A paraneoplastic retroperitoneal fibrosis resistant to corticosteroids treated with tamoxifen.
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.
Costanzi
S
S
Renal Unit, Catholic University of the Sacred Heart, Rome, Italy. stefano.costanzi@fastwebnet.it
Zoli
A
A
Ferraro
P M
PM
Danza
F M
FM
Ferraccioli
G F
GF
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D004965:Estrogen Antagonists / Q000627:therapeutic use*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012185:Retroperitoneal Fibrosis / Q000188:drug therapy* / Q000473:pathology; D013629:Tamoxifen / Q000627:therapeutic use*
D000305:Adrenal Cortex Hormones; D004965:Estrogen Antagonists; D013629:Tamoxifen