Background/Purpose: Retroperitoneal fibrosis (RPF) is a periaortic sclerotic disease that encases adjacent structures, particularly the ureters. Because it is unclear whether RPF is associated with malignancy, we examined standardized incidence ratios (SIRs) of cancers in patients with RPF compared with age- and sex-matched general population. Subsequently, the characteristics of patients with RPF accompanied by malignancy were analyzed.

Methods:

Medical records of 111 patients diagnosed as having RPF by computed tomography, positron emission tomography (PET), and/or histological evaluation were reviewed. Forty one cases of cancers, which were confirmed by biopsies, were identified in 35 patients with RPF. Cancer incidence rates were calculated and compared with that observed in the Korean general population, computing the standardized incidence ratios (SIRs), which were then stratified according to RPF-cancer intervals.

Results:

The mean ± SD age at RPF diagnosis was 59.2 ± 15.0 years, and 69.4% of the patients were male. Ninety two cases (82.9%) showed peri-aortitis, and 65 cases (58.6%) presented with hydronephrosis. The cancer SIR (95% confidence intervals) in patients with RPF relative to age- and sex-matched individuals in the general population was 3.18 (2.23-4.41) [2.65 (1.70 – 3.94) in men; 5.34 (2.76 – 9.32) in women]. The most frequent cancer was unspecified urinary organ cancers with SIR of 733 (238 – 1711). SIRs of multiple myeloma [27.6 (3.34 – 99.6)], renal cell cancers [9.53, (1.15-34.4)] and adenocarcinoma of unknown primary cancers [16.9, (2.05 – 61.1)] were also significantly higher than in the general population. When stratified by RPF-cancer intervals, SIR was 10.4 (6.59 – 15.60) within 1 year of RPF diagnosis, while no significant increase in SIR was found out of 1 year around RPF diagnosis. Among malignancies (n=23) within 1 year of RPF diagnosis (RPF with concurrent cancers), predominant cellular origin was epithelial cells, such as transitional cell carcinoma (n=4) and adenocarcinoma (n=13). Although RPF patients with concurrent cancers were significantly older at the diagnosis of RPF than RPF patients without concurrent cancers (65.6 ± 12.3 vs 57.5 ± 15.2 years, respectively; p=0.017), there was no significant differences in the laboratory findings including erythrocyte sedimentation rate, C-reactive protein, and immunoglobulin G4. Moreover, the maximum standardized uptake values at RPF measured by PET were not significantly different between RPF patients with concurrent cancers (n=3) and those without (n=16) (5.17 ± 0.40 vs 4.93 ± 2.14, respectively).

Conclusion:

RPF was strongly associated with cancers within 1 year of RPF diagnosis. Our results indicate that cancer screening should be performed in patients with RPF, particularly in the aged patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/association-of-retroperitoneal-fibrosis-with-malignancy/