Overview
What are urothelial tumors of the renal pelvis and ureters?
What is the prevalence of urothelial tumors of the renal pelvis and ureters?
What causes urothelial tumors of the renal pelvis and ureters?
What are the histologic types of urothelial tumors of the renal pelvis and ureters?
What are the molecular markers for urothelial tumors of the renal pelvis and ureters?
What is the progression of urothelial tumors of the renal pelvis and ureters?
What is the site distribution of upper tract transitional cell carcinoma (TCC)?
What are the signs and symptoms of urothelial tumors of the renal pelvis and ureters?
When is surgery indicated for urothelial tumors of the renal pelvis and ureters?
What is the anatomy of the kidney relevant to urothelial tumors of the renal pelvis and ureters?
What are the contraindications to surgery for urothelial tumors of the renal pelvis and ureters?
Workup
What is the role of lab tests in the workup of urothelial tumors of the renal pelvis and ureters?
What is the role of CT scanning in the workup of urothelial tumors of the renal pelvis and ureters?
What is the role of cytology in the workup of urothelial tumors of the renal pelvis and ureters?
What is the role of ureteroscopy in the workup of urothelial tumors of the renal pelvis and ureters?
How is upper tract transitional cell carcinoma (TCC) diagnosed?
How are urothelial tumors of the renal pelvis and ureters staged?
Treatment
How is upper tract transitional cell carcinoma (TCC) treated?
How are metastatic and node-positive urothelial tumors of the renal pelvis and ureters treated?
What are the EAU guidelines on the treatment of low-risk upper tract urothelial carcinomas?
What is included in the long-term monitoring of urothelial tumors of the renal pelvis and ureters?
What are the possible complications of urothelial tumors of the renal pelvis and ureters?
What is the prognosis of urothelial tumors of the renal pelvis and ureters after treatment?
Which treatments are being investigated for urothelial tumors of the renal pelvis and ureters?
Guidelines
Which organizations have released guidelines on urothelial tumors of the renal pelvis and ureters?
What are the guidelines on the diagnosis of urothelial tumors of the renal pelvis and ureters?
What are the NCCN treatment guidelines for urothelial tumors of the renal pelvis and ureters?
Medications
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Intravenous pyelogram (IVP) demonstrating an upper calyx filling defect characteristic of upper tract urothelial carcinoma (UTUC). Blunting of the involved calyx is noted. Courtesy of Andrew J. Taylor, MD, University of Wisconsin Medical School.
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CT scan demonstrating right renal pelvis upper tract urothelial carcinoma (UTUC). Contrast in the renal pelvis is displaced by the tumor. Courtesy of Andrew J. Taylor, MD, University of Wisconsin Medical School.
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CT scan demonstrating left distal ureteral upper tract urothelial carcinoma (UTUC). The left ureter is dilated and a medial filling defect is noted. Courtesy of Andrew J. Taylor, MD, University of Wisconsin Medical School.
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Left retrograde ureterogram demonstrating the classic "goblet" sign of ureteral upper tract urothelial carcinoma (UTUC). Ureteral dilation distally and proximally to the tumor is present. The narrowed wall of the ureter is irregular. Courtesy of Andrew J. Taylor, MD, University of Wisconsin Medical School.
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CT scan demonstrating bulky right renal pelvis upper tract urothelial carcinoma (UTUC) replacing the majority of the renal parenchyma. A pericaval lymph node metastasis is noted. Courtesy of Andrew J. Taylor, MD, University of Wisconsin Medical School.
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CT scan demonstrating metastatic upper tract urothelial carcinoma (UTUC) of the right adrenal gland. A heterogeneous adrenal mass is noted adjacent to the spine. The superior portion of the right kidney is observed. Courtesy of Andrew J. Taylor, MD, University of Wisconsin Medical School.
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Graphic representation of templates for lymph node dissection in patients with upper tract urothelial carcinoma, as proposed by Matin et al. For tumors in the right pelvis and upper ureter, dissection encompassing the right hilar, paracaval, and retrocaval regions (orange) will remove 82.9% of the involved lymph nodes. Adding the inter-aortocaval region (green) will improve coverage to 95.8%. For left-sided pelvic tumors, removal of hilar and para-aortic lymph nodes (violet) will ensure removal of 86.9% of the involved nodes. Adding inter-aortocaval lymph nodes (green) will increase the coverage to 90.2% of involved nodes. The level of dissection along the great vessels varies for pelvic tumors. The lower limit is the inferior mesenteric artery. For upper ureteric tumors, dissection should extend up to the aortic bifurcation. For distal ureteric tumors, pelvic template dissection involving the common iliac, external iliac, obturator, and internal iliac nodes will remove 75% of involved nodes on the right side and 83.3% of involved nodes on the left side (orange and violet circles). However, adding paracaval groups for tumors on the left side (orange rectangle) and para-aortic groups for those on the right side (violet rectangle) will improve coverage to almost 100%.
Tables
Stage |
Tumor |
Node |
Metastasis |
0a |
Ta |
N0 |
M0 |
0is |
Tis |
N0 |
M0 |
I |
T1 |
N0 |
M0 |
II |
T2 |
N0 |
M0 |
III |
T3 |
N0 |
M0 |
IV |
T4 |
NX, N0 |
M0 |
Any T |
N1 |
M0 |
|
Any T |
N2 |
M0 |
|
Any T |
Any N |
M1 |
Reference |
Study design |
Outcome |
Number of patients enrolled |
Leow JJ, Martin-Doyle W, Fay AP, et al. 2014
|
Systematic review and meta-analysis |
Pooled hazard ratio (HR) for overall survival (OS) was 0.43 (95% confidence interval [CI], 0.21–0.89; P = 0.023) for adjuvant therapy group compared with controls without adjuvant therapy |
Prospective study (n = 36) investigating adjuvant carboplatin–paclitaxel and nine retrospective studies, with a total of 482 patients receiving cisplatin-based or non-cisplatin–based AC after nephroureterectomy |
Porten S, Siefker-Radtke AO, Xiao L, et al. 2014
|
Retrospective review between neoadjuvant chemotherapy group and initial surgery group |
Neoadjuvant chemotherapy had improved OS and disease-specific survival (DSS) with a 5-year DSS rate of 90.1% and a 5-year OS rate of 80.2% versus DSS and OS rates of 57.6% for those who underwent initial surgery (P = 0.0204 and P = 0.0015, respectively |
Neoadjuvant chemotherapy +surgery (n =31) and surgery only (n= 81)
|
Huang YC, Chen MF, Shi CS, et al. 2015
|
Retrospective review of patient records with pT3N0M0 upper tract urothelial carcinoma (UTUC) treated with radical nephroureterectomy and adjuvant therapy versus control group |
Statistically significant differences were found between the adjuvant and control groups in 5-year cancer-specific survival rates (80.5% vs 57.6%, P = 0.010) and recurrence-free survival rates (74.4% vs 52.9%, P = 0.026), but no statistically significant difference in overall survival (71.9% vs 49.0%, P = 0.072) |
Postoperative adjuvant chemotherapy (n= 60) vs surgery only (n=111) |
Urakami S, Yuasa T, Yamamoto S, et al. 2015 |
Retrospective analysis of clinicopathological response to induction chemotherapy and identification of prognostic factors for OS |
Clinically objective response to the induction chemotherapy occurred in 75% of patients. Histopathological analysis indicated pT0 status in 20% and pN0 in 33%. Clinical tumor response correlated significantly with achievement of pathological complete response |
60 urothelial cancer patients; primary cancer site was the urinary bladder (n= 31; 52%) and upper urinary tract (n=29; 48%) |
Lucca I, Kassouf W, Kapoor A, et al. 2015
|
Retrospective analysis of data of patients with lymph node (LN)–positive UTUC, who underwent full surgical resection followed by adjuvant chemotherapy (AC) |
In all patients (T(all) N+), administration of AC had no significant impact on UTUC-related mortality on univariable (P = 0.49) and multivariable (P = 0.11) analysis. Further stratified analyses showed that only N+ patients with pT3-4 disease benefited from AC. In this subgroup, AC reduced UTUC-related mortality by 34% (P = 0.019). |
263 patients with LN-positive UTUC underwent full surgical resection. Study group (n=107, 41%) received three to six cycles of AC, while controls (n=156; 59.3%) were treated with RNU alone |
Kim DK, Kim JW, Jung HD, et al. 2019 |
Systematic review and meta-analysis of adjuvant therapy after radical nephroureterectomy (RNU) in patients with locally advanced UTUC
|
Compared with patients who underwent RNU only, those who received adjuvant chemotherapy after RNU had HRs for disease-free survival of 0.59 (P = 0.001), cancer-specific survival of 0.73 (P = 0.02), and OS of 0.84 (P = 0.02) |
11 studies, comprising 1496 patients who underwent RNU alone and 798 patients who received ACH after RNU |
Margulis V, Puligandla M, Trabulsi EJ, et al. 2020
|
Prospective phase II trial of neoadjuvant systemic chemotherapy followed by extirpative surgery for patients with high-grade UTUC
|
Accelerated methotrexate, vinblastine, doxorubicin, and cisplatin neoadjuvant chemotherapy in patients with high-grade UTUC and creatinine clearance > 50 mL/min was safe and demonstrated predefined activity with a 14% pathologic complete response rate. |
23 men and 6 women with a median age of 65 years (range 40 to 84); 80% completed all planned treatments |
NCT01261728 |
Prospective phase II study of gemcitabine and cisplatin as neoadjuvant chemotherapy in patients with high-grade UTUC |
Expected to complete by Dec 2023 |
NA |
