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What has become of our idiopathic inflammatory pseudo-tumors of the orbit?

OBJECTIVE. We looked for the development of specific systemic disease or malignant lymphoma in patients whose initial diagnosis was idiopathic inflammatory pseudo-tumor of the orbit (IOPT). PATIENTS AND METHODS. IOPT was diagnosed in 24 patients on the basis of imaging findings and surgical biopsies (70%). Nineteen of these 24 patients (10 men and 9 women, age range 19- 83 years) were reassessed 1-12 years after the initial diagnosis. RESULTS. The initial diagnosis described diffuse inflammation (33%), dacryoadenitis (20%) or myositis (47%). At reassessment, 4 patients had developed a specific disease: generalized lymphoma, Wegener's disease, necrotizing vasculitis, and Crohn's disease. All four were recognized within one year of the IOPT diagnosis. A biopsy was obtained in these four patients and was not contributive. One case of non-specific granulomatous proliferation was found 6 years after the initial diagnosis. One case of orbital meningioma occurred 10 years after radiotherapy of the orbit and could not be attributed to a definite cause. The other patients had a common non-specific clinical course; recurrent inflammation required corticosteroid therapy in 55% and complementary external radiotherapy of the orbit in 22%. DISCUSSION AND CONCLUSION. Specific diseases that developed after an initial diagnosis of inflammatory pseudo-tumor of the orbit occurred early and appeared more often in diffuse forms, suggesting the need for more extensive histopathological diagnostic procedures. The clinical course tended to be more quiescent in patients who passed the critical period of the first year.