Kaposiform Haemangioendothelioma
Pronunciation: kah-POH-zee-form hee-MAN-jee-oh-en-doh-THEE-lee-OH-mah
Also written as: KHE — Kaposiform Haemangioendothelioma
A rare locally aggressive vascular tumour of infancy and early childhood associated with the potentially fatal coagulopathy known as Kasabach–Merritt phenomenon.
Full Definition
Kaposiform haemangioendothelioma (KHE) is a locally invasive vascular neoplasm that typically presents as a large, violaceous, indurated mass in the retroperitoneum, mediastinum, or extremities of neonates and young children. It is histologically characterised by infiltrating nodules of spindle cells with slit-like vascular spaces resembling Kaposi sarcoma, often with haemosiderin deposition. KHE is the principal tumour associated with Kasabach–Merritt phenomenon (KMP), a consumptive thrombocytopenia and coagulopathy driven by platelet trapping within the tumour vasculature. GLUT1 immunostaining is negative, distinguishing KHE from infantile haemangioma. Editors should not confuse KHE with Kaposi sarcoma, which is an entirely different entity predominantly seen in immunocompromised adults.
Usage
Usage note: Not to be confused with Kaposi sarcoma; always distinguish KHE from infantile haemangioma using GLUT1 status when editing pathology reports.
In Context
- "The retroperitoneal mass, associated with severe thrombocytopenia, was diagnosed as kaposiform haemangioendothelioma with Kasabach–Merritt phenomenon." — Paediatric surgical pathology report
- "Unlike infantile haemangioma, kaposiform haemangioendothelioma is GLUT1-negative and does not undergo spontaneous involution." — Review article