Bilateral Ureteral and Vesical Carcinomas Occurring Asynchronously: Report of a Case

THE JoURNA.r~ OF UROLOGY

Yol. 88, No. 4 October 1962 Copyright © 1962 by The Williams & Wilkins Co.

Printed in U.S.A.

BILATERAL URETERAL AND VESICAL CARCINOMAS ASYNCHRONOUSLY: REPORT OF A CASE DAVID C. UTZ, CARL D. BRUNSTING

AND

OCCURRING

EDGAR G. HARRISON", JR.

From the Mayo Clinic and Mayo Foundation, Rochester, Minn.

Since carcinoma in the renal collecting system, ureters and bladder occurs commonly at multiple sites, one might expect to see a moderate number of bilateral ureteral tumors. As a matter of fact, primary bilateral ureteral cancer is a pathologic oddity, only a few cases having been reported. No doubt more have been seen and others have gone unrecognized. Secondary or metastatic involvement of both ureters is not so rare, but this usually is a necropsy finding in cases of carcinomatosis. REVIEW

Wising and Blix1 published in 1878 the first report of bilateral ureteral carcinoma proved at necropsy. Chauvin and Cerati2 in 1931 mentioned two such cases without further comment. Ratliff, Baum and Butler3 documented the first clinically determined case in recent times, that of a 60-year-old man who presented with bilateral hydronephrosis and anuria. After preliminary right nephrostomy, the right ureter was explored and two malignant neoplasms were excised. One month later the non-functioning left kidney was removed, the left ureteral tumor being found unexpectedly. Two months later the patient felt good and apparently was free of recurrence. In 1953 Felber 4 reported the case of a woman whose bilateral ureteral tumors occurred 8 years apart. These neo11lasms, which were removed (the first by nephro-ureterectomy, the second by local excision and fulguration), were called papillomas and were thought to be benign. Still later a transitional cell epithelioma, grade 3, Accepted for publication March 2, 1962. Wising, P. J. and Blix, C.: Fall af primar kancer i hiigra ureteren med sekundar kancer i mesenterial-kiirtlarne, rectum och lefvern samt hydronefros. Hygiea, 60: 468-476, 1878. 2 Chauvin, E. and Cerati: Quoted in Gracia, V. and Bradfield, E. 0. 6 3 Ratliff, R. K., Baum, W. C. and Butler, W. J.: Bilateral primary carcinoma of ureter: a case report. Cancer, 2: 815-818, 1949. 4 Felber, E.: Asynchronous bilateral benign papilloma of the ureter with subsequent cancer of ureteral stump, bladder and vagina. J. M. A. Georgia, 42: 198-200, 195:3. 1

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grew in the stump of one incompletely resected ureter near the bladder and was excised. Felber urged more conservative surgical management for the more benign lesions. Crassweller 5 in 1958 added a case of concurrent bilateral papillary transitional cell epitheliomas, invasive on the left. The adjacent ureteral segments presented extensive ureteritis cystica. Both lower ureters, including the tumors and 3 cm. of normal ureter above them, were resected with a cuff of adjacent bladder. Replacement was by a U-sl1'1ped segment of ileum. The patient was free from recurrence 16 1nonths later and felt good, though the value for non-protein nitrogen was elevated and the urine infected. Also in 1958 Gracia and Bradfield 6 reported a similar case. Both tumors were transitional cell epitheliomas, grade 2, with early infiltration into muscle. Two years after right nephro-ureterectomy and lower left ureterectomy, the patient was doing well with a left nephrostomy catheter. Gaca7 has reported a case most recently, that of a man whose bilateral ureteral carcinomas occurred 39 months apart. A followup examination 6 months later reyealed no recurrence. In discussing a presentation by Colston of an interesting case of bilateral epithelial carcinoma of the kidney with an additional left ureteral neoplasm, the latter removed by a segmental ureterectomy, Leadbetter 8 in 1959 stated that he had personal knowledge of 4 patients who within the preceding 3 years had had either bilateral ureteral or renal pelvic tumors associated with bladder cancers. He gave more details of two of the cases: in one the tumors were in the right ureter, the upper left ureter, and the left renal 5 Crassweller, P. 0.: Bilateral primary carcinoma of the ureter with use of ileal graft for ureteral replacement: case report. Brit. J. UroL,

30: 152-160, 1958.

6 Gracia, V. and Bradfield, E. 0.: Simultaneous bilateral transitional cell carcinoma of the ureter: a case report. J. Urol., 79: 925-928, 1958. 7 Gaea, A.: Das doppelseitige papillare Harnleiterkarzinom. Z. Urol., 53: 261-265, 1960. 8 Leadbetter, W: Discussion: Tr. Am. A. GenitoUrin. Surgeons, 51: 149, 1959.

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pelvis; in the other the growths involved the left ureter and the right urcteropclvic ,iuncture. REPORT OF CASE

69- year-old retired farmer brought his brother to the l\fayo Clinic for a major operation in July 1959. 'fVhile here, he underwent a general examination. For 4 years or more he had noted mild shortness of breath, worse with exertion. He was anorectic and had. lost 40 pounds (18.2 kg.) in 2 years. He volunteered no other compla.ints, though later on specific questioning he admitted that one episode of gross hematuria had occurred 8 months previously. The familial history disclosed that 9 siblings were living. Inquiry for precipitating carcinogmic agmts was not rewarding. Physical examination revealed hypertension, the blood pressure measuring 180 mn1. Hg systolic and 100 mm. diastolic. The pulmonary findings were' consistent with chronic emphysema. Laboratory finding8 were as follows: hemoglobin, 13.7 gm. per 100 ml. of blood; leukocytes, 8400 per cubic millimeter; erythrocyte sedimentation rate, 8 mm. in the first hour (lVestergren); and urea, 44 mg. per l 00 ml. of blood. The chest

Frn. 1. Preoperative exeretory urogram: nonfunctioning left kidIH'Y, normal right kidney and uret,er

48\l

appeared normal on roentgenograµhy. Bccu.use of the microhematuria and hypertension, excretory urogram was made. The right upper urinary tract appeared normal. On tbc left rH, excretion of medium wao evident 45 miuutes 1i.fkr injection (fig. l). Cystoscopy performed with general aneHtliesia revealed n normal bladder arnl moderate prns tatic hyperplasia. A Braasch bulb uretcmJ catheter, size 18F, was passed just inside the leH orifice. A retrograde ureterogrnm demonstrnkd a filling defect suggestive of ureteral tumor (fig. 2) completely obstructing the lower portion of the ureter. Minute fragments of tissu<, recovered with the Dormia stone extractor and cytologic studies were reported as showing nest~ of atypical transitional cells. In August, the patient was c:xplorncl for tumor of the left ureter; and the left and ureter, with a cuff of bladckr, 1Ycrc removed. Pathologic examination of the disclosPd that the tumor formed a polypoid ma,;s 3 by 2 cm. at a distance of .J. cm. from the lmYe1 margin of resected ureter (fig. :3, a). cally (fig. 3, b), the neoplasm ,ms a papillary and superficially infiltrating transitional cell thelioma, grade 2 (Broders). ThC' ureter and renal collecting system proximal to the lesion were moderately dilated. vYhen the patient rcturnrd examination .January 1960, he was :\Iilcl right hydronephrosi, and definiw 11rPtercctasis of the lumbar portion of the ureter were· inclicatecl by the excrrtor~· urogram (fig. 4, C~·stoscopy revealed multiple small vesical neoplasms, which biopsy identified a~ non-infiltrating transitional cell cpithcliornm;, grade 2. These were rcmo1·ecl transurcthrnll:1

FJG. 2. Left retrograde uncterogram defect from tumor in pelvic portion.

filling

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UTZ, BRUNSTING AND HARRISON

Fm. 3. a, Gross specimen of left kidney and ureter, including ureteral neoplasm. b, Section of papillary and superficially infiltrative transitional cell epithelioma, grade 2, with branching frond-like pattern. No involvement of muscle was seen (hematoxylin and eosin; X30).

with the Nesbit electroresectoscope. In an attempt to make a right retrograde pyeloureterogram, the catheter met an obstruction at about mid-ureter. Ureterograms demonstrated a filling defect in the lumbosacral ureter (fig. 4, b). The patient was explored for primary tumor of the right ureter. Fortunately the new growth was a small though obstructing polyp. It was excised "'ith a margin of normal ureter in such a manner that continuity of the ureter was preserved by a narrow bridge of normal tissue. A whistle-tip latex SF catheter was employed as a ureteral splint and brought out through the lower calyx adjacent to a nephrostomy tube. These catheters were removed 7 weeks later,

after a pyelogram made with contrast medium introduced via the nephrostomy tube indicated the ureter to be patent. Postoperative recovery was uneventful. Pathologic examination revealed a polypoid tumor, 2 by 1.8 by 1.5 cm., with a collar of normal ureter around the stalk. Microscopically this was found to be a papillary and superficially infiltrative transitional cell epithelioma, grade 2, which did not invade muscle (fig. 5). In June and again in September, small vesical neoplasms were noted at cystoscopy. These were fulgurated transurethrally. The right kidney appeared normal on excretory urography and the ureter was only slightly dilated in the middle

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Fm. 4. a, Excretory urogram made 5 months after left nephro-ureterectomy, showing mild hydronephrosis and ureterectasis. b, Right retrograde ureterogram: filling defect suggesting neoplasm. third. The value for blood urea in September was42mg. In May and October 1961, no vesical neoplasms were discovered; and a right retrograde pyeloureterogram accomplished the latter date, 21 months after the segmental ureteral resection, indicated the ureter was normal except for slight ureterectasis near the surgical site (fig. 6). COMMENT

As in many other kinds of cancer, and specifically because of the insidiousness of their growth, when ureteral tumors become symptomatic, therapy becomes ineffectual. Asymptomatic microhematuria is usually the earliest clue; and as Greene and co-workers 9 have emphasized, it must be acknowledged as justification of urography and cystoscopy. When advanced degrees 9 Greene, L. F., O'Shaughnessy, E. J., Jr. and Hendricks, E. D.: Study of 500 patients with asymptomatic microhematuria. J .A.M.A., 161:

610-613, 1956.

of hydronephrosis or renal malfunction are evident as the result of a ureteral neoplasm, usually the tumor has penetrated the wall of the ureter, presaging an ominous course. The fact 10 that only 36 per cent of ureteral neoplasms are disclosed by preoperative studies suggests that the quality of the ureterography is not good. Cytologic examination of sediment from centrifuged ureteral urine has not been uniformly fruitful, owing to the difficulty of separating reactive transitional cells, such as may be associated with renal calculi, from the cells of well-differentiated transitional cell epitheliomas. Some provident operators occasionally recover fragments of tumor in ureteral stone extractors and these may be studied as tissue sections. Others may be able to visualize grossly villous neoplasms projecting from the ureteral orifice. Most urologists, however, will be confronted by 10 Willmarth, C. L.: Carcinoma of ureter. Am. Surg., 25: 474-481, 1959.

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FIG. 5. (January 1960). a, Specimen from bladder biopsy, showing papillary transitional cell epithelioma, grade 2, with abundant capillaries in some connective tissue stalks of papillae (hematoxylin and eosin; XlOO). b, Section of lesion from right ureter: papillary and superficially infiltrative transitional cell epithelioma, grade 2, showing slight nuclear anaplasia (hematoxylin and eosin; X50).

FIG. 6. Right retrograde pyeloureterogram made 21 months postoperatively: appearance normal except for postoperative ureteral scarring.

the diagnostic impasse of a filling defect in the ureterogram, which could represent a non-opaque calculus, ureteritis glandularis or cystica, a blood clot, pressure from an extraureteral lesion, or a ureteral tumor.

When precise diagnosis is lacking, exploration is prudent. For lesions of the upper third of the ureter it is best to make a flank incision, which can be combined with a lower abdominal incision if a tumor is discovered and complete nephro-ureterectomy indicated. If the tumor exists in the middle or lower third of the ureter, we favor a single anterior incision as described by Culp_ll Departure from. the standard operation of nephro-ureterectomy with bladder cuff for ureteral neoplasms is appropriate if the involved kidney cannot be sacrificed or, in the judgment of the urologist, the tumor is bmign. If the involved kidney must be preservC'd, any one of a variety of so-called conservative surgical procedure's may be utilized. This choice depends on the location, the degree of ureteral involYement, and the malignancy of the tumor. Segmental resection of the ureter preserving continuity, as ,Yas feasible in the case presented, is most desirable from all functional aspects. However, entire uretera.l ablation with nephros11 Culp, 0. S.: Anterior nephro-ureterectomy: advantages and limitations of a single incision.

J. Urol., 86: 193-198, 1961.

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tomy or with ileal replacement may be appropriate. Regarding the acknowledgment of benign ureteral growths, Vest12 in 1945 argued for the distinction between tumors that implant and those that metastasize, and featured 3 patients who, in his opinion, were benefited by conservative ureteral surgical therapy for benign tumors. He was not discussinir the rare nonepithelial benign growths such as myomas, fibrolipomas, and hemangiomas; but tumors he considered transitional cell epithelial papillomas with a minimum of cellular pleomorphism and mitosis and with an intact basement membrane. He postulated that nephro-ureterectomy is not the only appropriate operation for all tumors of the ureter. The controversy engaging the exist" Vest, S. A.: Conservative surgery in certain benign tumors of the ureter. J. Urol., 53: 97-121, 1945.

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ence of benign epithelial papillomas is a no man's land of endless nuances and stout prejudices. Nevertheless, in view of the propensity of even the relatively benign appearing ureteral tumors to recur, we are convinced that limited procedures such as segmental ureteral resection or focal electrofulguration should be used only in extenuating circumstances as exemplified by the case presented. SUMMARY

An unusual case of transitional cell epitheliomas involving the 2 ureters at different times and associated with similar vesical neoplasms is presented. The surgical treatment preserved 1 kidney and ureter. Conservatism is not recommended in therapy of such tumors, except as vital considerations require it.