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Genetic tumor syndromes are due to inherited genetic mutations, which have recently come to the attention of clinicians due to the widespread adoption of DNA sequencing, ultimately leading to imaging for surveillance. As a result, radiologists must be familiar with the clinical, genetic, and radiologic features of these syndromes. This article reviews genetic tumor syndromes of the head and neck according to the recently updated WHO’s 5th edition.

Objective Schwannomas are common nerve sheath tumors and may occur anywhere in the body. 4% of head and neck schwannomas occur in the sinonasal cavity, and fewer yet have an intracranial component, making these presentations extremely rare. Furthermore, schwannomas present with nonspecific imaging signs and can only be definitively differentiated via histopathologic review, leading to misdiagnosis as various nasal tumors. We aim to conduct a review of published literature on sinonasal schwannomas with and without intracranial extension and provide additional case representations.Methods A literature review was conducted using the PubMed Database with the terms “sinonasal schwannoma,” “intracranial,” “anterior skull base,” and “schwannoma.” Results were reviewed, and additional cases identified were referenced and included in the study. Inclusion criteria were any case with intracranial extension of the schwannoma. There were no exclusion criteria. Review data was compiled into Excel and used for data analysis and comparison. Additionally, a search was done within our institution to identify additional cases of sinonasal schwannoma.Results We identified 17 cases of sinonasal schwannoma with intracranial extension, five from our institution and twelve from literature. Analysis revealed: 8 females (47%), 9 males (53%), 9 patients presented with headaches (53%), 6 patients presented with anosmia (35%), 4 patients presented with nasal obstruction (24%), and 2 patients with no symptoms (12%). Mean age and median were 39.4 ± 10.1 and 40, respectively. For treatment, 4 patients underwent endoscopic resection (24%), 11 underwent craniotomy (65%), and data was unavailable for 2 patients. Post-treatment complications occurred in 6 patients, 5 had CSF leaks (29%) and 1 had a hematoma (6%).Conclusion We identified and discussed 17 cases of sinonasal schwannoma with intracranial extension. We hope our review provides insight for clinicians to maintain schwannoma as a potential differential when evaluating nasal and anterior skull base masses.