Nasopharyngeal angiofibroma in an adult with Proteus syndrome. First reported case.

BACKGROUND: Proteus Syndrome (PS) is a rare hamartoneoplastic disorder consisting of a disproportionate and asymmetric overgrowth of body parts, leading to severe body and face disfigurement. Individuals with PS frequently need to undergo a number of surgical procedures including Otolaryngology interventions. Their peculiar anatomy turns even the most straightforward intervention to a challenging one.

CASE DESCRIPTION: A 31-year-old adult male with PS presented with recurrent epistaxis and nasal obstruction. A nasal mass was found during the physical examination, which was surgically removed under general anesthesia. Histology revealed juvenile angiofibroma.

CONCLUSION: This is the first reported case of a patient with PS presenting with juvenile nasopharyngeal angiofibroma. Although vascular malformations may appear in these patients, juvenile nasopharyngeal angiofibroma should be taken under consideration when PS patients present with recurrent epistaxis. HIPPOKRATIA 2017, 21(3): 147-149.