Extensive epithelioid hemangioendothelioma of the maxillary sinus: A case report

BACKGROUND: Epitheliod hemandioendothelioma (EHE) is a rare vascular tumor which was first reported by Weiss and Enzinger in 1982. It can be seen in many locations whose paranasal sinus is extremly rare. Its main treatment is surgery. To our best knowledge, no report of EHE treated by radiation therapy and chemotherapy has been described. CASE SUMMARY: A 18 years old man presented to our hospital with a 3 months history of right intermittent epistaxis, permnanent nasal obstruction and right hearing loss. The physical exam found a right exophtalmia, swelling deformatted right hemifacia and a bulky whitish tumor filling the right nasal cavity. The magnetic resonance imaging (MRI) showed an extensive hyperascularized nasopharyngeal process filling the right nasal cavity whose histopathological exam revealed an epitheloid hemangioendothelioma. Due to intracranial extension, surgery was contraindicated. The patient received 65 Gy of radiation therapy and chemotheray (cisplatin). After 18 months of follow up, the exopthalmous regressed and the MRI showed a 50% regression of the tumour size. CONCLUSION: Extensive EHE can be treated by radiation therapy and chemotherapy when surgery can’t be perfomed.