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Grégory Verhoest md, Department of Urology, CHU Pont‘chaillou, Rue Henri Le Guilloux, 35033 Rennes Cedex, France. Email: email@example.com
Abstract Ectopic ureter is a rare abnormality, so presenting a transitional cell carcinoma (TCC) arising from an ectopic ureter is extremely rare. We report here a case of a man with an invasive transitional cell carcinoma arising from a right ectopic ureter and managed by laparoscopy. To our knowledge, this is the fourth case described in the literature, and the second case of a TCC arising in a right ectopic ureter.
Ectopic ureter is a rare congenital anomaly, more frequently observed in women and in the pediatric age group. In men, it most often opens in the vas deferens or the seminal vesicle. It is likely to be associated with a dysplastic kidney.1 Transitional cell carcinoma (TCC) arising from an ectopic ureter is extremely rare.
We report here a case of a man with an invasive transitional cell carcinoma arising from a right ectopic ureter opening in the prostatic urethra, close to the verumontanum.
To our knowledge, this is the fourth case described in the literature. All published cases arose in male patients.2–4
A 56-year-old man presented with a history of intermittent frank hematuria of 1-year duration. He admitted a small tobacco exposure that was stopped a long time ago. He was obese and had type 2 diabetes mellitus. He was known to have a single left functioning kidney. The clinical examination was unremarkable, and a digital rectal examination found a 20 g regular prostate.
Prostate-specific antigen (PSA) level was 1.41 ng/mL. Urinary culture was negative. Intravenous urogram revealed a tubular image receding from the verumontanum, and interpreted as an incidental opacification of one of the seminal vesicles. An abdominal computed tomography (CT) scan showed a normal left urinary system, and a right dysplastic kidney. Cystoscopy found an ectopic ureteral orifice, opening into the right side of the verumontanum. Retrograde pyelogram revealed multiple intraureteral lesions which were confirmed by a ureteroscopy. These included urinary calculi, and an area of papillary tumor. Biopsy revealed a pT1 G3 TCC. There was no metastasis at this time.
An ‘en bloc’ right nephroureterectomy combined with a radical prostatectomy was performed by laparoscopy. A transperitoneal approach was used. The same installation was used for the whole procedure. Four trochars of 5 mm, two of 10 mm and one of 12 mm were needed. It started with the dissection of the ureter from the prostate to the kidney. Right nephrectomy was first performed, then completed by the prostatectomy. Operation time was 6 h, and blood loss was only 200 mL. The tumor was classified as a pT4 N0 M0 tumor on histologic examination (Figs 1,2).
Nine months later, the patient complained of recurrent hematuria. Cystoscopy revealed multiple bladder tumors staged as pT1 G3. A radical cystectomy with ileal conduit urinary diversion was performed. Final pathological stage was pT4, with peritoneal carcinosis. Bone metastases were present on the nuclear scan. The patient died 18 months later.
Ectopic ureter is a rare abnormality. It is often left-sided and associated with dysplastic kidney. The ectopic site in men is often located in the seminal vesicle (37%) or posterior urethra (33%).1 Primary TCC of the ureter represents 1% of all malignant tumors of the upper urinary tract. It is usually diagnosed in men in the seventh or eighth decades. Proposed etiologies for ureteral TCC are similar to those for bladder tumors.5 We report here the second case of a TCC arising in a right ectopic ureter.2
Unlike other published reports, our case was managed purely by a laparoscopic approach. In a recent study, Schulze showed that compared to open surgery, the laparoscopic nephroureterectomy was associated with less blood loss, less analgesic requirement, quicker resumption of physical activity and shorter hospital stay.6 In the present case, the laparoscopic transperitoneal approach allowed us to perform the whole procedure including nephroureterectomy and radical prostatectomy by a single installation. By starting the dissection with the distal ureter it was easy to find the dysplastic kidney. This technique has also been described by other groups.7
The standard treatment of TCC is nephroureterectomy including excision of the ureteral orifice and the adjacent bladder, so that the risk of local recurrence is limited.7 Based on this principle we decided to perform a combined radical prostatectomy. In only one case of the literature, a combined radical prostatectomy was performed.4 Other authors performed a dissection limited to the apex of the prostate3 or the bladder neck.2 Despite this aggressive approach, our patient experienced an early tumor progression.
The main concern is that this unfavorable outcome could have been favored by laparoscopy. Although laparoscopic nephroureterectomy is associated to a decreased morbidity compared to open surgery,7 trochar site recurrences after laparoscopic treatment of urothelial tumors have been reported.8 However it must be noted that the histologic features in our case were far more aggressive compared to previous reports. The efficacy of laparoscopic radical nephroureterectomy for upper tract TCC has been well documented and a plastic bag for specimen retrieval was used avoiding contact between tumor and the abdominal wall or peritoneum.
Because of a lack of data concerning long-term follow up, the safety of laparoscopic management of urothelial malignancies remains, however, open to debate.