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.
Wesley
R E
RE
Department of Ophthalmology, Vanderbilt University School of Medicine, Nashville, Tennessee.
Cooper
J
J
Litchford
D W
DW
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000150:complications; D004803:Eosinophilic Granuloma / Q000150:complications; D005909:Glioblastoma / Q000150:complications; D006801:Humans; D007108:Immune Tolerance; D007249:Inflammation / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy; D008223:Lymphoma / Q000150:complications; D008297:Male; D009916:Orbital Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D009918:Orbital Neoplasms / Q000150:complications* / Q000276:immunology; D010157:Palatal Neoplasms / Q000150:complications; D010160:Palate, Soft; D014057:Tomography, X-Ray Computed
D000305:Adrenal Cortex Hormones