The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

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.

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.

• Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor, often associated with a life threatening coagulopathy, and can be challenging to manage.

• 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.