Comprehensive Management Of Upper Tract Urothelial Carcinoma

Main Category: Urology / Nephrology
Also Included In: Cancer / Oncology
Article Date: 02 Feb 2009 - 3:00 PDT

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UroToday.com - Primary urothelial carcinoma of the upper tract is a rare urological disease and has a propensity for multifocality, local recurrence, and development of metastases. Almost 5% of all urothelial neoplasms occur in the kidney and ureters. The vast majority of upper tract tumors arise in the kidney, comprising 4% to 15% of all primary kidney neoplasms in the United States, whereas ureteral tumors represent only 1%. As a result, urothelial carcinoma of the bladder has been examined to a greater extent than urothelial tumors elsewhere.

The main treatment for patients with upper tract urothelial neoplasms and a normal contralateral kidney is a complete nephroureterectomy with removal of a cuff of urinary bladder. Due to the high rate of ureteral stump recurrence, which has been reported to be between 30% and 75%, it is important to complete the nephroureterectomy with a cuff of urinary bladder. Hall et al. reported in one of the largest series in the literature on 252 patients who were treated for upper tract urothelial tumors with a median follow-up of 64 months. One hundred ninety-four (76.6%) patients underwent open radical nephroureterectomy with removal of bladder cuff. Forty-two (16.7%) patients underwent parenchymal-sparing surgery, 14 (5.6%) patients underwent nephrectomy alone, and 2 (0.8%) had exploration only for nonresectable disease. Overall, patients undergoing parenchymal-sparing surgery had a lower actuarial 5-year disease-free survival rate than those treated with initial aggressive surgical resection (23% versus 45%, P < .0009). Patients with grades 1 and 2 tumors were equally distributed in these 2 groups. This study supported the use of aggressive open surgical resection for initial treatment of upper tract urothelial tumors, with a 5-year disease-free survival rate of 45%.

Nevertheless, the gold standard of open radical nephroureterectomy with resection of a bladder cuff is being challenged by minimally invasive approaches to the managing of upper tract transitional cell carcinoma (TCC). For upper tract urothelial carcinoma, laparoscopic nephroureterectomy has been used as an alternative to an open procedure. Since the first laparoscopic nephroureterectomy, performed by Clayman in May 1991 at Washington University (St.Louis, Mo, USA), numerous reports regarding the safety and efficacy of that procedure have been published. This paper covers the therapeutic approaches to upper tract TCC, including laparoscopic nephroureterectomy, endoscopic approaches, and the prognostic value of lymphadenectomy in patients with muscle invasion. Topical immunotherapy, adjuvant chemotherapy, and adjuvant radiation therapy are also discussed.

Treatment of upper-tract urothelial carcinoma has developed and changed with advances in technology. Treatment has evolved from open radical nephroureterectomy to percutaneous resection to ureteroscopic treatment. Adjuvant treatments are also evolving with topical immunotherapy, radiation, and chemotherapy. Before any decision for optimal treatment, the specifics of each individual patient with regard to renal function, medical comorbidities, location of disease, tumor stage, and tumor grade must be taken into account.

Due to the fact that the incidence and prevalence of this tumor is low, the majority of series in the literature are of limited number. What is clear from the literature with regard to surgical outcomes for upper-tract TCC is that this is a potentially lethal disease if not treated appropriately. Due to its relative rarity, many decisions regarding treatment are extrapolated from our experience in managing bladder urothelial carcinoma (such as node dissections, topical chemotherapies, immunotherapies, and adjuvant treatments). The problem with the studies utilizing minimally invasive techniques is that they lack long term follow-up. Almost all of the studies are retrospective in nature and therefore flawed with selection biases.

As a result, the standard way still remains surgical removal with radical nephroureterectomy, and for selected patients segmental ureterectomy may be performed. Endoscopic management is also reasonable in patients with lowgrade and low-stage disease as long as they adhere to a strict follow-up protocol that includes frequent cytology and endoscopy. The benefits of adjuvant radiation and chemotherapy are still debated, but the literature does reveal some improvement in disease-specific survival using both forms of treatment.

Written by Georgios Koukourakis1, Georgios Zacharias2, Michael Koukourakis3, Kiriaki Pistevou-Gobaki4, Christos Papaloukas4, Athanasios Kostakopoulos5 and Vassilios Kouloulias1 as part of Beyond the Abstract on UroToday.com

1 Radiation Therapy Unit, 2nd Department of Radiology, Athens University Medical School, Attikon University Hospital, Rimini 1 Street, Haidari, 12462 Athens, Greece
2 Section of Pathology, Policlinic of Athens, Piraeus 5 Street, 11474 Athens, Greece
3 Radiation Therapy Unit, University Hospital of Thrace, 68100 Alexandroupolis, Greece
4 Radiation-Oncology Department, AHEPA University Hospital of Thessalonica, 54301 Thessalonica, Greece
5 Section of Urology, University Hospital of Athens, 10029 Athens, Greece

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