Transitional cell carcinoma of the ureter are uncommon compared to similar tumors elsewhere along the urinary tract but are nonetheless the most common primary tumor of the ureter.
This article concerns itself with transitional cell carcinomas of the ureter specifically. For a general discussion of this tumor please see transitional cell carcinoma of the urinary tract. For other locations, please refer to transitional cell carcinoma of the renal pelvis and transitional cell carcinoma of the bladder.
Epidemiology of transitional cell carcinomas of the renal pelvis are similar to those of the rest of the urinary tract: please refer to TCCs of urinary tract for further details.
Clinical presentation is relatively non-specific, most frequently consisting of hematuria and/or flank pain due to obstructive hydronephrosis. Clots may cause renal colic and mimic ureterolithiasis.
The ureter is the least common location for transitional cell carcinoma (TCC) of the urinary tract, 2-3 times less common that TCC of the renal pelvis and 100 times less common that TCC of the bladder. It accounts for only 1% of all upper urinary tract malignancies 1.
The distal ureter is more frequently affected, presumably due to greater stasis 3-4:
- proximal third: 3%
- mid third: 24%
- distal third: 73%
In 2-5% of patients, bilateral tumors are found 3-4.
As is the case elsewhere, two main morphologic patterns are recognized:
- account for 60% tumors 1
- multiple frond-like papillary projections
- tend to be low grade and invasion beyond the mucosa is a late feature
- sessile or nodular tumors
- tend to be high grade with early invasion beyond the mucosa
Ultrasound has little role to play in the diagnosis of TCC of the ureter, although it may suggest the diagnosis by diagnosing hydronephrosis.
Conventional CT with or with contrast often struggle to diagnose a small ureteral TCC as all that may be visible is focal soft tissue thickening of the ureter. If hydronephrosis and hydroureter are present then careful examination of the transition point for a soft tissue mass should be carried out.
CT or conventional urography and direct pyelography
The collecting system can be opacified by contrast in a number of ways:
- by intravenous injection (CT IVP and conventional IVP)
- by direct instillation of contrast into the collecting system
- antegrade pyelography via a nephrostomy
- retrograde pyelography via cannulation of the ureter via cystoscopy
CT urography (CT IVP) has largely replaced conventional plain film urography and is the mainstay of both diagnosis and staging (see staging of TCC's of the ureter) with sensitivity (96%) and specificity (99%) 3.
Due to the small caliber of the ureter, tumors are more likely to obstruct the kidney at small tumor size. Obstruction may lead to hydronephrosis with or without hydroureter and may also result in a non-functioning kidney or delayed nephrogram.
Smaller or polypoid tumors may be seen as filling defects, and if they cause partial long-standing obstruction may result in focal dilatation of the ureter at the site of the tumor. This may lead to the so-called goblet sign, best seen on retrograde ureterography 2-6.
Occasionally circumferential thickening of the ureteric wall results from diffuse infiltration and may have an apple core appearance 4.
Treatment and prognosis
Typically, and certainly, in the case of locally advanced tumors, treatment is surgical consisting of a nephroureterectomy, taking not only the kidney but also the ureter and a cuff of the bladder at the vesicoureteral junction 4-5.
In low stage tumors and especially in patients with bilateral tumors (e.g. Balkan nephropathy) or solitary kidneys, renal-sparing surgery may be attempted, in which tumors are locally excised often endoscopically (percutaneous or transurethral approach) 4-5.
Instillation of bacille Calmette-Guerin (BCG) or mitomycin C into the upper tract has been investigated as an alternative to surgery in some cases 5.
Prognosis depends on the stage of the tumor (see staging of TTCs of the ureter) and histological grade (see grading of TCCs) has little influence 3. Metastases are most frequently to liver, bone, and lung 4.
- 1. Leder RA, Dunnick NR. Transitional cell carcinoma of the pelvicalices and ureter. AJR Am J Roentgenol. 1990;155 (4): 713-22. AJR Am J Roentgenol (abstract) [pubmed citation]
- 2. Dyer RB, Chen MY, Zagoria RJ. Classic signs in uroradiology. Radiographics. 2004;24 Suppl 1 (suppl 1): S247-80. doi:10.1148/rg.24si045509 [pubmed citation]
- 3. Vikram R, Sandler CM, Ng CS. Imaging and staging of transitional cell carcinoma: part 2, upper urinary tract. AJR Am J Roentgenol. 2009;192 (6): 1488-93. doi:10.2214/AJR.09.2577 [pubmed citation]
- 4. Browne RF, Meehan CP, Colville J et-al. Transitional cell carcinoma of the upper urinary tract: spectrum of imaging findings. Radiographics. 25 (6): 1609-27. doi:10.1148/rg.256045517 [pubmed citation]
- 5. Current Diagnosis and Treatment Surgery Thirteenth Edition. Gerard Doherty. McGraw-Hill Medical ISBN:0071635157 (find it at amazon.com)
- 6. Daniels RE. The goblet sign. Radiology. 1999;210 (3): 737-8. doi:10.1148/radiology.210.3.r99mr04737 - Pubmed citation