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Paranasal sinus cholesteatoma is a rare occurrence for which only a few cases have been reported in the literature, particularly in the frontal and maxillary sinuses. The clinical features are non-specific, and thus a broad differential diagnosis should be considered. In most cases, diagnostic imaging is carried out through CT and/or MRI scans, and the treatment goal is complete eradication regardless of the approach. In our review, we followed the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines and Egger et al.’s approach for Systematic Reviews in Health Care. The papers included were in English or Italian, with an available and congruous full text. Out of the 140 selected articles, we included only 22 papers, ranging from 1958 to 2020 and accounting for 24 patients. Eleven had cholesteatoma in the frontal, 9 in the maxillary sinus, and 4 in other sites. Most patients underwent CT scans or combined CT and MRI, and an open approach was the most selected procedure. Only 2 patients showed postoperative complications, and 5 patients had a recurrence over an average follow-up period of 25 months. In this first systematic review on this subject, we provide an overview of the diagnostic procedures, the proper surgical approach and the postoperative follow-up found in the literature without precise and shared guidelines.
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