Medical management of tumors associated with Kasabach-Merritt phenomenon: an expert survey

Area of Science:

• Hematology
• Pediatric Oncology
• Vascular Anomalies

Background:

• Kasabach-Merritt phenomenon (KMP) is a rare, life-threatening coagulopathy associated with vascular tumors like kaposiform hemangioendothelioma (KHE) and tufted angioma (TA).
• Optimal medical management for KMP remains challenging due to a lack of established treatment guidelines and consensus.

Purpose of the Study:

• To survey expert opinions from vascular anomaly centers on the management of KHE/TA with and without KMP.
• To identify current treatment trends and establish potential recommendations for KMP management.

Main Methods:

• A survey was distributed to 27 vascular anomaly centers in the US and Canada.
• Centers responded to 2 representative cases: KHE/TA with KMP and KHE/TA without KMP.

Main Results:

• Most centers (92%) lack a standardized practice for KMP management.
• Common initial therapy for KHE with KMP involved corticosteroids and vincristine (50%), followed by corticosteroids alone (29%).
• Management for KHE/TA without KMP showed variability, with corticosteroids, monitoring, vincristine, propranolol, aspirin, and rapamycin being used.

Conclusions:

• Current management of KMP-associated tumors lacks standardized protocols and consensus among experts.
• Trends indicate a preference for combination therapy with corticosteroids and vincristine for KMP, but significant variability exists.
• Further research and collaborative efforts are needed to establish evidence-based treatment guidelines for KMP.