Epithelioid Haemangioendothelioma
Pronunciation: ep-ih-THEE-lee-oyd HEE-man-jee-oh-en-doh-THEE-lee-OH-mah
Also written as: EHE — Epithelioid Haemangioendothelioma
A rare, intermediate-grade malignant vascular neoplasm arising in soft tissue or bone—including the jaws—composed of epithelioid endothelial cells embedded in a myxochondroid stroma, with unpredictable clinical behaviour.
Full Definition
Epithelioid Haemangioendothelioma (EHE) is a vascular tumour of intermediate malignant potential that can occur in the soft tissues, liver, lung, and bone, including the gnathic bones. It is characterised histologically by cords and nests of rounded, epithelioid endothelial cells with intracytoplasmic vacuoles (primitive vascular lumina) set in a myxochondroid matrix. Its behaviour ranges from indolent to frankly malignant with metastasis. Genetically, most cases harbour WWTR1–CAMTA1 or YAP1–TFE3 fusions, and these are diagnostically relevant molecular markers. Editors should note the British and American spelling variants: 'haemangioendothelioma' (British) vs 'hemangioendothelioma' (American), and must apply house style consistently. Do not confuse with epithelioid haemangioma (a benign entity) or angiosarcoma (fully malignant).
Usage
Usage note: Apply house style for British ('haemangioendothelioma') vs American ('hemangioendothelioma') spelling. Do not abbreviate to 'EHE' without first expansion. Confirm that the text clearly distinguishes intermediate-grade from fully malignant vascular tumours.
In Context
- "Immunohistochemistry confirmed CD31 and ERG positivity in the epithelioid cells, supporting a diagnosis of epithelioid haemangioendothelioma (EHE) of the mandible." — Surgical Pathology Report
- "The copy-editor standardised all instances of 'hemangioendothelioma' to 'haemangioendothelioma' in accordance with the journal's British English house style." — Journal Manuscript