Orbital inflammatory pseudotumor associated with multifocal systemic neoplastic immunoincompetence

A 52-year-old man developed proptosis from an orbital mass which was documented histopathologically to be an inflammatory orbital pseudotumor. When the lesion failed to resolve after six months of adrenocorticosteroid treatment, a second biopsy showed an inflammatory mass with nests of eosinophilic granuloma. The patient soon developed a lymphoma of the soft palate and a glioblastoma multiforme which led to the patient's rapid demise. Eosinophilic granuloma is known to represent the more benign end of the spectrum of histiocytic disorders in which a proliferation of Langerhans' cells and an abnormality of T-suppressor cells occur. Our case represents the first report of multifocal tumor immunoincompetence occurring with eosinophilic granuloma in an orbital inflammatory pseudotumor.