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Idiopathic Retroperitoneal Fibrosis (Ormond’s Disease)
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- Ormond’s disease is a diffuse or localized fibroblastic proliferation associated with chronic inflammation. Although benign (in the sense that it does not metastasize), this lesion can be locally aggressive, with compression or obstruction of the ureters and vascular structures, including the aorta. Gastrointestinal involvement is unusual, but may occur.
- Clinical:
- Usually occurs in adults; rarely in children
- patients present with a history of pain in the lower abdomen, flank, or lower back.
- One third of cases associated with drugs (esp. methysergide and adrenergic blocking agents).
- May be a part of multiple fibrosclerosis (systemic idiopathic fibrosis)
- Gross: Ill-defined fibrous mass often encasing one or both ureters ( hydronephrosis) and/ or vasculature as well.
- Microscopic:
- fibroblastic proliferation with densely hyalinized collagen
- variable (but generally obvious) chronic inflammatory infiltrate composed of lymphocytes and plasma cells.
- germinal center formation may occur with marked inflammation
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