Survey of ophthalmology vol:41 issue:1 pages:66-78
We have reviewed the literature in order to delineate the clinicopathologic definition of orbital pseudotumor, also called idiopathic nonspecific orbital inflammation. The clinical picture of orbital pseudotumor varies widely, with signs of mass effect, inflammation and/or infiltration. On computed tomography, orbital pseudotumor presents as a unilateral focal or diffuse mass. The histopathologic hallmark of orbital pseudotumor is a mixed inflammatory infiltrate with fibrosis of varying degree. Contrary to an old belief, orbital pseudotumor is not related to orbital reactive lymphoid hyperplasia (pseudolymphoma) and is not a lymphoid tumor. Atypical histopathologic findings of orbital pseudotumor include dominant sclerosis, granulomatous inflammation, vasculitis, and tissue eosinophilia. In the absence of systemic fibroinflammatory, granulomatous, and vasculitic disease, these atypical histopathologic patterns can be considered to represent subclasses of orbital pseudotumors rather then distinct entities. Clinical and prognostic characteristics of both histopathologically classical and atypical orbital pseudotumors appear to be heterogeneous. The etiology of orbital pseudotumor is unknown, but infection, autoimmune disorder, and aberrant wound healing have all been put forward as possibilities. In conclusion, orbital pseudotumor is one distinct disease albeit with many clinical and histopathologic guises.