Case 16-2025: A 34-Year-Old Man with a Nasopharyngeal Mass

This Case Records of the Massachusetts General Hospital article presents a 34-year-old man with a prolonged history of left ear pain, conductive hearing loss, facial droop, and a nasopharyngeal mass that ultimately extended intracranially and encased the left internal carotid artery. Despite multiple treatments for presumed otitis media and allergic rhinitis, symptoms progressed. Imaging revealed a masslike lesion with enhancement of multiple cranial nerves and surrounding tissues. Multiple biopsies culminated in the diagnosis of granulomatosis with polyangiitis (GPA), confirmed by histopathology showing necrotizing granulomatous vasculitis and positive proteinase-3 antineutrophil cytoplasmic antibodies (PR3-ANCA). The patient was treated with rituximab, cyclophosphamide, glucocorticoids, and avacopan. Symptoms and inflammation improved substantially with therapy. This case highlights the diagnostic complexity of GPA with isolated head and neck involvement and the value of repeated biopsies when initial findings are inconclusive.

This Case Records of the Massachusetts General Hospital article presents a 34-year-old man with a prolonged history of left ear pain, conductive hearing loss, facial droop, and a nasopharyngeal mass that ultimately extended intracranially and encased the left internal carotid artery. Despite multiple treatments for presumed otitis media and allergic rhinitis, symptoms progressed. Imaging revealed a masslike lesion with enhancement of multiple cranial nerves and surrounding tissues. Multiple biopsies culminated in the diagnosis of granulomatosis with polyangiitis (GPA), confirmed by histopathology showing necrotizing granulomatous vasculitis and positive proteinase-3 antineutrophil cytoplasmic antibodies (PR3-ANCA). The patient was treated with rituximab, cyclophosphamide, glucocorticoids, and avacopan. Symptoms and inflammation improved substantially with therapy. This case highlights the diagnostic complexity of GPA with isolated head and neck involvement and the value of repeated biopsies when initial findings are inconclusive.