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Synchronous tumors in both ureters and left renal pelvis
SYNCHRONOUS TUMORS IN BOTH URETERS AND LEFT RENAL PELVIS N. J. MARUF.
h4.D.
C. J. GODEC. ‘4. KAHN,
h4.D.
M.D.
A. S. CASS. M.B..
B.S.
From the Urolog\r Division, Department of Surgeq: Hennepin County Medical Center. Pllinneapolis, Minnesota
ABSTRACT .- A case of bilateral. synchronous ureteral tumors and a tumor in the l+ renal pelcis i.v precerlted. Con.rervatiz;e surgical treatment inuolued local resection of both uretrral tumors and partial wphrectomy of the left kidney. Multiple urothelial tumors subsequently deoeloped in the bladder which u:erc resected. The patient has had good kidney function and no ez?idencc of disease.
Primaq. neoplasms of the ureter comprise less than 1 per cent of all genitourinary malignancies. and bilateral, synchronous ureteral or pelvic tumors are rarer.‘.” Patients with socalled Balkan nephropathy, howexrer, appear to have a higher frequenq. of bilateralit?. affecting about 10 per cent of these patients? The first case of bilateral pelvic tumors was reported by Sanford in 1931,g and the first case of bilateral, synchronous ureteral tumor was reported by Ratliff, Baum, and Butler in 1949.“’ Since then, numerous reports have been made of bilateral svnchronous as well as aqrnchronous ureteral*tun~ors.“.“-‘7 However. our case of bilateral. synchronous ureteral tumors Lvith an associated renal pelvic tumor appears to be the first of its kind. Case Report A sevjent!*-five-)-ear-old man \vas admitted \vith a four-day histor?. of gross hematuria, suprapubic tenderness, and dysuria accompanied by a sudden onset of nausea and vomiting. Prior to admi,ssion, treatment with propox!-phene napsylate (Darvocet) and sulfisoxazole (Gantrisin) brought no relief. Four years pre\piousl\; the patient had had an episode of gross hematuria u-hich \\.as treated with antibiotics. C!rstos-
copy and bilateral retrograde pyelograms, done at that time, gave normal findings. During the following three years, he had had three additional episodes of hematuria. Past medical histoqr also included a subtotal colectom!. for carcinoma of the colon twenty-five years ago, and a fortv-vear history of smoking 2 pack5 of cig_ _ arettes a day At admission, the patient had oliguria and leas mildl!. obese, General and systemic examinations did not reveal any significant findings. His hemoglobin was 12.2 Gm!dl; blood urea nitrogen (BUN) was 83 mgidl. creatinine 10.3 mgiml, and urine n-as grossly bloody. Urine c\rtology revealed atypical urothelial cells. Se;um protein electrophoresis relyealed ele\Tated levels of alpha-l and alpha-2 globulin. Renal blood flow studies sho\ved good blood flo\v to left kidney and poor blood flal\\. to the right one. The renogram displayed diminished function on the right side and bilateral obstruction. Ultrasound showed right hydronephrosis. Cystoscopy revealed a normal bladder. blood) efflux from the right ureteric orifice. and clear efflux from the left. Retrograde pJ,elograms sho\s.ed complete obstruction of the right midureter and a filling defect in left ureter at pelvic brim as \\rell as in the louver pole of the left kidne\. (Fig. 1).
Bilateral retrograde FIGURE 1. pole of left kidney (B). .’
pqelograms _ .
displayingfilling i
examination of all three FICUHE 2. Histologic specimens rez;ealed almost identical grade 111 transitional cell tumor with muscular invasion.
defects in both ureters (A and C). and in lozcer
Postoperatively the patient’s renal function and one month later his serum improved, creatinine was 1.8 mgldl and BUN was 24 mgi dl. Retrograde pyelogram, done after the last operation, showed a mild degree of narrowing of the right ureter but with bilaterally good drainage. Five months later, during routine cystoscopic examination, a bladder tumor was discovered and was resected transurethrally Light microscopy revealed grade III Stage A transitional cell carcinoma. Recurrent bladder tumors were resected two months later. Random bladder biopsies revealed only mild atypia. This patient has been free of tumors for the last thirteen months. Comment
After dialysis, surgical intervention confirmed the right midureter was grossly dilated with palpable tissue, and segmental resection of the right ureter with end to end anastomosis was performed. No enlarged lymph nodes were found, and the resected margins were free of tumor. Light microscopy revealed grade III transitional cell carcinoma with invasion of muscle layer. Tvvo weeks later, segmental resection of left ureter and left lower pole partial nephrectomy were performed. Light microscopy again revealed grade III transitional cell carcinoma with invasion of muscle layer (Fig. 2).
Transitional cell carcinoma is a generalized urothelial disorderI that accounts for about 7 per cent of all renal tumors.fi-s Nephroureterectom!; with excision of the bladder cuff, is the usual treatment for high-grade transitional cell tumors of renal pelvis and/or ureter. Conservative surgical management is indicated for such tumors in a solitary kidney, in multiple bilateral tumors, and in small, low-grade tumors in young patients. lgez2 In patients with bilateral ureteral transitional cell tumors, conservative treatment with segmental resection of the ureter with tumorfree ureteric margins and reanastomosis can be
performed. If the tumor is located in the lower one third of the ureter, partial ureterectomy, including resection of ureterovesical junction and reimplantation with a bladder flap or hitchlg may be suggested. Aggressive surgery can be considered. Bilateral total ureterectomy and “high diversion” with anastomosis of renal pelvis to ileum reduces the target organ (urothelium) and allows endoscopic resection or fulguration of recurrent tumors, similar to the treatment of patients with struvite stones which can be extracted endoscopically.23 Petterson et ~1.~~ suggested total ureterectomy and autotransplantation with calicovesicostomy which allows recurrent tumors of the renal pelvis to be controlled endoscopically by transurethral resection or fulguration. Complete excision of the ureter and renal pelvis and substitution by an ileal segment has been used to remove as much of the urothelium as possible.24.25 Partial nephrectomy of the tumor-bearing area can be performed in cases of bilateral renal pelvic tumors or tumor in solitary kidney. In up to 30 per cent of the patients with ureteral tumors bladder tumors subsequently have developed within an average of two years.22~2e Bladder tumors developed in our patient within five months, possibly due to his relatively highgrade ureteral cancer.
3. Petkovic SD: Treatment of bilateral renal pelvic and ureteral tumors. Eur Ural 4: 397 (1978). 4. Petkovic S, Mutavolzic M‘, Petiovic V, and Markovic V: Xlmars of the renal pelvis and ureter. clinical and etiologic studies. Br J Urol 44: 1 (1972). 5. Hall P, and Dammin G: Balkan nephropath!. Nephron 22: 281 (1968). 6. Rubenstein M, Walz B, and Bucv I: Transitional cell carcinoma of the kidney, 25-year experiende,.J llrol 119: 596 (1978). 7. Hunt VC: Pauillarv enithehoma of the renal nelvis. ibid 18: 225 (1927). _ ’ A 8. Thomas GH, and Regnier EA: Tumors of the kidney. pelvis and ureter, ibid 11: 205 (1924). 9. Sanford H: Carcinoma of both kidneys, Surq Gynecol Obstet 53: 360 (1931). 10. Ratliff R, Baum W, and Butler W: Bilateral primary carcinoma of the ureter, Cancer 2: 815 (1949). 11. Babaian R, and Johnson D: Primar!. carcinosma of ureter, J Urol 123: 357 (1980). 12. McIntyre D, Pyrah LN, and Raper FP: Primary ureteric neoplasms, Br J Ural 37: 160 (1965). 13. Viek N, Uhlman R, and Verrilli R: Simultaneous bilateral transitional cell carcinoma of ureters, J Ural 89: 49 (1963). 14. Gracia V, and Bradfield E: Simultaneous bilateral transitional cell carcinoma of the ureter, ihid 79: 925 (1958). 15. Carroll G: Bilateral transitional cell carcinoma of renal pelvis, ibid 93: 132 (1965). 16. Gillenwater J, Howard R, and Paquin A: Bilateral primary carcinoma of the ureter, JAMA 197: 210 (1966). 17. Colston JAC, and Arcadi J: Bilateral renal papillomas, J Ural 73: 460 (1955). 18. Batata M, et al: Primary carcinoma of the ureter, Cancer 35: 1626 (1975). 19. Brown HE, and Roumani GK: Conservative surgical management of transitional cell carcinoma of upper urinary tract, J Ural 112: 184 (1974). 20. Petterson S, Brynger H, Johansson S. and Nilson A: Extracorporeal surgery and autotransplantation for carcinoma of the pelvis and ureter, Stand J Urol Nephrol 13: 89 (1979). 21. Chazi M, Morales P, and Al-Askari S: Primary carcinoma of ureter, Urology 14: 18 (1979). 22. Bloom N. Vidone R, and Lytton B: Primary carcinoma of ureter, case report of 102 new cases, J Urol 103: 590 (1970). 23. Godec CJ, and Cass AS: Endoscopic removal of staghorn calculi in patients with urinary diversion, Urology 19: 529 (1980). 24. Pyrah LN, and Raper FP: Some use of an isolated loop of ilium in genitourinary surgery, Br J Surg 42: 22 (195.5). 25. Crassweller PO: Bilateral primary carcinoma of ureter with use of ileal graft for ureteral replacement. Br J Ural 30: 152 (1958). 26. Batata M, and Grabstald H: Upper urinary tract urothelial tumors, Urol Clin North Am 3: 79 (1976).
701 Park Avenue South Minneapolis, Minnesota 55415 (DR. GODEC) References 1. Foord AG. and Ferrier PA: Primary carcinoma of ureter with report of seven cases, JAMA 112: 596 (1939). 2. Abeshouse BS: Primary benign and malignant tumors of the ureter, review of literature and report of one benign and 12 malignant tumors. Am J Surg 91: 237 (1956).
UROLOGY
MARCII
1983
VOLUME
XXI, NUMBER
3
h4.D.
C. J. GODEC. ‘4. KAHN,
h4.D.
M.D.
A. S. CASS. M.B..
B.S.
From the Urolog\r Division, Department of Surgeq: Hennepin County Medical Center. Pllinneapolis, Minnesota
ABSTRACT .- A case of bilateral. synchronous ureteral tumors and a tumor in the l+ renal pelcis i.v precerlted. Con.rervatiz;e surgical treatment inuolued local resection of both uretrral tumors and partial wphrectomy of the left kidney. Multiple urothelial tumors subsequently deoeloped in the bladder which u:erc resected. The patient has had good kidney function and no ez?idencc of disease.
Primaq. neoplasms of the ureter comprise less than 1 per cent of all genitourinary malignancies. and bilateral, synchronous ureteral or pelvic tumors are rarer.‘.” Patients with socalled Balkan nephropathy, howexrer, appear to have a higher frequenq. of bilateralit?. affecting about 10 per cent of these patients? The first case of bilateral pelvic tumors was reported by Sanford in 1931,g and the first case of bilateral, synchronous ureteral tumor was reported by Ratliff, Baum, and Butler in 1949.“’ Since then, numerous reports have been made of bilateral svnchronous as well as aqrnchronous ureteral*tun~ors.“.“-‘7 However. our case of bilateral. synchronous ureteral tumors Lvith an associated renal pelvic tumor appears to be the first of its kind. Case Report A sevjent!*-five-)-ear-old man \vas admitted \vith a four-day histor?. of gross hematuria, suprapubic tenderness, and dysuria accompanied by a sudden onset of nausea and vomiting. Prior to admi,ssion, treatment with propox!-phene napsylate (Darvocet) and sulfisoxazole (Gantrisin) brought no relief. Four years pre\piousl\; the patient had had an episode of gross hematuria u-hich \\.as treated with antibiotics. C!rstos-
copy and bilateral retrograde pyelograms, done at that time, gave normal findings. During the following three years, he had had three additional episodes of hematuria. Past medical histoqr also included a subtotal colectom!. for carcinoma of the colon twenty-five years ago, and a fortv-vear history of smoking 2 pack5 of cig_ _ arettes a day At admission, the patient had oliguria and leas mildl!. obese, General and systemic examinations did not reveal any significant findings. His hemoglobin was 12.2 Gm!dl; blood urea nitrogen (BUN) was 83 mgidl. creatinine 10.3 mgiml, and urine n-as grossly bloody. Urine c\rtology revealed atypical urothelial cells. Se;um protein electrophoresis relyealed ele\Tated levels of alpha-l and alpha-2 globulin. Renal blood flow studies sho\ved good blood flo\v to left kidney and poor blood flal\\. to the right one. The renogram displayed diminished function on the right side and bilateral obstruction. Ultrasound showed right hydronephrosis. Cystoscopy revealed a normal bladder. blood) efflux from the right ureteric orifice. and clear efflux from the left. Retrograde pJ,elograms sho\s.ed complete obstruction of the right midureter and a filling defect in left ureter at pelvic brim as \\rell as in the louver pole of the left kidne\. (Fig. 1).
Bilateral retrograde FIGURE 1. pole of left kidney (B). .’
pqelograms _ .
displayingfilling i
examination of all three FICUHE 2. Histologic specimens rez;ealed almost identical grade 111 transitional cell tumor with muscular invasion.
defects in both ureters (A and C). and in lozcer
Postoperatively the patient’s renal function and one month later his serum improved, creatinine was 1.8 mgldl and BUN was 24 mgi dl. Retrograde pyelogram, done after the last operation, showed a mild degree of narrowing of the right ureter but with bilaterally good drainage. Five months later, during routine cystoscopic examination, a bladder tumor was discovered and was resected transurethrally Light microscopy revealed grade III Stage A transitional cell carcinoma. Recurrent bladder tumors were resected two months later. Random bladder biopsies revealed only mild atypia. This patient has been free of tumors for the last thirteen months. Comment
After dialysis, surgical intervention confirmed the right midureter was grossly dilated with palpable tissue, and segmental resection of the right ureter with end to end anastomosis was performed. No enlarged lymph nodes were found, and the resected margins were free of tumor. Light microscopy revealed grade III transitional cell carcinoma with invasion of muscle layer. Tvvo weeks later, segmental resection of left ureter and left lower pole partial nephrectomy were performed. Light microscopy again revealed grade III transitional cell carcinoma with invasion of muscle layer (Fig. 2).
Transitional cell carcinoma is a generalized urothelial disorderI that accounts for about 7 per cent of all renal tumors.fi-s Nephroureterectom!; with excision of the bladder cuff, is the usual treatment for high-grade transitional cell tumors of renal pelvis and/or ureter. Conservative surgical management is indicated for such tumors in a solitary kidney, in multiple bilateral tumors, and in small, low-grade tumors in young patients. lgez2 In patients with bilateral ureteral transitional cell tumors, conservative treatment with segmental resection of the ureter with tumorfree ureteric margins and reanastomosis can be
performed. If the tumor is located in the lower one third of the ureter, partial ureterectomy, including resection of ureterovesical junction and reimplantation with a bladder flap or hitchlg may be suggested. Aggressive surgery can be considered. Bilateral total ureterectomy and “high diversion” with anastomosis of renal pelvis to ileum reduces the target organ (urothelium) and allows endoscopic resection or fulguration of recurrent tumors, similar to the treatment of patients with struvite stones which can be extracted endoscopically.23 Petterson et ~1.~~ suggested total ureterectomy and autotransplantation with calicovesicostomy which allows recurrent tumors of the renal pelvis to be controlled endoscopically by transurethral resection or fulguration. Complete excision of the ureter and renal pelvis and substitution by an ileal segment has been used to remove as much of the urothelium as possible.24.25 Partial nephrectomy of the tumor-bearing area can be performed in cases of bilateral renal pelvic tumors or tumor in solitary kidney. In up to 30 per cent of the patients with ureteral tumors bladder tumors subsequently have developed within an average of two years.22~2e Bladder tumors developed in our patient within five months, possibly due to his relatively highgrade ureteral cancer.
3. Petkovic SD: Treatment of bilateral renal pelvic and ureteral tumors. Eur Ural 4: 397 (1978). 4. Petkovic S, Mutavolzic M‘, Petiovic V, and Markovic V: Xlmars of the renal pelvis and ureter. clinical and etiologic studies. Br J Urol 44: 1 (1972). 5. Hall P, and Dammin G: Balkan nephropath!. Nephron 22: 281 (1968). 6. Rubenstein M, Walz B, and Bucv I: Transitional cell carcinoma of the kidney, 25-year experiende,.J llrol 119: 596 (1978). 7. Hunt VC: Pauillarv enithehoma of the renal nelvis. ibid 18: 225 (1927). _ ’ A 8. Thomas GH, and Regnier EA: Tumors of the kidney. pelvis and ureter, ibid 11: 205 (1924). 9. Sanford H: Carcinoma of both kidneys, Surq Gynecol Obstet 53: 360 (1931). 10. Ratliff R, Baum W, and Butler W: Bilateral primary carcinoma of the ureter, Cancer 2: 815 (1949). 11. Babaian R, and Johnson D: Primar!. carcinosma of ureter, J Urol 123: 357 (1980). 12. McIntyre D, Pyrah LN, and Raper FP: Primary ureteric neoplasms, Br J Ural 37: 160 (1965). 13. Viek N, Uhlman R, and Verrilli R: Simultaneous bilateral transitional cell carcinoma of ureters, J Ural 89: 49 (1963). 14. Gracia V, and Bradfield E: Simultaneous bilateral transitional cell carcinoma of the ureter, ihid 79: 925 (1958). 15. Carroll G: Bilateral transitional cell carcinoma of renal pelvis, ibid 93: 132 (1965). 16. Gillenwater J, Howard R, and Paquin A: Bilateral primary carcinoma of the ureter, JAMA 197: 210 (1966). 17. Colston JAC, and Arcadi J: Bilateral renal papillomas, J Ural 73: 460 (1955). 18. Batata M, et al: Primary carcinoma of the ureter, Cancer 35: 1626 (1975). 19. Brown HE, and Roumani GK: Conservative surgical management of transitional cell carcinoma of upper urinary tract, J Ural 112: 184 (1974). 20. Petterson S, Brynger H, Johansson S. and Nilson A: Extracorporeal surgery and autotransplantation for carcinoma of the pelvis and ureter, Stand J Urol Nephrol 13: 89 (1979). 21. Chazi M, Morales P, and Al-Askari S: Primary carcinoma of ureter, Urology 14: 18 (1979). 22. Bloom N. Vidone R, and Lytton B: Primary carcinoma of ureter, case report of 102 new cases, J Urol 103: 590 (1970). 23. Godec CJ, and Cass AS: Endoscopic removal of staghorn calculi in patients with urinary diversion, Urology 19: 529 (1980). 24. Pyrah LN, and Raper FP: Some use of an isolated loop of ilium in genitourinary surgery, Br J Surg 42: 22 (195.5). 25. Crassweller PO: Bilateral primary carcinoma of ureter with use of ileal graft for ureteral replacement. Br J Ural 30: 152 (1958). 26. Batata M, and Grabstald H: Upper urinary tract urothelial tumors, Urol Clin North Am 3: 79 (1976).
701 Park Avenue South Minneapolis, Minnesota 55415 (DR. GODEC) References 1. Foord AG. and Ferrier PA: Primary carcinoma of ureter with report of seven cases, JAMA 112: 596 (1939). 2. Abeshouse BS: Primary benign and malignant tumors of the ureter, review of literature and report of one benign and 12 malignant tumors. Am J Surg 91: 237 (1956).
UROLOGY
MARCII
1983
VOLUME
XXI, NUMBER
3