Orbit & optic nerve

Inflammatory pseudotumor

Last author update: 1 February 2014
Last staff update: 18 May 2022

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PubMed Search: Inflammatory pseudotumor orbit

Nat Pernick, M.D.
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Cite this page: Pernick N. Inflammatory pseudotumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyeorbitinflammatorypseudo.html. Accessed September 28th, 2023.
Definition / general
  • May not be a specific disease process, but due to various causes (paranasal sinus tumors, Rosai-Dorfman disease, inflammatory fibrosclerosis, dysthyroid ophthalmopathy, cholesterol or keratin granulomas, traumatic fat necrosis, prior hemorrhage or abscess)
  • More common than infectious granulomas
  • Usually ages 20 - 49 years with good health and sudden onset of exophthalmos with variable lid or conjunctival edema
Case reports
  • Steroids (alleviate signs and symptoms)
  • Excision
Gross description
  • Indurated orbital mass, often surrounding optic nerve and enveloping extraocular muscles
Gross images

AFIP images
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Fibrotic mass surrounds the eye

Microscopic (histologic) description
  • General:
    • Edematous tissue with excessive production of ground substance, chronic inflammatory cells, vascular proliferation and hyperplastic connective tissue
    • May have periphlebitis with tissue eosinophilia
  • Inflammatory myofibroblastic tumor:
    • Combinations of fibroblasts and myofibroblasts in background of plasma cells and other inflammatory cells
  • Rosai-Dorfman related:
    • Large histiocytes, some with lymphocytophagocytosis, lymphocytes and plasma cells, often with prominent fibrosis
Whole mount images

AFIP images
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Dense fibrotic tissue

Microscopic (histologic) images

AFIP images
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Inflammation and fibrosis

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Chronic inflammatory cells

Positive stains
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