Retroperitoneal Fibrosis Differential Diagnoses

Updated: Sep 02, 2021
  • 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 retroperitoneal fibrosis due to malignancy, infection, retroperitoneal injury, or drugs.

Retroperitoneal fibrosis 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. [29]