ROSAI-DORFMAN-DISEASE OF THE PARANASAL SINUSES

A 57-year-old man presented with a history of nasal obstruction of fiv e to six years duration. 'Nasal polyps' were removed on several occasi ons. He had previously had an episode of paraplegia which resolved aft er the removal of a spinal tumour. Histology from both sites was thoug ht to represent a malignant fibrous histiocytoma. On presentation the patient had computed tomographical (CT) evidence of extensive ethmoida l disease, with threatened intracranial extension. He also had evidenc e of lung and retroperitoneal disease with pancreas and kidney involve ment. The ethmoidal disease was considered potentially lethal and ther efore a craniofacial resection was performed. Review of all the histol ogy revealed that the diagnosis was extranodal Rosai-Dorfman disease ( sinus histiocytosis). The patient's course is described, and the liter ature on this disease of unknown aetiology is reviewed.