Tamoxifen monotherapy in the treatment of retroperitoneal fibrosis.

OBJECTIVE: To evaluate the therapeutic effect of tamoxifen monotherapy in patients with retroperitoneal fibrosis (RPF). PATIENTS AND METHODS: From 2007 on, 31 patients with idiopathic RPF were treated with tamoxifen monotherapy. Follow-up investigations included magnetic resonance imaging, laboratory measurements, registration of side effects and changes or removal of ureteral stents. Data were stored in the Else Kröner-Fresenius Registry of Retroperitoneal Fibrosis. RESULTS: 25 men and 6 women with a mean age of 56.6 years were treated with tamoxifen monotherapy. Mean duration of treatment was 13.3 months, mean follow-up 26.8 months. A total of 44 renal units were affected by hydronephrosis and covered by DJ stents. Radiological regression of fibrosis was detected in 22 cases (71.0%); removal of ureteral stents was possible in 27/44 renal units (61.4%) and 17/29 patients (58.6%), respectively. Most patients showed only mild or no side effects of therapy. In 7 cases (22.3%) tamoxifen therapy had to be abandoned because of severe side effects, progression of fibrosis or persistent intolerance. CONCLUSIONS: Tamoxifen is an alternative in the medical treatment of RPF, especially if patients want to avoid glucocorticoids. The potential of regression of fibrosis seems to be slightly inferior and the relapse rate is higher compared to steroids, but the rate of successful DJ removals is comparable.

An aid to decision-making in therapy of retroperitoneal fibrosis: dynamic enhancement analysis of gadolinium MRI.

BACKGROUND: Idiopathic retroperitoneal fibrosis (IRF) as an uncommon cause of obstructive uropathy is often primarily treated medically by the attending urologist. We evaluated dynamic enhancement analysis (DEA) as a possible predictor of response to medical treatment and for treatment monitoring. METHODS: From 2007, 24 patients with fibrosis were assessed by magnetic resonance imaging (MRI) with DEA. The dynamic enhancement quotient (DEQ) was measured before therapy with prednisone (n = 12) or tamoxifen (n = 12) and in follow-up investigations after 3 and 6 months. Response to medical treatment was recorded by changes in the retroperitoneal mass on MRI and possible relief of ureteral obstruction, which was monitored by intravenous pyelogram and/or MAG3 scan after removal of DJ stents. RESULTS: Treatment groups did not differ significantly as to age, gender, or laboratory values, and response to medical treatment showed no significant difference between agents. Overall there were no cases of progression, 2 cases of stable disease, 11 cases of mild fibrotic regression, and 11 of significant or complete regression. DJ stents could successfully be removed in 21 of 35 renal units (60.0%). In a total of 61 DEAs the DEQ was significantly higher (P < 0.001) in patients with a good response (DEQ = 4.02) than in those with an average response (3.11) or none (2.14). CONCLUSIONS: DEA was able to distinguish between patients with different response rates to medical treatment of IRF and may be useful to individualize therapeutic decision-making.