Abstract
Background: Cholesteatoma, characterized by the abnormal growth of keratinizing squamous epithelium in ectopic locations, most commonly arises in the middle ear. Its occurrence in the sinonasal tract is rare and presents significant diagnostic and management challenges. These lesions can lead to severe complications like bone erosion, intracranial involvement, and orbital spread. This narrative review aims to summarize the current knowledge on cholesteatomas in these regions, focusing on epidemiology, pathophysiology, diagnosis, and treatment. Methods: A comprehensive review of the English literature was conducted, focusing on reported cases of cholesteatomas in the nasal cavity and paranasal sinuses. This review examines key aspects, including epidemiological data, imaging findings, surgical strategies, and postoperative outcomes. The role of diagnostic tools, particularly computed tomography and diffusion-weighted magnetic resonance imaging, in distinguishing cholesteatomas from other sinonasal lesions is also discussed. Results: As of March 2025, 51 cases of paranasal sinus cholesteatoma were reported. The frontal sinus is the most commonly affected site, followed by the maxillary, ethmoid, and sphenoid sinuses. Diagnosis is often delayed due to nonspecific symptoms, such as nasal congestion and recurrent infections. Surgical excision is the primary treatment, with endoscopic techniques being favored for their minimally invasive nature. Recurrence remains a major concern, and although very rare, cases of squamous cell carcinoma have also been observed in association with cholesteatoma. Conclusions: Nasal and paranasal sinus cholesteatomas require early recognition and intervention to prevent complications. Advances in imaging and surgery have improved outcomes; however, further research is needed to refine therapies and understand disease mechanisms.