Urothelial carcinoma of the renal pelvis accounts for about 7 to 15% of all kidney tumors. Urothelial carcinoma of the ureters accounts for about 4% of upper tract tumors. Risk factors are the same as those for bladder cancer (smoking, excess phenacetin use, long-term cyclophosphamide use, chronic irritation, exposure to certain chemicals). Inhabitants of the Balkans with endemic familial nephropathy are inexplicably predisposed to develop upper tract urothelial carcinoma. Genetically, urothelial carcinomas of the upper tract have been linked to Lynch syndrome and may be the presenting cancer.
Symptoms and Signs
Most patients present with hematuria; dysuria and urinary frequency may occur if the bladder also is involved. Colicky pain may accompany obstruction (see Obstructive Uropathy Obstructive Uropathy Obstructive uropathy is structural or functional hindrance of normal urine flow, sometimes leading to renal dysfunction (obstructive nephropathy). Symptoms, less likely in chronic obstruction... read more ). Uncommonly, hydronephrosis results from a renal pelvic tumor.
Ultrasonography or CT with contrast
Cytology or histology
In patients with unexplained urinary tract symptoms, typically ultrasonography or CT with contrast is done. If the diagnosis cannot be excluded, cytologic or histologic analysis is done for confirmation. Ureteroscopy is done when biopsy of the upper tract is needed or when urine cytology is positive but no source of the malignant cells is obvious. Abdominal and pelvic CT and chest x-ray are done to determine tumor extent and to check for metastases.
Prognosis depends on depth of penetration into or through the uroepithelial wall, which is difficult to determine. Likelihood of cure is > 90% for patients with a superficial, localized tumor but is 10 to 15% for those with a deeply invasive tumor. If tumors penetrate the wall or distant metastases occur, cure is unlikely.
Excision or ablation
Posttreatment surveillance with cystoscopy
Usual treatment is radical nephroureterectomy, including excision of a cuff of bladder and regional lymphadenectomy. Partial ureterectomy with or without reimplantation is indicated in some carefully selected patients (eg, patients with a distal ureteral tumor, decreased renal function, or a solitary kidney). Immediate postoperative intravesical chemotherapy with gemcitabine and mitomycin-C is recommended to reduce risk of intravesical recurrence. Neoadjuvant chemotherapy prior to nephroureterectomy is recommended for high-grade and high-stage lesions because other treatments can decrease renal function, often preventing subsequent use of adjuvant chemotherapy. PD-1 and/or PD-L1 checkpoint inhibitors are now being used for upper tract urothelial cancer and may prove to be a useful alternative treatment for patients ineligible for cisplatin (often used as neoadjuvant chemotherapy).
Laser fulguration for accurately staged and adequately visualized renal pelvic or low-grade ureteral tumors is sometimes possible. Occasionally, a drug, such as mitomycin C or bacille Calmette-Guérin (BCG), is instilled.
Periodic surveillance cystoscopy is indicated because renal pelvic and ureteral cancers tend to recur in the bladder, and such recurrence, if detected at an early stage, may be treated by fulguration, transurethral resection, or intravesical instillations. Management of metastases is the same as that for metastatic bladder cancer.
Risk of renal pelvis and ureteral cancers increases with smoking, phenacetin or cyclophosphamide use, chronic irritation, or exposure to certain chemicals.
Do ultrasonography or CT with contrast if urinary tract symptoms are unexplained.
Confirm the diagnosis histologically.
Excise or ablate tumors, usually using radical nephroureterectomy, and monitor patients with periodic cystoscopy.