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

## TL;DR

A rare case of vaginal cancer called epithelioid haemangioendothelioma is reported, with metastasis to the lungs and challenges in diagnosis.

## Contribution

This is the first reported case of primary vaginal epithelioid haemangioendothelioma.

## Key findings

- The patient had primary vaginal EHE that metastasized to the lungs.
- The case highlights diagnostic challenges in distinguishing EHE from epithelioid angiosarcoma.
- Molecular confirmation is emphasized for accurate diagnosis of this rare malignancy.

## Abstract

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.