Granulomatosis With Polyangiitis Presenting as an Infratemporal Fossa Mass

Abstract

Granulomatosis with polyangiitis is a rare autoimmune disease that affects small to medium-sized blood vessels throughout the body. Here, we present a case of an infratemporal mass that was the result of granulomatosis with polyangiitis. A 51-year-old male presented to the emergency department due to right cheek and facial pain that he had been experiencing for 2 to 3 months. An MRI revealed a mass within the right infratemporal and pterygopalatine fossae extending into the inferior right orbital fissure along the maxillary division of the trigeminal nerve (V2) and the vidian nerve causing concern for malignancy. Histology from an endoscopic biopsy demonstrated multiple arteries with luminal obliteration with non-necrotizing granulomas. The patient was started on steroids and immunosuppressive therapy, which improved his symptoms and decreased the size of the residual mass. This case illustrates the need for laboratory testing, imaging, and biopsy of the involved tissue in cases where GPA is suspected to prevent treatment delays that could lead to the destruction of vital organs.

Combination Treatment of Locoregionally Aggressive Granulomatosis with Polyangiitis and Cranial Base Infiltration

“…Moreover, we have compared our cases to the available literature. We have searched for case reports and case series, yet we have only found several that are applicable [ 42 , 43 , 44 , 45 , 46 , 47 ], as we had to exclude cases without histopathological confirmation, infection, suspicion of malignancy, and lacking in data or describing other limited disease manifestations in the head and neck regions. All the presented cases are compared in Supplementary Table S1 .…”