Transitional cell carcinoma of the ureter

Transitional cell carcinoma of the ureter are uncommon compared to similar tumours elsewhere along the urinary tract, but are nonetheless the most common primary tumour of the ureter.

This article concerns itself with transitional cell carcinomas of the ureter specifically. For a general discussion of this tumour 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

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

Clinical presentation is relatively non-specific, most frequently consisting of haematuria and / or flank pain due to obstructive hydronephrosis. Clots may cause renal colic and mimic ureterolithiasis.

Distribution

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 tumours are found ^3-4.

Pathology

As is the case elsewhere, two main morphologic patterns are recognised:

1. papillary
   • account for 60% tumours ¹
   • multiple frondlike papillary projections
   • tend to be low grade and invasion beyond the mucosa is a late feature
2. non-papillary
   • sessile or nodular tumours
   • tend to be high grade with early invasion beyond mucosa

Radiographic features

Ultrasound

Ultrasound has little role to play in the diagnosis of TCC of the ureter, although it may suggest the diagnosis by diagnosing hydronephrosis.

CT

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:

1. by intravenous injection (CT IVP and conventional IVP)
2. by direct instillation of contrast into the collecting system
   1. antegrade pyelography via a nephrostomy
   2. 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 TTCs of the ureter) with sensitivity (96%) and specificity (99%) ³. 

Due to the small calibre of the ureter, tumours are more likely to obstruct the kidney at small tumour size. Obstruction may lead to hyrdonephrosis with or without hydroureter and may also result in a non-functioning kidney or delayed nephrogram.

Smaller or polypoid tumours 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 tumour. This may lead to the so-called goblet sign, best seen on retrograde ureterography ².

Occasionally circumferential thickening of the ureteric wall results from diffuse infiltration an may have an apple core appearance ⁴.

Treatment and prognosis

Typically, and certainly in the case of locally advanced tumours, treatment is surgical consisting of a nephroureterectomy, taking not only the kidney but also the ureter and a cuff of bladder at the vesicoureteric junction ^4-5.

In low stage tumours and especially in patients with bilateral tumours (e.g. Balkan nephropathy) or solitary kidneys, renal sparing surgery may be attempted, in which tumours 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 tumour (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.