Otolaryngologic evaluation and management of nasal chondromesenchymal hamartoma.
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INTRODUCTION: Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor of the nasal cavity and paranasal sinuses. Its clinical relevance has increased following the discovery of its association with DICER1 syndrome-a genetic condition predisposing individuals to multiple neoplasms. These two cases highlight important clinical extremes of this disease entity and offer practical evaluation and management recommendations for practicing otolaryngologists. METHODS: A retrospective review was conducted from 1999 to 2025 at a single institution. The report includes two female teenagers presented at ages 13 and 17 years, respectively, diagnosed with NCMH-one with and one without DICER1 syndrome. Key outcomes included recurrence rates, anatomical spread, genetic testing results (including evaluation for DICER1 variants), and disease-status at follow-up. A thorough literature review on NCMH and DICER1 syndrome was conducted to provide the reader with management recommendations. RESULTS: CONCLUSIONS: Genetic testing for DICER1 variants should be standard in all newly diagnosed NCMH cases. Annual nasal endoscopic and imaging follow up is recommended in all individuals with a constitutional or mosaic pathogenic DICER1 variant. There is no published guidance for recurrent NCMH at the olfactory groove/cribriform plate region. Conservative local excisions should be pursued for tumor recurrences.