Retroperitoneal Fibrosis Differential Diagnoses

Updated: Jul 21, 2023
  • Author: Chandra Shekhar Biyani, MS, MBBS, DUrol, FRCS(Urol), FEBU; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
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Diagnostic Considerations

In the evaluation of patients with presumed idiopathic retroperitoneal fibrosis (RPF), exclude secondary RPF due to malignancy, infection, retroperitoneal injury, or drugs.

RPF can be a feature of Erdheim-Chester disease, a rare form of histiocytosis that is often characterized by symmetric osteosclerosis but may also manifest as diabetes insipidus, exophthalmos, xanthelasmas, cardiovascular involvement, bilateral adrenal enlargement, renal impairment, testis infiltration, or interstitial lung disease. Affected tissues demonstrate xanthomatous infiltration by foamy histiocytes, surrounded by fibrosis. [37]