Objective: Our aim is to present two cases of infantile sinonasal tract myxoma with orbital involvement and conduct a comprehensive literature review of the topic. We aim to provide a summary of the presentation of infantile sinonasal tract myxomas to effectively aid clinicians in considering this rare entity as a potential diagnosis. Methods: We present a case series and a retrospective review of the published literature in the English language. A search was conducted between 1945 and 2023 on sinonasal myxoma. Cases of infantile (<3 years) sinonasal myxoma in the literature were reported as well as two cases of our own. Data was collected from each of the identified articles on the age of presentation, sex, initial presentation, tumor location, imaging, pathology, and treatment. Results: Forty-eight cases of sinonasal myxoma in children <3 years of age were identified. These cases had a slight male preponderance (F:M, 1:1.39) and an average age of diagnosis of sixteen months. Of the cases included, the majority involved the maxilla and maxillary sinuses (83.3%) and commonly presented with a painless slow-growing mass in the region of the tumor. Characteristic CT findings are homogenous solid masses with heterogeneous contrast enhancement. On MRI, these lesions appear hypointense on T1 with highly variable contrast enhancement and hyperintense on T2. Conclusions: We present two cases of infantile sinonasal myxoma with secondary orbital involvement followed by a major review. Treatment of these cases with surgical excision (confirmed clear margins) provided 0% rates of recurrence in the reported cases. Due to the rarity of these tumors and recent histological reclassification, a comprehensive review of this condition will assist clinicians in their management of it.
Keywords: WNT/β-catenin; cranial fasciitis; orbital tumor; pediatric; sinonasal tract myxoma.