Background: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that frequently occurs in soft tissues. Patients suffer from local recurrence and remote metastasis because of its malignant potential. Here, we present a rare case of EHE that originated from nasal cavity and invaded intracranially through the anterior skull base. Case Description: This is a 27-year-old woman who presented a local physician with intermittent epistaxis and a facial pain around her nose. Preoperative studies demonstrated that the tumor invaded into anterior skull base and the dura matter. Therefore, we performed combined skull base and transnasal surgery, which achieved complete resection of the tumor. Postoperative course of the patient was uneventful. No recurrence or distant metastasis was observed in the patient for 2 years following the radical resection. Conclusions: To date, four cases of EHE in the nasal cavity were reported. This is the frst case in which EHE demonstrated invasive potentials with intracranial extension. Radical surgical resection plays an important role for better management of invasive paranasal EHE.
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