New VASCERN Diagnostic and Management Pathways published for Kaposiform Hemangioendothelioma

A rare condition that requires shared expertise

Kaposiform Hemangioendothelioma, often referred to as KHE, is a rare and locally aggressive vascular tumour that mainly affects infants and young children. Management can be particularly complex when the condition is associated with Kasabach-Merritt phenomenon, a severe and potentially life-threatening coagulopathy.

Because KHE is rare, many clinicians encounter only a small number of cases in their careers. This can lead to variation in diagnosis, treatment approaches, and follow-up across centres and countries. For rare conditions such as this, structured pathways based on shared expertise are essential.

To support clinicians facing these challenges, members of VASCERN’s Vascular Anomalies Working Group (VASCA) have published newly developed diagnostic and management pathways in a peer-reviewed journal.

From expert discussion to structured pathways

The diagnostic and management pathways were developed through a structured consensus process. Clinicians and patient representatives from VASCERN expert centres across Europe took part in a series of face-to-face meetings and online discussions.

The group used the nominal group technique, a well-established method for reaching consensus in complex clinical settings. This approach allowed experts to discuss real-world cases, agree on best practices, and translate collective experience into clear and practical pathways. Patient representatives were involved to ensure that the pathways reflect not only clinical considerations but also patient experience and long-term care needs.

The result is an algorithmic approach that supports clinicians throughout the patient journey, from initial suspicion to diagnosis, treatment decisions, and follow-up.

What the pathways include

The published pathways provide a structured framework for managing Kaposiform Hemangioendothelioma, including:

• Diagnostic work-up based on clinical features, laboratory findings, and imaging
• Differentiation of KHE from other vascular tumours and conditions
• Assessment and management of Kasabach-Merritt phenomenon
• Treatment strategies adapted to disease severity
• Guidance on monitoring response to treatment and long-term follow-up

These pathways are designed to support consistent clinical decision-making, particularly in centres with limited experience of this rare tumour.

Read the full research article

The full article, “The VASCERN–VASCA diagnostic and management pathways for Kaposiform Hemangioendothelioma,” is available online and includes the complete algorithmic flow chart developed by the working group.