Multifocal transitional cell carcinoma with synchronous involvement of bilateral upper urinary tracts is rare. We present one such case presenting with haematuria and renal insufficiency.
Authors: Gupta, Narmada; Nayyar, Rishi E-mails: [email protected], [email protected]
Corresponding Author: Dr Shailesh Chandra Sahay, MBBS, MS ( General Surgery), MCh (Urology) Corresponding address: Room No 5030, Department of Urology, All India Institute of Medical Sciences, Ansari Nagar, Post: New Delhi, Pin: 110029, India Email: [email protected]
Multifocal transitional cell carcinoma with synchronous involvement of bilateral upper urinary tracts is rare. We present one such case presenting with haematuria and renal insufficiency. The challenges in diagnosis and surgical management of such a case are highlighted and various available treatment options are discussed. Endourological techniques through retrograde and percutaneous access for the right side, along with left robotic nephroureterectomy and cuff of bladder excision, provided excellent peri-operative outcomes in this patient.
Patients presenting with bilateral synchronous upper tract transitional cell carcinoma have always been a challenge for the urologist. This case describes a patient with bilateral upper tract transitional cell carcinoma who was managed by a combination of upper and lower tract endourological and minimal invasive approaches.
A 57 year oldmale presented with painless gross haematuria for 1 month. He was a chronic smoker and had controlled diabetes mellitus for 4 years. Blood investigations revealed hemoglobin-11.1gm/dl, urea-43mg/dl and creatinine-2.1mg/dl. Liver function tests were normal. Ultrasound abdomen showed right renal and left vesicoureteric junction mass with hydroureteronephrosis. Chest X ray was normal. Renal dynamic scan revealed global glomerular filtration rate (GFR) of 27.58ml/min and differential function of 89% and 11% on right and left side respectively. MRI confirmed the bilateral localized tumours (Figure 1).
Figure 1. Coronal section of MRI Abdomen showing right renal pelvic tumour (3.7×2.2cm) and left lower ureteric tumour (3.1×2.7cm) (arrows) with hydroureteronephrosis and small kidney.
An informed consent was taken for cystoscopy, right retrograde pyelography (RGP) and ureteroscopy (Figure 2). Cystoscopy showed tumour protruding out from the left ureteric orifice. Biopsies were taken from the tumour and bladder trigone. Right RGP showed filling defect in the pelvis and inferior calyx. Ureteroscopic biopsy was taken from the tumour and a double-J stent was placed. Histopathology revealed papillary neoplasm of low malignant potential (PUNLMP) on both sides. Biopsy from bladder trigone was normal.
With diagnosis of PUNLMP and bilateral clinical stage-T1, an endourological approach was chosen for treating the right side first. Percutaneous infracostal superior calyceal puncture was used for resection/ fulguration with holmium laser. A nephrostomy tube was kept for subsequent re-look procedures. Nephrostomy was clamped after 3 hours and intravesical Mitomycin-C 40mg given in right lateral and Trendelenberg position (dwelling time-90min). Histopathology showed low grade transitional cell carcinoma (TCC).
For the left side, robotic nephroureterectomy was done after 3 days. Left flank position was used for nephroureterectomy and regional lymphadenectomy. Robot was then de-docked and patient’s position changed to steep Trendelenberg for distal ureterectomy, cuff of bladder excision and pelvic lymphadenectomy parts of the procedure. Figure 2 shows the port positions used in this case. Specimen was retrieved in an ‘endocatch bag’ by extending the left side 8mm robotic port. Blood loss was 100cc. Patient was allowed oral intake after 24 hours. Urethral catheter was kept for 10 days. Histopathology revealed multiple low grade TCCs in the kidney and ureter, with free margins. All lymph nodes were also free.
Figure 2. (A) Right retrograde pyelography (RGP) showing filing defect in inferior calyx (B) Ureteroscopic view while taking biopsy with a dormia basket (C) Percutaneous nephroscopic view showing superficial tumour involving the inferior calyx (D) Resected tissue on nephroscopy (E) Port position for left robotic nephrourecterectomy with cuff of bladder excision. 8 mm ports were used for robotic arms while 12 mm port was used for assistance (F) Resected specimen showing multiple tumours (arrows) (G) Right RGP at 1 year follow up showing no filling defect
A re-look right nephroscopic resection/ fulguration was done after 2 weeks. Mitomycin C was given through the nephrostomy after 4 hours in Trendelenberg position. Total hospital stay was 27 days. He was discharged with stent and nephrostomy tube. Hemoglobin was 10.2gm/dl, urea-56mg/dl, and creatinine-1.8mg/dl.
Subsequently, induction course of Mitomycin C (6 weekly doses) was given through the nephrostomy. Check cystoscopy and nephroscopy at 3 months were normal. Biopsy from previous resection site was normal. The double-J stent was removed during cystoscopy and nephostomy tube was clamped to be removed after 24 hours. Follow up cystoscopies at 6, 9 and 12 months were normal, without any evidence of tumour. CT scan abdomen and pelvis done at 1 year showed no evidence of tumour. Renal dynamic scan at 1 year showed non obstructed solitary right kidney with GFR improved from 24.53 ml/min to 30.4 ml/ min after operation.
Upper tract TCC constitutes about 5% of urothelial malignancies. Although it is associated with ‘field change’ and is frequently multifocal (44%)1, synchronous bilateral involvement is rare, constituting only 1% of all upper tract TCC2. No specific guidelines for management have so far been formulated, given the rarity of disease.
The most common presenting symptom is haematuria. A large number of cases (>50%) present with renal failure3 not related to obstructive uropathy. Exposure to some nephrotoxic/ carcinogenic compound has been proposed as a hypothesis to the origin of TCC along with renal dysfunction4. Renal failure seems to be a very poor prognostic factor. One series of 10 cases with bilateral synchronous TCC reported all 7 cases with compromised renal function to die within 36 months of diagnosis, despite radical or conservative surgery combined with maintenance dialysis3.
There are several treatment options for managing localized upper tract TCC with nephroureterectomy and cuff of bladder excision being the standard treatment, in view of high recurrence rates and thin muscle coat in the upper tract. This may be done by open, laparoscopic or robotic approach. However in presence of severe comorbidities precluding extensive surgery or patients with renal insufficiency, solitary kidney or synchronous bilateral disease endoscopic management through antegrade or retrograde access provides an acceptable alternative without rendering the patient anephric. Recent advances in endoscopic techniques have further improved their efficacy. Ureteroscopy is preferred for solitary, small (<1.5cm) tumours in accessible portions of the upper tract. Other cases require percutaneous access5. Multiple staged procedures may be required to ensure complete resection. Unfortunately, recurrence is reported to occur in majority of such patients with imperative indications for conservative management6. For bilateral large, high grade or high stage disease (T2 or above, N0, M0) bilateral radical nephroureterectomy with dialysis or transplant may still provide the best survival possibility7,8,4.
Very few cases of total robotic radical nephroureterectomy have been reported in world literature. It is limited by the need of dedocking the robot and repositioning the patient for the pelvic part of the surgery. However as we have shown in this case, it is feasible and effective with little increase in total operative time.
Other management options include partial nephrectomy and renal autotransplantation. Partial nephrectomy is useful for solitary renal units with localized disease where negative resection margin can be achieved without compromising the vascular supply. Renal autotransplantation with pyeloneocystostomy provides a good alternative for cases with multiple ureteric tumours. It preserves the kidney and also provides easy access for subsequent follow up procedures. However, it requires expertise in transplant surgery.
The role of adjunctive therapy is not very clear in management of upper tract TCC. A large recent retrospective review has shown no overall benefit with adjuvant induction course of BCG with regards to disease recurrence, interval to recurrence, and progression of disease9.
The compliance of such patients should be strictly ensured. They should be counseled extensively to undergo multiple interventions over a short period and to rigorously pursue the protocols for adjunctive therapy, radiological, cytological and endoscopic follow up. The risk of recurrence persists even at 5-10 years, so surveillance should be continued for 10 years and perhaps longer1. No standardized surveillance protocol for upper tract TCC after endoscopic management has been established. We follow such cases with urinary cytology, microscopy, CECT and endoscopic evaluation at 3 monthly intervals for the first 2 years and half yearly thereafter. Associated renal insufficiency, high grade and stage of disease, and positive lymph node status are known poor prognostic factors.
The case was unique in view of bilateral synchronous transitional cell carcinoma with extensive involvement. Successful management was done using endourological and robotic approaches.
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Date added to bjui.org: 16/11/2010