Cyclophosphamide induced remission in relapsed, progressive idiopathic orbital inflammation ('Pseudotumour')

A patient with orbital pseudotumour with intracranial extension is reported. At presentation, the disease was confined to the orbit. Steroid therapy and orbital irradiation failed to control the condition, which then extended intracranially. Progression continued in spite of further treatment with cranial irradiation and azathioprine. Cyclophosphamide was introduced, which produced a dramatic clinical response with marked radiological improvement within a few months. This was maintained over 2 years.