Necrotizing sarcoid granulomatosis with extrapulmonary involvement

Abstract

Pulmonary lesions, with sarcoid-like granulomas exhibiting noncaseous necrosis, with associated granulomatous arteritis fulfil the diagnostic criteria of necrotizing sarcoid granulomatosis (NSG). We report the case of a woman who presented with recurrent headaches, transient right hemipareses and left-sided ophthalmoplegia. An excised left retro-orbital lesion demonstrated sarcoid like changes, and the illness responded to steroid therapy. Twelve years later, the patient developed a tumour in the right lung. The resected specimen showed the histological hallmarks of NSG, and careful review of the retro-orbital lesion, removed 12 years previously, revealed similar histology. Extrapulmonary involvement in NSG is rare and has been histologically proven on only one previous occasion. The presentation of necrotizing sarcoid granulomatosis in two different systems 12 years apart is unusual and was considered worth reporting.