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  • Review Article
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Diagnostic dilemmas in patients with upper tract urothelial carcinoma

Key Points

  • High-evidence-level recommendations for diagnosis, treatment and follow-up monitoring of patients with upper tract urothelial carcinomas (UTUCs) are lacking, but radical nephroureterectomy should no longer be considered the gold-standard treatment in all patients

  • Tumour grade and pathological stage are the main predictors of treatment outcome and the diagnostic challenge is selecting those patients with low-grade and low-stage tumours eligible for kidney-sparing therapy

  • Preoperative tumour staging is difficult with currently available imaging modalities but clinical variables have been identified to enable risk stratification and distinguishing high-risk and low-risk tumours

  • Available urine tests and markers show increased sensitivity for detection of urothelial carcinoma in the lower urinary tract compared with cytology but evidence of their benefits in the upper tract is lacking

  • Endoscopic evaluation of the urinary tract together with histological evaluation of the tumour using biopsy samples are the best diagnostic instruments currently available for tumour grading

  • Developments in real-time (optical) diagnostic techniques, such as optical coherence tomography, confocal laser endomicroscopy and endoluminal ultrasonography, can potentially improve diagnosis and treatment selection for patients with UTUC

Abstract

Upper tract urothelial carcinoma (UTUC) is a rare condition and recommendations based on a high level of evidence for diagnosis, treatment and follow-up monitoring are lacking. Current decision-making is often based on evidence from trials investigating urothelial carcinoma of the lower tract. Radical nephroureterectomy has been the standard of care for UTUC but kidney-sparing treatment using endoscopic approaches has been established for a select patient group with low-grade and low-stage disease. Optimal treatment choice requires correct tumour characterization. According to available recommendations, diagnostic work-up of UTUC includes evaluation by CT urography or MRI urography, cystoscopy and urine cytology. Ureterorenoscopy and lesion biopsy are grade C recommendations in patients with suspected UTUC. When kidney-sparing treatment is planned, ureterorenoscopy and biopsy should be considered and are the procedures of choice in most cases. These diagnostics have limitations and their accuracy varies in defining tumour characteristics and predicting grade and stage. Urinary tests have higher sensitivity than cytology for detection of lower tract urothelial carcinoma but evidence of their benefit in UTUCs is lacking. New optical and image enhancement techniques are being developed to facilitate real-time diagnostics with increased accuracy. A new diagnostic algorithm for patients with suspected UTUC that integrates the diagnosis, treatment and clinical risk stratification is required.

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Figure 1: Detection of upper tract urothelial carcinoma (UTUC) with CT urography.
Figure 2: Microscopy-based assessment for upper tract urothelial carcinoma (UTUC).
Figure 3: In vivo imaging enhancement technologies.
Figure 4: New fibre-optic-based diagnostic methods for upper tract urothelial carcinoma (UTUC).
Figure 5: Proposed diagnostic and treatment algorithm for patients with suspected upper tract urothelial carcinoma (UTUC).

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M.P.L and J.B. researched data and wrote the article. D.M.d.B., P.J.Z. and J.d.l.R. made a substantial contribution to discussion of the article's content. All authors reviewed and/or edited the manuscript before submission.

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Correspondence to M. Pilar Laguna.

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Baard, J., de Bruin, D., Zondervan, P. et al. Diagnostic dilemmas in patients with upper tract urothelial carcinoma. Nat Rev Urol 14, 181–191 (2017). https://doi.org/10.1038/nrurol.2016.252

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