Urothelial Tumors of the Renal Pelvis and Ureters Guidelines

Updated: Nov 14, 2022
  • Author: Kyle A Richards, MD, FACS; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
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Guidelines

Guidelines Summary

Guidelines on the diagnosis and treatment of urothelial tumors of the renal pelvis and ureters have been published by the following organizations:

  • National Comprehensive Cancer Network (NCCN)
  • European Association of Urology (EAU)

Diagnosis

The NCCN guidelines recommend including the following tests in the workup of suspected renal pelvic and ureteral tumors [24] :

  • Cystoscopy
  • CT or MR urography
  • Renal ultrasound or CT without contrast with retrograde pyelography for patients who cannot receive iodinated or gadolinium-based contrast agentsl.
  • Ureteroscopy with biopsy and/or selective washings.
  • Kidney function tests
  • Chest x-ray to help evaluate for possible metastasis and assess for comorbidities
  • Urine cytology to help identify carcinoma cells

Additional imaging studies, such as renal or bone scanning, may be indicated by the test results or presence of specific symptoms. Evaluation for Lynch syndrome should be considered for those at high risk.

The EAU guidelines in general concur with NCCN and include the following key recommendations [1] :

  • Cystoscopy is performed to rule out concurrent bladder tumor
  • CT urography is performed for upper tract evaluation and staging
  • Diagnostic ureteroscopy and biopsy are performed only in cases where additional information will impact treatment decisions
  • Magnetic resonance urography or  18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET)/CT may be used when CT is contraindicated.

Treatment

The NCCN guidelines provide treatment recommendations based on grade and tumor location. For low-grade renal pelvic tumors, the guidelines recommend the following treatment [24] :

  • Nephroureterectomy with cuff of bladder with or without perioperative intravesical chemotherapy or
  • Endoscopic resection with or without postsurgical intrapelvic chemotherapy or bacillus Calmette-Guerin (BCG) 

For high-grade renal pelvic tumors and upper ureter tumors, NCCN treatment recommendations include the following [24] :

  • Nephroureterectomy with cuff of bladder and regional lymphadenectomy with or without perioperative intravesical chemotherapy
  • Neoadjuvant chemotherapy may be considered in selected patients

For low-grade upper- and mid-ureter tumors, NCCN recommended treatment options include the following [24] :

  • Endoscopic resection

For high-grade upper- and mid-ureter tumors, NCCN recommended treatment options include the following [24] :

  • Nephroureterectomy with cuff of bladder and regional lymphadenectomy
  • Consider neoadjuvant chemotherapy in selective patients
  • Excision and ureteroureterostomy/ileal ureter in highly selected patients

For distal ureter tumors, NCCN recommended treatment options include the following [24] :

  • Distal ureterectomy and regional lymphadenectomy (high grade) and reimplantation of ureter (preferred if clinically feasible) and consider neoadjuvant chemotherapy in selected patients
  • Endoscopic resection (low grade)
  • Nephroureterectomy with cuff of bladder and regional lymphadenectomy and consider neoadjuvant chemotherapy in selected patients

For metastatic disease in both renal pelvis and ureter tumors, systemic therapy is recommended by both guidelines. [1, 24]

The EAU guidelines include the following key treatment recommendations [1]

  • Kidney-sparing management is the primary treatment option for low-risk tumors.
  • Kidney-sparing management should be offered to patients with high-risk distal ureteral tumors.
  • Kidney-sparing management should be offered to select patients with solitary kidney and/or impaired renal function, provided it will not compromise survival.
  • For high-risk tumors, radical nephroureterectomy with cuff of bladder and regional lymphadenectomy and postoperative bladder instillation of chemotherapy should be offered.