Idiopathic
Retroperitoneal Fibrosis
Ormond Disease
Sclerosing Retroperitonitis
Epidemiology:
- Middle to late
ages
- Possible etiological
factors:
- Drugs
- Methysergide (ergot)
- Beta-adrenergic
blockers
- Previous surgery
- Radiation therapy
- autoimmune
Common sites:
- retroperitoneum
- mesentery
- (associated with
other fibrosing disorders):
- mediastinum
- bile ducts
- thyroid
Gross features:
Histologic features:
- infiltrative
- fibrosis
- inflammatory
infiltrate usually:
- lymphocytes – germinal
centres often
- plasma cells
- neutrophils
- sometimes granulomatous
- no anaplasia
Immunophenotype:
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Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- may encroach on ureters to produce hydronephrosis
- associated with
other sclerosing diseases:
- mediastinal fibrosis
- sclerosing cholangitis
- Riedel fibrosing thyroiditis
References: