Abstract
Epithelioid haemangioendothelioma (EHE) is a rare vascular neoplasm characterised by proliferation of vascular endothelial and pre-endothelial cells. The prevalence is less than one in a million people. It is principally observed in the soft tissues of the extremities but can also occur in the bone, brain, liver, lung and lymph nodes. EHE in the head and neck region is very rare. The most common site of occurrence in the head and neck is the submandibular region. To the best of our knowledge, there are only four cases of EHE reported in the orbit. We herein present the unusual case of a 22-year-old female patient with an EHE of the lateral wall of the right orbit and describe the clinical findings, histopathology, differential diagnoses and treatment. EHEs exhibit the potential for malignancy and recurrence, but metastasis remains rare. Due to its noticeable potential for malignancy and recurrence, complete excision and regular long-term follow-up would be the appropriate treatment protocol.