The authors present a rare case of endovascular papillary angioendothelioma also known as “Dąbska tumor”, which occurs mainly in children and young adults. This neoplasm was found in the right maxillary sinus and nasal cavity in a 22-year-old woman that remained undiagnosed for one year. After the initial diagnostic imaging, the tumor was removed through the endoscopic approach. After a year, the patient developed a mucopurulent cyst in the postoperative cavity, which was removed without any recurrence of the neoplastic disease in the follow-up histopathological examination. The authors present the clinical picture of this potentially malignant neoplasm and review the current literature.
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