Key result
Complete surgical resection of a rare orbital epithelioid haemangioendothelioma with a custom orbito-zygomatic implant achieved clear margins and good healing in a 22-year-old female.
Why the study?
Epithelioid haemangioendothelioma in the head and neck is very rare, with only four previously reported cases in the orbit.
Case Report (n=1)
No
Complete surgical resection with custom implant reconstruction is a viable treatment for the extremely rare presentation of orbital epithelioid haemangioendothelioma.
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.
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Abdullah et al. (2025) conducted a case report in Epithelioid Haemangioendothelioma of the lateral orbit (n=1). Surgical resection and custom orbito-zygomatic implant was evaluated on Tumor resection margins and clinical recovery. Complete surgical resection of a rare orbital epithelioid haemangioendothelioma with a custom orbito-zygomatic implant achieved clear margins and good healing in a 22-year-old female.
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