Glomus tumors are rare and usually benign. The malignant form (glomangiosarcoma) comprises <1% of all glomus tumors. There are limited reports that describe glomus tumors in the nasal cavity. However, to the best of our knowledge, glomangiosarcoma of the nasal cavity was never reported in humans. We report on the first case of nasal cavity glomangiosarcoma in a 59-year-old male who presented with a bleeding mass in his right nostril. We completely excised the lesion with a 0.7-mm free margin, and the histopathologic examination was consistent with glomangiosarcoma. A 6-month follow-up illustrated no evidence of recurrence or distant metastasis. Although it is rare, glomus tumors should be in the differential diagnosis of nasal cavity tumors. Histopathologic examination is essential for glomangiosarcoma diagnosis. Treatment requires complete excision with free margin, alongside careful clinical and radiological follow-up.
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