A paraneoplastic retroperitoneal fibrosis resistant to corticosteroids treated with tamoxifen.

Abstract

Retroperitoneal fibrosis (RPF) is a rare disease characterized by an inflammatory proliferative fibrosing process occurring in the retroperitoneum, often causing urinary tract obstruction. Medical therapy is not well-defined, but glucocorticoids have been the mainstay of therapy. Recently, positive response to tamoxifen, an antiestrogen drug, has been reported among patients with RPF. We report the case of a 65-year-old male with a renal cell carcinoma in the upper pole of the right kidney showing acute renal failure due to a biopsy-confirmed RPF determining bilateral hydronephrosis. After polar resection of the right kidney, a high-dose oral steroid therapy did not modify the hydronephrosis. At 6 months, therapy with tamoxifen determined the retroperitoneal fibrotic mass regression and resolved the ureteral obstruction, that persists at the 13th month of follow-up. Tamoxifen can be considered as an effective alternative to corticosteroids and immunosuppressors in treating RPF.

• Authors: S Costanzi, A Zoli, P M Ferraro, F M Danza, G F Ferraccioli
• Journal: Clinical nephrology (Clin Nephrol) Vol. 70 Issue 2 Pg. 172-5 (Aug 2008) ISSN: 0301-0430 [Print] Germany
• PMID: 18793535 (Publication Type: Case Reports, Journal Article)

Chemical References

• Adrenal Cortex Hormones
• Estrogen Antagonists
• Tamoxifen

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Estrogen Antagonists (therapeutic use)
• Humans
• Magnetic Resonance Imaging
• Male
• Middle Aged
• Retroperitoneal Fibrosis (drug therapy, pathology)
• Tamoxifen (therapeutic use)