Teaching Case

A 69-year-old man presented with episodes of macroscopic hematuria and bouts of mild right flank discomfort dating back 2 months.

A 69-year-old man presented with episodes of macroscopic hematuria and bouts of mild right flank discomfort dating back 2 months.
The patient underwent intravenous urography (IVU). The excretion of contrast medium from the right kidney was delayed. The acquisition of delayed images revealed pelvicalyceal dilatation of the right kidney. The ipsilateral ureter was tortuous and dilated up to its lower third where an intraluminal filling defect was faintly visualised due to the poor excretory function of the right kidney (figure 1). Subsequently the patient was submitted to retrograde pyelography. Findings included dilatation of the ureter around and distal to the filling defect, which was cup-shaped, referred to as the goblet sign (figure 2). Both studies suggested the presence of an intraluminal space-occupying lesion.
CT confirmed the presence of an intramural soft tissue density mass in the lower third of the ureter. There was no evidence of infiltration of the peri-ureteral fat (figure 3).
The patient was finally submitted to nephro-ureterectomy. Gross pathology revealed the presence of an exophytic tumor with papillary surface, which distended the urereric lumen in its distal third (figure 4a & b). Histology revealed a papillary transient cell carcinoma of the ureter, grade II (figure 5). The neoplasm infiltrated the mucosa and was in close proximity to the superficial parts of the inner muscular layer.

Most cases of transient cell carcinoma (TCC) present with hematuria. Flank pain happens in 25% due to obstruction by the tumor or blood clots.
TCC tends to be multifocal and this is why the whole urinary tract must be scrutinized. In cases of TCC, synchronous lesions are detected in 39% and their incidence is almost the same for kidney and bladder. The probability of metachronous lesions of TCC is far greater for the bladder.
Intravenous urography (IVU) is used for the work-up of patients with hematuria and the diagnosis of TCC is frequently made so. This is changing as CT urography becomes better and multidetector CT more available. MR imaging is infrequently used in the primary assessment of TCC. Non-contrast MR urography can help in patients with poor renal function or those who can not tolerate iodinated contrast. Newer techniques with fast sequences have increased the use of MR.
Ureteral TCC is depicted as single or multiple intraluminal filling defects with proximal ureteral dilatation. Pelvicaliceal dilatation above a tumor or a stone will vary depending on the degree, duration of obstruction, as well as its location. Long-standing obstruction by a tumor causes massive hydronephrosis and poor excretory function, limiting the role of IVU. Pelvicaliceal obstruction with poor-functioning kidneys may obscure synchronous lesions in the distal ureter and retrograde pyelography (RP) can be performed to further assess these patients. RP is also useful in cases of and in allergic patients. During RP, TCC presents as a solitary, polypoid filling defect with dilatation of the ureter proximal to the lesion. The localized ureteral dilatation around and distal to the filling defect forms the goblet sign. The presence of the goblet sign is very helpful for diagnosing TCC. It represents a collection of contrast with a cup-like shape in the place of the intramural filling defect and is best depicted during RP. This sign is not characteristic of more acute causes of obstruction, like calculi and helps differentiate tumors from stones.
Polypoid intramural masses from urothelial carcinomas grow slowly, causing dilatation of the ureter from both ends of the lesion. Stones tend to cause a fast-developing mural edema, while the slow growth of the ureteral TCC allows the gradual dilatation of the ureteral lumen around the tumor. This is why tumors present infrequently with acute colicky pain. Tumors are also propulsed towards the bladder during peristalsis causing further dilatation of the peripheral ureter. This also plays a role in the formation of the goblet-like collection of contrast to the lower part of the lesion. Conversely, in cases of obstruction due to stones the ureteral lumen distal to the lesion will show evidence of stenosis due to spasm, edema or both.
Finally it must be noted that papillary TCC, as in this case is usually a low grade tumor. This means that it infiltrates and metastasizes slowly, with patients having a better overall prognosis.