Primary Vaginal Epithelioid Haemangioendothelioma with Pulmonary Metastasis: A case report and review of the literature

Epithelioid haemangioendothelioma (EHE) is a rare vascular sarcoma of intermediate malignancy. In 2023, a 31-year-old female presented to a tertiary care hospital in Muscat, Oman, with symptoms later diagnosed as primary vaginal EHE. The patient had primary, locally invasive vaginal EHE that subsequently metastasised to the lungs. The patient experienced progression of systemic disease despite chemotherapy and was therefore transitioned to palliative chemotherapy. The clinical, radiological and histopathological findings as well as the diagnostic features that distinguish EHE from its aggressive mimic, epithelioid angiosarcoma, are described in this report. This case highlights the diagnostic challenges, the importance of molecular confirmation and the unpredictable clinical course of this ultra-rare malignancy. To the best of the authors' knowledge, this is the first reported case of primary vaginal EHE.