World Journal of Oncology, ISSN 1920-4531 print, 1920-454X online, Open Access
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Volume 12, Number 1, February 2021, pages 20-27

Diagnostic and Therapeutic Challenges of Rare Urogenital Cancers: Urothelial Carcinoma of the Renal Pelvis, Ureters and Urethra


Figure 1.
Figure 1. Imaging of upper urinary tract tumor. (a) CT urography showing suspicion zone of stenosis causing right unilateral hydroureteronephrosis. (b) Coronal view CT urography with filling defect in the proximal left ureters. (c) Ureteroscopy showing a non-obstructing tumor along mid ureters (white arrow showing the guidewire). CT: computed tomography.


Table 1. Available Guidelines and Summary of Clinical Presentations, Risk Factors and Diagnostics of Upper Tract Urothelial Carcinoma and Primary Urethral Cancer
Urothelial cancerAvailable guidelinesClinical presentationRisk factorsDiagnosis and imagingStaging
BOO: bladder outlet obstruction; CT: computed tomography; EAU: European Association of Urology; ESMO: European Society of Medical Oncology; MRI: magnetic resonance imaging; NCCN: National Comprehensive Cancer Network; STD: sexually transmitted disease; TMN: tumor-node-metastasis; UCF: urethrocutaneous fistula; WHO: World Health Organization.
Upper urinary tract urothelial carcinomaEAU [2], ESMO [9], NCCN [10]Early: hematuria flank pain
Late: hematuria + obstructive urinary symptoms, lumbar mass [2, 9, 10]
Smoking, aristocholic acid, Chinese herbs, aromatic amines [2, 11-13]Histopathology, CT urography, MRI urography, cystoscopy diagnostic ureteroscopy [2, 10, 14]TNM classification [2], WHO tumor grade [2]
Primary urothelial carcinoma of the urethraEAU [6], NCCN [10]Early: hematuria + bloody urethra discharge
Late: extra-urethral mass, BOO, pelvic pain, UCF, abscess, dyspareunia [6, 15]
Urethral stricture, chronic irritation, urethritis/STDs, recurrent UTI [6, 15]Histopathology, urinary cytology, diagnostic urethrocystoscopy, MRI, CT scan [6, 10]TNM classification [6], WHO tumor grade [6]


Table 2. Summary of the Management, Prognostics and Outcome of Urothelial Cancer of the Upper Urinary Tract and Urethra
Urothelial cancerLocalized diseasesLocally advancedAdvanced and metastaticPrognostic indicators5-year specific survivalFollow-up
LND: lymph node dissection; RNU: radical nephroureterectomy; SCC: squamous cell carcinoma; T: tumor; UC: urothelial carcinoma; URS: ureteroscopy.
Renal calyxLow risk: URS, RNU [2]; High risk: RNU + LND [2]Multimodal: neoadjuvant chemotherapy for select patients + RNU + LND + adjuvant chemotherapy [2, 9, 10]Systemic therapy extrapolated from bladder cancer treatmentPreoperative [2]: age/gender, ethnicity, tobacco, tumor location, surgical delay; Postoperative [2]: tumor stage/grade, lymph node status, lymphovascular invasion, surgical margin, histologypT2/pT3 < 50% [2]; pT4 < 10% [2]Follow-up is more often and rigorous in patients who have undergone kidney-sparing treatment compared to patients who had radical nephroureterectomy [2, 9, 10]
Renal pelvisLow risk: URS, percutaneous [2]; High risk: RNU + LND [2]
Proximal and mid uretersLow risk: URS [2], uretero-ureterostomy; High risk: RNU + LND [2]
Distal uretersLow risk: URS [2], distal ureterectomy; High risk: RNU + LND [2]
Primary urothelial carcinoma of the urethra in malesDistal tumor: penile-preserving surgery; primary urethrectomy [6, 15]Multimodal: neoadjuvant. chemotherapy (cisplatin-based) radiotherapy for SCC [6, 10, 15]Age, race, tumor stage and grade, nodal stage, metastasis histology, tumor size, tumor location, concomitant bladder cancer type and modality of treatment [6, 10, 15]The 5-year relative survival rate of primary UC of the urethra is 52% [6, 10, 15]Despite limited data, it is practical to adhere stricter follow-up in patients undergoing urethra-sparing surgery, with urinary cytology, urethrocystoscopy and cross-sectional imaging [6, 10, 15]
Primary urothelial carcinoma of the urethra in femalesAnterior tumor: urethral-sparing surgery [6, 10], primary urethrectomy [6, 15]Multimodal: neoadjuvant. chemotherapy (cisplatin-based) radiotherapy for SCC [6, 10, 15]