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<p>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.</p><p><i>Conclusion</i>: 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.</p><p><Table Float="No" ID="Taba"> <tgroup cols="1"> <colspec align="left" colname="c1" colnum="1" /> <tbody> <row> <entry align="left" colname="c1"> <p><b>What is Known:</b></p> <p>•&#xa0;<i>Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor, often associated with a life threatening coagulopathy, and can be challenging to manage.</i></p> </entry> </row> <row> <entry align="left" colname="c1"> <p><b>What is New:</b></p> <p>•&#xa0;<i>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.</i></p> </entry> </row> </tbody> </tgroup> </Table></p>

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The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年12月13日

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  • 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

摘要

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.