270 CHAPTER 6 Urological neoplasia
Transitional cell carcinoma (UC) of the renal pelvis and ureter
UC accounts for 90% of upper urinary tract tumors, the remainder being benign inverted papilloma, fibroepithelial polyp, squamous cell carcinoma (associated with longstanding calculus disease), adenocarcinoma (rare), and various rare nonurothelial tumors, including sarcoma.
•Renal pelvic UC is uncommon, accounting for 10% of renal tumors and 4% of all UC.
•Ureteral UC is rare, accounting for only 1% of all newly presenting UC. Half are multifocal; 75% are located distally; while only 3% are located in the proximal ureter.
Risk factors are similar to those for UC in the bladder (see p. 246).
•Males are affected three times more than females.
•Incidence increases with age.
•Smoking confers a two-fold risk, and there are various occupational causes.
UC does not have a genetic hereditary form, although there is a high incidence of upper tract UC in families from some villages in Balkan countries (Balkan nephropathy) that remains unexplained.
Pathology and staging
The tumor usually has a papillary structure, but occasionally it is solid. It is bilateral in 2–4%. It arises within the renal pelvis, less frequently in one of the calyces or ureter. Histologically, features of UC are present, described below. Staging is by the TNM classification. Spread is by
•Direct extension, including into the renal vein and vena cava
•Lymphatic spread to para-aortic, paracaval, and pelvic nodes
•Blood-borne spread, most commonly to liver, lung, and bone
Presentation
•Painless hematuria (80%)
•Flank pain (30%), often caused by clots passing down the ureter (“clot colic”)
•Associated with synchronous bladder UC (4%)
•At follow-up, ~50% of patients will develop a metachronous bladder UC and 2% will develop contralateral upper tract UC
Investigations
Diagnosis is usually made on urine cytology and IVP or CTU, respectively, revealing malignant cells and a filling defect in the renal pelvis or ureter. If doubt exists, selective ureteral urine cytology, retrograde pyeloureterography, or flexible ureteroscopy are indicated.
If ultrasound and cystoscopy are normal during the investigation of hematuria, an IVP or CT is recommended. Staging imaging is obtained by contrast-enhanced abdominal CT, chest X-ray, and, occasionally, isotope bone scan.
TRANSITIONAL CELL CARCINOMA OF RENAL PELVIS AND URETER 271
Staging is by the TNM (2002) classification (see Table 6.10) following histological confirmation of the diagnosis. All cases rely on physical examination and imaging, the pathological classification corresponding to the TNM categories See Box 6.4 for corresponding 5-year survival rates.
Treatment and prognosis
Nephroureterectomy
If staging indicates nonmetastatic disease in the presence of a normal contralateral kidney, the gold-standard treatment with curative intent is nephroureterectomy, open or laparoscopic.
The open approach uses either a long transperitoneal midline incision or separate flank and iliac fossa incisions. The entire ureter is taken with a cuff of bladder, because of the 50% incidence of subsequent ureteral stump recurrence. Follow-up should include annual cystoscopy and IVP or CTU to detect metachronous UC development.
Table 6.10 TNM staging of carcinomas of the renal pelvis and ureter
Tx |
Primary tumor cannot be assessed |
|
|
T0 |
No evidence of primary tumor |
|
|
Ta |
Noninvasive papillary carcinoma |
|
|
Tis |
Carcinoma in situ |
|
|
T1 |
Tumor invades subepithelial connective tissue |
|
|
T2 |
Tumor invades muscularis propria |
|
|
T3 |
Tumor invades beyond muscularis propria into perinephric or |
|
|
|
periureteral fat or renal parenchyma |
|
|
T4 |
Tumor invades adjacent organs or through kidney into perinephric fat |
|
|
Nx |
Regional (para-aortic) lymph nodes cannot be assessed |
|
|
N0 |
No regional lymph node metastasis |
|
|
N1 |
Metastasis in a single lymph node <2 cm |
|
|
N2 |
Metastasis in a single lymph node 2–5 cm or multiple nodes <5 cm |
|
|
N3 |
Metastasis in a single lymph or multiple nodes >5 cm |
|
|
Mx |
Distant metastasis cannot be assessed |
|
|
M0 |
No distant metastasis |
|
|
M1 |
Distant metastasis present |
|
|
|
|
|
|
Box 6.4 5-year survival
• |
Organ-confined disease |
T1,2 |
60–100% |
• |
Locally advanced |
T3,4 |
20–50% |
• |
Node-positive disease |
N+ |
15% |
• |
Pulmonary, bone metastases |
M+ |
10% |
272 CHAPTER 6 Urological neoplasia
Multiple techniques for laparoscopic management of the distal ureter have been described, including pure laparoscopic, endoscopic through the bladder, and open resection of the distal ureter after laparoscopic dissection of the kidney and ureter.
Percutaneous/ureteroscopic resection/ablation
For patients with a single functioning kidney or bilateral disease, or those who are unfit, percutaneous or ureteroscopic resection or ablation of the tumor are the minimally invasive options. Topical chemotherapy (e.g., mitomycin C) may subsequently be instilled through the nephrostomy or ureteral catheters. This nephron-sparing approach is less likely to be curative than definitive surgery.
Systemic combination chemotherapy for unresectable or metastatic disease using cyclophosphamide, methotrexate, and vincristine is associated with a 30% total or partial response at the expense of moderate toxicity.
Palliative surgery or arterial embolization may be necessary for troublesome hematuria.
Radiotherapy is generally ineffective.
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