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Brief Communication: Tamoxifen Therapy for Nonmalignant Retroperitoneal Fibrosis

Eric F.H. van Bommel, MD, PhD; Tadek R. Hendriksz, MD; Antonius W.L.C. Huiskes, MD; and Antoine G.M. Zeegers, MD
[+] Article, Author, and Disclosure Information

From Albert Schweitzer Hospital, Dordrecht, the Netherlands.

Acknowledgment: The authors thank A.J.M. Cleophas, MD, PhD, and C. Siemes, MD, for their statistical advice.

Grant Support: None.

Potential Financial Conflicts of Interest: None disclosed.

Requests for Single Reprints: Eric F.H. van Bommel, MD, PhD, Department of Internal Medicine, Albert Schweitzer Hospital, Dordrecht, P.O. Box 444, NL-3300 AK Dordrecht, the Netherlands; e-mail, e.f.h.vanbommel@asz.nl.

Current Author Addresses: Dr. van Bommel and Drs. Hendriksz, Huiskes, and Zeegers: Albert Schweitzer Hospital, P.O. Box 444, NL-3300 AK Dordrecht, the Netherlands.

Author Contributions: Conception and design: E.F.H. van Bommel.

Analysis and interpretation of the data: E.F.H. van Bommel, T.R. Hendriksz, A.W.L.C. Huiskes, A.G.M. Zeegers.

Drafting of the article: E.F.H. van Bommel.

Critical revision of the article for important intellectual content: E.F.H. van Bommel, T.R. Hendriksz, A.W.L.C. Huiskes, A.G.M. Zeegers.

Final approval of the article: E.F.H. van Bommel, T.R. Hendriksz, A.W.L.C. Huiskes, A.G.M. Zeegers.

Statistical expertise: E.F.H. van Bommel.

Collection and assembly of data: E.F.H. van Bommel, T.R. Hendriksz, A.W.L.C. Huiskes.

Ann Intern Med. 2006;144(2):101-106. doi:10.7326/0003-4819-144-2-200601170-00007
Text Size: A A A

Patient characteristics at presentation are shown in Table 1. The median duration of treatment was 8.5 months (range, 5.5 to 15.0 months). Post-treatment follow-up in patients who completed 2 years of tamoxifen treatment was 9 and 59 months in 2 patients and 1 patient, respectively. Follow-up did not reveal other pathologic lesions that could have accounted for the findings at presentation.

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Anterior gallium-67 images in a 47-year-old man with retroperitoneal fibrosis before (top) and after (bottom) 6 weeks of treatment with tamoxifen (20 mg orally twice daily).

At presentation, there was intense pathologic activity in the prevertebral midabdominal region, which disappeared at follow-up gallium scanning. L = left; R = right.

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Appendix Figure 1. AZA = azathioprine; CT = computed tomography.
Flow of patients through the study.
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Appendix Figure 2.  = 0.001.
Erythrocyte sedimentation rate (ESR) at presentation and after 4 months of tamoxifen therapy.P
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Submit a Comment/Letter
Tamoxifen for retroperitoneal fibrosis
Posted on January 26, 2006
Augusto Vaglio
Department of Clinical Medicine, Nephrology and Health Science, University of Parma, Italy
Conflict of Interest: None Declared

van Bommel and colleagues report a series of 19 patients with nonmalignant retroperitoneal fibrosis (RPF) treated with tamoxifen, and suggest that it may be an effective treatment option (1). Their study is a relevant contribution, mostly because the use of tamoxifen for RPF had previously been described only in anecdotal case reports. However, a few points warrant discussion.

The authors report that RPF was idiopathic in 37% of their patients, while in 63% it was secondary to atherosclerosis (chronic periaortitis and perianeurysmal fibrosis) or recurrent pancreatitis. This is somewhat unclear. Although there are no standardised classification criteria for RPF, idiopathic RPF is usually included in the spectrum of chronic periaortitis, which also encompasses inflammatory abdominal aortic aneurysms and perianeurysmal RPF (2,3). Moreover, it is still obscure whether chronic periaortitis is actually secondary to atherosclerosis, since it may also result from an underlying systemic autoimmune disease (3,4). Additionally, we question the appropriateness of including RPF secondary to pancreatitis in this series, which is composed of RPF cases not associated/secondary to other diseases.

Another issue is the surprisingly low frequency of ureteral involvement reported in van Bommel's study: only 47% of their patients had hydronephrosis, whereas in most RPF series- although enrolled in non- urological departments- it occurs in 75%-100% of the cases (4,5). In addition, the evolution of ureteral disease during treatment is only briefly mentioned. Because creatinine levels were almost unchanged at the end of follow-up, it is likely that the patients' renal function did not substantially improve, which contrasts with the significant reduction in size of the retroperitoneal mass. Ureteral obstruction and consequent renal failure are the most severe complications of RPF, thus we believe that they should always be carefully reported, and that their outcome should represent a major end-point of treatment trials on RPF.

The authors conclude that tamoxifen may be a suitable alternative to corticosteroids. To support this statement, they report that the response rate to immunosuppressive treatment in RPF patients varies from 50% to 90% and that their results fall within this range. In RPF, response rates are not sufficient to define the effectiveness of therapy and to compare different therapeutic regimens. RPF has a chronic-relapsing course, therefore treatment studies should also aim at determining the relapse rate after treatment withdrawal. This question was not addressed by van Bommel's study because most of the enrolled patients were still receiving tamoxifen at the end of follow-up.


1. van Bommel EF, Hendriksz TR, Huiskes AW, Zeegers AG. Brief communication: tamoxifen therapy for nonmalignant retroperitoneal fibrosis. Ann Intern Med 2006; 144: 101-6

2. Parums DV. The spectrum of chronic periaortitis. Histopathology 1990; 16: 423-31

3. Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006; 367: 241-51

4. Vaglio A, Corradi D, Manenti L, Ferretti S, Garini G, Buzio C. Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med 2003; 114: 454-62

5. Marcolongo R, Tavolini IM, Laveder F, Busa M, Noventa F, Bassi P, et al. Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases. Am J Med 2004; 116: 194-7

Conflict of Interest:

None declared

tamoxifen for retroperitoneal fibrosis
Posted on March 1, 2006
Eric F.H. van Bommel
Albert Schweitzer hospital
Conflict of Interest: None Declared

IN RESPONSE: We agree with Vaglio et al that standardised classification criteria for retroperitoneal fibrosis (RPF) are lacking. However, our own experience and interpretation of the literature led us to believe that in a subset of patients with presumed idiopathic RPF, the disease is actually a local complication of advanced atherosclerosis [1]. The increased incidence of RPF in men may also be explained by the higher incidence of symptomatic atherosclerotic disease in men. Some even suggest that the future of this disorder lies in the prevention and treatment of atherosclerosis [2]. Hence, the proposed term chronic peri-aortitis (encompassing patients with and without aortic aneurysm)[3] seems more appropriate in these particular patients, which we consider a secondary form of RPF. However, in other patients such as children or those without significant atherosclerotic disease in which no potential etiologic factor can be identified, "˜true' idiopathic disease may be present. We deliberately included all patients with RPF in our study, except for those who needed immunosuppressive treatment or had concurrent malignancy. This was done to assess the efficacy of tamoxifen in patients with RPF, irrespective of the possible underlying cause. Our failure to observe an improvement of renal function over time may reflect the relatively low frequency of severe renal failure at presentation but it also reflects the presence of pre-existent chronic renal failure due to nephrosclerosis and/or diabetic nephropathy in several of our patients. In addition, long- standing obstructive nephropathy may result in irreversible renal damage. Of note, short-term improvement of renal function may simply reflect renal drainage (e.g., after ureteral stenting). We agree that RPF has a chronic- relapsing course and that we do not yet have data on the long-term efficacy of tamoxifen. However, our encouraging results with tamoxifen may be particularly relevant for patients with chronic peri-aortitis. Corticosteroid therapy may be hazardous in these patients with established vascular disease as it accelerates the atherosclerotic process and increases the risk of cardiovascular morbidity and death [4]. Conversely, increasing data suggest that tamoxifen may have significant cardioprotective effects [5]. In our study, we also observed a decrease of the serum cholesterol level following initiation of tamoxifen therapy. Therefore, we suggest serious consideration of tamoxifen as first-line treatment in patients with chronic peri-aortitis and in other patients with RPF in whom there are (relative) contra-indications for the long-term use of corticosteroids.


1. Van Bommel EFH. Retroperitoneal fibrosis. Neth J Med 2002;60:231- 242. 2. Baker LRI. Auto-allergic periaortitis (idiopathic retroperitoneal fibrosis). BJU 2003;92:663-665. 3. Parums DV. The spectrum of chronic peri-aortitis. Histopathology 1990;16:423-431. 4. Wei L, MacDonald TM, Walker BR. Taking glucocorticoids by prescription is associated with subsequent cardiovascular disease. Ann Intern Med 2004;141:764-770. 5. Grainger DJ, Schofield PM. Tamoxifen for the prevention of myocardial infarction in humans: preclinical and early clinical evidence. Circulation 2005;112:3018-3024.

Conflict of Interest:

None declared

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Summary for Patients

Tamoxifen for the Treatment of Retroperitoneal Fibrosis

The summary below is from the full report titled “Brief Communication: Tamoxifen Therapy for Nonmalignant Retroperitoneal Fibrosis.” It is in the 17 January 2006 issue of Annals of Internal Medicine (volume 144, pages 101-106). The authors are E.F.H. van Bommel, T.R. Hendriksz, A.W.L.C. Huiskes, and A.G.M. Zeegers.


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