Juvenile 'Perinasal' Angiofibroma

The extranasopharyngeal angiofibroma is a separate clinical entity but those involving infratemporal fossa and cheek resemble juvenile nasopharyngeal angiofibroma (JNA) and hence have been labelled as juvenile perinasal angiofibroma (JPA) in this paper. This paper presents a 7th case of JPA and attempts to review the world literature on JPA, along with a proposal of staging the disease. A 16 year male presented with a painless compressible facial swelling since 7 months without any epistaxis or nasal obstruction. Initially a vascular lesion was suspected but JNA without nasal extension was strongly suspected on imaging. A deep trucut biopsy confirmed the histopathology. The vascular enhancement was significant and the tumour was excised through open approach (Weber Fergusson). JPA that can be regarded as a variant of JNA that fails to extend medially. Imaging demonstrates classical JNA findings with a clear nose/nasopharynx. A deep trucut biopsy under control in inpatient settings may sometimes help. JPA presents most commonly in Stage II where an open facial approach preferably following selective preoperative embolization is indicated. Hence with painless compressible (or non-compressible) cheek swelling suspected to be of a vascular etiology, a high degree of clinical suspicion for JPA needs to maintained in order to prevent a misdiagnosis.