Epithelioid Hemangioma of the Nasal Cavity with Atypical Features: A Case Report and Review of the Literature.

Abstract Introduction: We report a unique case of a patient with epithelioid hemangioma (EH) of the nasal cavity showing atypical histological features diagnosed using Next-Generation targeted RNA Sequencing that revealed a GATA6-FOXO1 fusion transcript. The patient’s main concern and important clinical findings: It is a 53-year-old man who was experiencing recurrent episodes of right epistaxis. Nasal endoscopy revealed an exophytic well defined lesion at the head of the right middle turbinate. The primary diagnosis, interventions, and outcomes: the lesion was resected endoscopically under general anesthesia. Microscopic examination revealed solid sheets of epithelioid tumor cells with some slit-like vascular channels with moderate to severe nuclear atypia. Tumor cells were positive for ERG, CD31, CD34 and GATA6 immunostainings. The proliferation index (Ki-67) was relatively high. A Next-Generation targeted RNA Sequencing shows a GATA6-FOXO1 fusion transcript confirming the diagnosis of epithelioid hemangioma with atypical features. No clinical recurrence was detected on nasal endoscopy during the 12 months follow-up period. Conclusion:EH of the head and neck region is a diagnostic challenge for both head and neck surgeons and pathologists. The diagnosis is confirmed through histological and immunohistochemical analysis.