Upper tract urothelial carcinoma arising in a horseshoe kidney presents unique treatment challenges. The patient underwent right heminephrectomy for a T1 low-grade tumor, with subsequent recurrences in the bladder and ureter. Close follow-up is important given the high risk of recurrence in UTUC, particularly in the bladder due to field cancerization effects. While adjuvant therapies have unclear benefits, intravesical BCG may help prevent recurrence in selected cases. Complete surgical resection remains the mainstay of treatment for UTUC in horseshoe kidneys.

1. Upper tract urothelial
cancer in horse shoe
kidney
Dr Anil Gupta
Jr/ dept of radiotherapy
PGIMER, chandigarh

2.  60 yr male, presented with painless gross hematuria since 2 months. Not a/w
clots, flank pain, suprapubic pain, LUTS
 Known chronic smoker
 GPE- GC good, no pallor/icterus/clubbing/cyanosis, generalized edema
 USG KUB s/o right renal mass
 CECT abdomen/pelvis (feb 5 2016) # 457/16 – horse shoe shaped kidney with
mild HDN right side. Heterogenously enhancing mass 37*55 mm in upper pole
of rt kidney
 HMG(13 apr 2016)- 12.4/14600/151000
 RFT (13 apr 2016) – 30/1.5

4.  Pt underwent right open NSS  Rt heminephrectomy
 I/O findings - rt 5*3 cm renal hilar mass, on excision of mass, tumor seen
arising from pelvis  Frozen section s/o urothelial carcinoma.
 Rt heminephrectomy done
 Post op period – uneventful except pt had vertigo and ENT examination s/o
CSOM
 HPR- S-9001/16 s/o low grade urothelial carcinoma? T1

5.  CPE 4/8/16 – bladder mucosa healthy, no tumor recurrence, passable stricture
in mid bulb
 CECT + CT urography 8 feb 17- poorly excreting residual Rt moiety with Rt
side HDN. Liver- irregular hypodense lesion 9*7 mm in segment VII/VIII of
liver? Mets
dilated mid/lower ureter with mild eccentric mural thickening and
enhancement? Recurrence

6.  Usg guide FNAC for liver lesion- not amenable ,USG features s/o hemangioma
 Underwent CPE on 22 feb 17-s/o 4*5 cm solid appearing growth @ rt ureteric orifice, severe
trabeculations present imp: bladder growth
 Lost to follow up
 Pt underwent TURBT on 9 may 17
Same growth seen
HPR- S-12374/17s/o T1 high grade, detrusor- free of tumor
 Pt had c/o hematuria on 11 june 17

7.  CECT abd/pelvis 8 July 17 – remnant right moiety with dilated mid and distal ureter with soft
tissue lesion in the Rt VUJ
 On 21 jul 17 pt underwent TURBT + open uretectomy + bladder cuff excision
 HPR S-19867/17
Ureter
Bladder
Urethra
Urothelial neoplasm,
low grade, lamina
propria invasion is not

8. Points of Discussion
 Treatment approach in upper tract urothelial carcinoma (UTUC)
 Adjuvant treatment in UTUC
 Treatment approach in horse shoe shaped kidney
 Pattern of relapse
 Treatment approach in case of relapse

9. Introduction
 Horseshoe kidney occurs in 1/400 or 0.25% of the population
 Adenocarcinoma comprises about 50% of tumors arising in the
horseshoe kidney, followed by transitional cell carcinoma and
Wilms tumor
 Transitional cell carcinoma of the renal pelvis is an uncommon
urologic lesion with an incidence of less than 5/100,000.
 Renal pelvic urothelial carcinoma with the horseshoe kidney is an
0.0025% or 2.1 cases/IO,OOO,OOO )
 The incidence of renal pelvic urothelial carcinoma in a horseshoe
kidney is approximately 3 to 4 times that in a normal kidney,

10.  Possibly due to
- chronic obstruction
- lithiasis
- infection
 The incidence of hydronephrosis with a horseshoe kidney range from 21 to 45%, but including
mild hydronephrosis, the incidence may be higher
 Seventy percent of horseshoe kidneys have vascular malformations
 Surgery of the horseshoe kidney is dangerous, because the frequency of anomalous vascular
supply of the horseshoe kidney is high and the isthmus consists of parenchyma
stasis Chronic
irritation
renal pelvic
urothelial
carcinoma/
squamous cell
carcinoma

11.  The gold standard of treatment for upper GUT is complete
nephroureterectomy with removal of a cuff of urinary bladder.
 It is important to complete the surgery due to the high rate of ureteral stump
recurrence, which has been reported to be between 30% and 75%

12. FIELD CANCERIZATION
 Multifocality of urothelial TCC based on two concepts-
1. Field Cancerization - Whole urothelium of GUT exposed to same urinary
carcinogens lead to transformation of many independent & separate
urothelial cells in multiple sites.
2. Intraepithelial migration – Single carcinogenic insult to a single group of
cells forming clonal cells which spread throughout bladder via
intraepithelial migration, cell shredding & re implantation.
 These events lead to multiple synchronous & metachronous tumours.

13. How to approach upper tract urothelial
carcinoma (UTUC)
 History
 Clinical examination
 Retrograde pyelography
 CT urogram (CTU) and MR urogram (MRU) to detect lesions of the
upper tracts to see multifocality
 Flexible ureteroscopy visualise and biopsy the ureter, renal pelvis, and
collecting system CT scans and MR imaging to stage nodal disease
 Bone scan for bony mets

14. 2013
???

15.  Can be used, selecting patients for preoperative systemic therapy and guiding
the extent of concomitant lymph node dissection at nephroureterectomy.
LND appears to be unnecessary in cases of Ta/T1 UTUCs
(found only in 2.2% of T1)

16. EUA Guidelines 2015

18. No Trials for adjuvant treatment in T1/ low grade

19. Adjuvant Chemotherapy
Median survival in the group with chemotherapy was 24 months and in the group
without chemotherapy it was 26 months
5 of the 16 patients (31%) in the adjuvant group and 4 of the 11 patients (36%) in the
nonadjuvant group had experienced a recurrence of their cancer after 40 months of
follow-up.
Recurrence was observed in 12 patients (37.5%) who underwent chemotherapy and in 7
patients (63.6%) who did not.

20. The theoretical advantages of neoadjuvant chemotherapy includes-
 The eradication of subclinical metastatic disease
 Improved patient tolerability prior to surgical extirpation
 The ability to deliver higher chemotherapy doses (due to the loss of
 renal function that occurs with nephroureterectomy).

21.  No evidence level 1 information from prospective randomized trials
 Due to many similarities with bladder carcinomas, chemotherapy with a
cisplatin-containing regimen is often proposed in metastatic or locally
advanced disease.
 Not all patients can receive chemotherapy because of comorbidity and
impaired renal function after radical surgery
 Most teams have proposed a neoadjuvant treatment based either on the
combination of methotrexate, vinblastine,adriamycin, and cisplatin (MVAC) or
on gemcitabine/cisplatin (GC)
 Recent studies have included limited numbers of patients and have reported
poor patient outcomes after both neoadjuvant and adjuvant chemotherapy
No randomized trials till
now

22. Adjuvant Radiotherapy
No role of adjuvant RT or chemo RT

23. Five-year actuarial survival was 44% with and 24% without adjuvant radiation therapy in
patients high grade or stage, close margins, or positive nodes, local control can be improved
with adjuvant radiation. Improvement in survival is of borderline significance no change in
OS, distant mets
Median survival of the irradiated and nonirradiated patients was 35 and 26 months
Only 1 out of 8 had persistent local disease, patient tolerated radiotherapy
well.
40 to 60 Gy

24. 133 patients with TCC of the
renal pelvis or ureter
67 patients received post op
RT
66 patients received
intravesical chemotherapy
Overall survival
At 1 yr- 98.8%
3 yr- 61.1%
5 yr- 49.6%
Median – 55 mnths
T3/T4 median- 29.9
mnths
Overall survival
At 1 yr- 75.5%
3 yr- 53.6%
5 yr- 44.7%
Median- 52.4 mnths
T3/T4 median- 11.4 mnths
Reduced the local recurrence for all patients and
improved the
overall survival for those patients with T3/T4
The clinical target volume included
the renal fossa, the course of the
ureter to the entire bladder, and
the paracaval and para-aortic
lymph nodes, which were at risk of
harbouring metastatsis

25.  Established the safety of BCG as an adjunct to “definitive” upper tract tumor
resection, even in patients with solitary kidneys
 Gave general guidelines for use ;-
-Interval of approximately 2 weeks from the time of tumor resection
-Radiographic confirmation of a nonobstructed, nonextravasating system at the
start of treatment and at regular intervals during subsequent infusions.

26. Immunotherapy
 In 16 of 18 treated renal units definitive percutaneous resection of the tumor was followed by 6
weekly percutaneous installations of BCG
 Of the 17 patients only 1 (5.9%) with high grade (3/3), invasive (pT2) primary tumor at initial
resection died of locally persistent or recurrent disease.

27.  Case report of 2 superficial renal pelvic TCC recurrence
 Both cases had recurrence as grade 3 non invasive TCC
 BCG installations were initiated 8 weeks later with 6 weekly course of 1 amp
BCG dissolved in 50 ml NS infused through nephrostomy tube for 2 hrs
 48 months of follow up showed no recurrence

31.  Presented with h/o multiple e/o of gross painless hematuria
 CECT s/o well defined , anteriorly situated renal pelvic mass
 Underwent right nephrouretectomy with bladder cuff excision en bloc
 Histology showed TCC, grade II invading in superficial lamina propria
 Three monthly follow up done, eighteen months of follow up showed no signs
of recurrence
 Suggested that TCC in horseshoe kidney has to be treated similar to UTUC

32.  40 y/ M presented with intermittent painless gross haematuria
 Cystoscopy showed a flow of gross hematuria from the left orifice
 Retrograde pyelography, CECT abd, MRI revealed a renal pelvic mass in the upper pole of left
kidney
 Left nephroureterectomy and isthmusectomy and bladder cuff excision en bloc were done
 Histology showed a grade 1, pTa, urothelial carcinoma on the left renal calix.
 The patient was clinically well 4 months after surgery

33.  A 62-year-old man suffering from gross hematuria. CECT abd s/o a horseshoe
kidney and renal pelvic tumor in the left kidney.
 Open nephrectomy with division of the isthmus was performed.
 The pathological diagnosis was urothelial carcinoma, grade 2, pT3.
 He is free of disease at 13 months after operation.
 In Japan, this is the 33th case of renal pelvic tumor in a horseshoe kidney.

34.  A 71 yr/F with loss of appetite
 Imaging revealed a large, cystically dilated left kidney with a solid tumor inside the cavity and right
hydronephrosis.
 A chest X-ray revealed multiple metastatic lesions.
 A horseshoe kidney was found intraoperatively and left nephroureterectomy with partial cystectomy was
performed
 Histological diagnosis was poorly differentiated transitional cell carcinoma. She died of progressive
pulmonary metastases 2 weeks after operation.
 This is the 19th case of a renal pelvic tumor associated with a horseshoe kidney reported in the Japanese
literature.
 The diagnosis was confounded by the extreme dilation and deformity of the hydronephrotic kidney.

37. Follow Up

38. RELAPSE CASES
 Relapse can occur as local failure/locally advanced disease or distant
metastasis.
 Nephroureterectomy were more likely to suffer a local relapse (46%) than
those who underwent complete nephroureterectomy (15%)
 Most common recurrence is found in urinary bladder, about 22-47%
 Bladder recurrence should not be considered as distant recurrence

39.  Patients with resected locally advanced (T3, T4N0, N+) TCC of the have a high
risk of relapse and death from disease despite postoperative radiotherapy.

40. Management of relapse in bladder
BCG therapy is given once a week for 6 weeks, followed
by a rest period of 4 to 6 weeks, with a full re-evaluation
at week 12 (ie, 3 months) after the start of therapy

41.  UTUC has long been considered the twin tumor of UCB
 Several researches showed it to be distinct from UCB
 These are less frequent than UCB, more invasive at diagnosis
 In high-risk tumors, radical nephroureterectomy (RNU) remains the standard
 Post-RNU intravesical instillation has been shown to decrease bladder cancer
recurrence rates
 The role of neoadjuvant cisplatin based chemotherapy and lymphadenectomy are
not clearly established, evidence suggests a survival benefit to these
 There is currently no evidence for adjuvant chemotherapy (AC) in UTUC
2015