Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon in an infant girl

Area of Science:

• Pediatric Oncology
• Vascular Biology
• Hematology

Background:

• Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor.
• Tufted angiomas share similar characteristics with KHE.
• Kasabach-Merritt Phenomenon (KMP) is a rare complication associated with KHE and tufted angiomas.

Observation:

• A female infant presented with a mediastinal KHE.
• The infant experienced complications including KMP, severe thrombocytopenia, hypofibrinogenemia, elevated D-dimer, life-threatening infections, and pericardial effusion.

Findings:

• Successful treatment of mediastinal KHE in an infant was achieved.
• Treatment involved a combination of prednisolone, vincristine, and sirolimus.
• This multimodal approach managed KHE, KMP, infections, and pericardial effusion.

Implications:

• This case highlights a successful, albeit complex, treatment strategy for a rare pediatric vascular tumor.
• The findings suggest that a combination of prednisolone, vincristine, and sirolimus can be effective in managing complicated KHE.
• Further research is needed due to the lack of standardized treatment protocols for KHE.