SIU 2018: Is it an Aggressive Disease? Upper Urinary Tract Transitional Cell Carcinoma

Seoul, South-Korea (UroToday.com) Makarand Khochikar, MD in place of the scheduled Dr. Pruthi, kicked off the session on upper tract urothelial carcinoma (UTUC) by focusing on the topic of UTUC aggressiveness.  He briefly introduced some basic demographic and clinical presentation information, the key points are listed below.

Presentation:
- 5-10% of renal tumors/masses are UTUC
- 2-5% of urothelial tumors occur in the upper tract (kidney and ureter)
- Peak incidence occurs between age 75-79
- Mean age at presentation: 65 (rare before age 40)
- Male: Female ratio 2:1
- Caucasian: Black ratio 2:1
- While the incidence of UTUC is rising, DSS is improving – but may be due to stage migration and earlier detection
- Symptoms:
  - Hematuria (almost routinely)
  - Flank pain

Risk factors:
- Bladder cancer
  - 2-4% incidence of UTUC in a patient with prior bladder urothelial carcinoma
  - Increased with CIS, multifocality, etc.
- Contralateral UTUC
  - 2-6% incidence
- Tobacco use
- Occupational exposure
- Cyclophosphamide
- Balkan nephropathy, NSAID abuse, Chinese herbal exposure (Aristolochia)
- Lynch syndrome (colon cancer, endometrial and UTUC)

Prognosis:
- Stage and grade dependent
- The grade was traditionally reported as Grade 1-4, but now just low grade (prior Grade 1-2) and high grade (grade 3-4)
- Stage and grade are associated with the location of disease
  - 5% of renal pelvis tumors are low-grade but 26% of ureteral tumors are low-grade          
- Low-grade has much better 5-year survival than high-grade (40-87% vs. 0-33%)
- Ta, Tis, T1 disease has much better 5-year survival than T2-T4
- N1 or M1 disease is almost universally fatal within 5 years
- Other factors in prognosis:
  -o Location? Conflicting data
  - CIS
  - Lymphovascular invasion

Anatomic considerations:
- May have similar outcomes as bladder UC stage-for-stage
- But, often presents at higher stage and grade than bladder cancer
  - 19% of UTUC patient present with metastatic disease
- Thin muscle layer of the upper urinary tract may allow for earlier invasion and spread than in bladder cancer
  - Also, make staging more difficult
  - Renal parenchyma may serve a similar protective role in renal pelvis tumors

Routes of spread:
- Direct invasion into periureteral or perinephric tissue
- Lymphatic spread
  - Para-aortic, paracaval, and ipsilateral iliac/pelvic nodes – depending on the location of disease
- Hematogenous dissemination
- Epithelial seeding
  - Metachronous bladder cancer in 15-75% of patient
  - Necessitates continued surveillance of the bladder

When seeing a patient being worked up for UTUC, what should be in the differential diagnosis?
- Urothelial carcinoma – 90% of the time
- Squamous cell carcinoma – 1-7% particularly in areas with schistosomiasis
- Adenocarcinoma – 1%, very rare
- Fibroepithelial polyps – look distinct on visual inspection, benign
- Neurofibromas – very rare

His conclusions are, appropriately, that this can be an aggressive disease if found late. Unfortunately, they are still found later than most bladder cancers. Hence, there is an unmet need for earlier detection!


Presented by: Makarand Khochikar, MS, DNB, Urology, FRCS, Urology Chief Uro-Oncologist at Siddhi Vinayak Ganpati Cancer Hospital, Miraj

Written By: Thenappan Chandrasekar, MD, Clinical Instructor, Thomas Jefferson University Twitter: @tchandra_uromd, @TjuUrology at the 38th Congress of the Society of International Urology - October 4- 7, 2018 - Seoul, South Korea

Further Related Content:
Diagnosis and Staging: The Pitfalls and the Challenges of Upper Urinary Tract Transitional Cell Carcinoma
Conservative Management in Upper Urinary Tract Transitional Cell Carcinoma