A Man with Juvenile Nasopharyngeal Angiofibroma, Vestibular Schwannoma, Cleft Lip and Cleft Palate, and Various Nevi: Case Report and Review

Juvenile nasopharyngeal angiofibroma is a vascular tumor that typically occurs in men. Vestibular schwannoma (acoustic neuroma) is a tumor affecting the vestibulocochlear nerve. A 38-year-old man with various pigmented lesions and history of juvenile nasopharyngeal angiofibroma, vestibular schwannoma, and cleft lip and cleft palate is described. Characteristics of patients with coexisting juvenile nasopharyngeal angiofibroma and vestibular schwannoma are summarized. A search, using PubMed, was performed for the following terms: acoustic, angiofibroma, blue, cleft, combined, dysplastic, juvenile, lip, nasopharyngeal, neuroma, nevus, palate, schwannoma, and vestibular. The relevant papers were obtained and their references were reviewed. Only one man with coincident juvenile nasopharyngeal angiofibroma and vestibular schwannoma has previously been described. The juvenile nasopharyngeal angiofibromas were both right-sided and diagnosed when the patients were 13 years old and 20 years old. Our patient’s vestibular schwannoma was ipsilateral with his juvenile nasopharyngeal angiofibroma and incidentally diagnosed on a magnetic resonance imaging (MRI) scan that was performed to monitor for recurrent juvenile nasopharyngeal angiofibroma when he was in his 30s. The other man’s vestibular schwannoma and juvenile nasopharyngeal angiofibroma were diagnosed concurrently, when he was 20 years old. The observation of a patient with cleft lip and cleft palate, various melanocytic nevi, juvenile nasopharyngeal angiofibroma, and vestibular schwannoma is unique. Although the appearance of juvenile nasopharyngeal angiofibroma and vestibular schwannoma may be coincidental, the occurrence of these tumors in the same individual may suggest an association with regards to their pathogenesis.