Palionis and Przemys{\l}aw Przewratil and Bitten Sch{\"o}newolf-Greulich and Maria-Corina Stanciulescu and Jaroslav {\v S}t{\v e}rba and Jukka Tolonen and Birute Vaisnyte and \{van der Vleuten\}, Carine and Dariusz Wyrzykowski and Kool, \{Leo Schultze\} and Miikka Vikkula",

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doi = "10.1007/s00431-025-06631-6",

language = "English",

volume = "185",

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TY - JOUR

T1 - The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

AU - Gasparella, Paolo

AU - Haxhija, Emir Q

AU - Andersen, Rune

AU - Barea, Maria

AU - Baselga, Eulalia

AU - Serrano, Miguel Bejarano

AU - Berger, Sigurd

AU - Bisdorff, Annouk Anne

AU - Boccara, Olivia

AU - Borgards, Petra

AU - Bom-Sucesso, Maria

AU - Boon, Laurence M

AU - Cimpean, Anca Maria

AU - Diociaiuti, Andrea

AU - Dvorakova, Veronika

AU - Hachem, May El

AU - Frisk, Sofia

AU - Ghaffarpour, Nader

AU - Holm, Annegret

AU - Irvine, Alan D

AU - Kaltoft, Mikkel

AU - Kapp, Friedrich G

AU - Koskova, Olga

AU - Kyrklund, Kristiina

AU - Madureira, Miguel

AU - Palionis, Darius

AU - Przewratil, Przemysław

AU - Schönewolf-Greulich, Bitten

AU - Stanciulescu, Maria-Corina

AU - Štěrba, Jaroslav

AU - Tolonen, Jukka

AU - Vaisnyte, Birute

AU - van der Vleuten, Carine

AU - Wyrzykowski, Dariusz

AU - Kool, Leo Schultze

AU - Vikkula, Miikka

PY - 2025/12/13

Y1 - 2025/12/13

N2 - UNLABELLED: 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.

AB - UNLABELLED: 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.

KW - Algorithm

KW - Diagnosis

KW - Kaposiform hemangioendothelioma

KW - Kasabach-Merrit phenomenon

KW - Patient pathway

KW - Sirolimus

KW - Treatment

KW - Vascular tumour

UR - https://www.scopus.com/pages/publications/105024822186

U2 - 10.1007/s00431-025-06631-6

DO - 10.1007/s00431-025-06631-6

M3 - Journal article

C2 - 41389323

SN - 0340-6199

VL - 185

SP - 14

JO - European Journal of Pediatrics

JF - European Journal of Pediatrics

IS - 1

M1 - 14

ER -

The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

Abstract

Keywords

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