THE SURGICAL CLINICS of NORTH AMERICA LAHEY CLINIC NUMBER
SYMPOSIUM ON GENITO-URINARY SURGERY PRIMARY TUMORS OF THE URETER EARL
E.
EWERT AND ROBERT
J.
DUNCAN
TUMORS of the ureters, heretofore considered rare, are, as O'Conor has stated, being reported in increasing numbers in the last ten years. In this article we will report our experiences with 8 patients who had primary tumors of the ureters; cases in which the renal pelvis was involved have been excluded from this study. One of the 8 had bilateral tumors classified as benign papillomas, with extensive involvement. Tumors of the ureters present at first glance two exceedingly serious aspects. First, they are recognized to have a poor prognosis and second, they have a tendency to involve the entire urinary tract. The same carcinogenic action, of course, that produces the tumors of the ureter must be acting upon the opposite ureter, bladder and the urethra itself. Actually, it affects the entire excretory tract, yet because bilateral ureteral tumors are so rare, the extirpation of an involved ureter in the light of our meager knowledge raises many interesting questions. In the present series the oldest patient was 74 and the youngest 31 years. All were males. One female with roentgenologic evidence of a tumor of the ureter did not come to surgery, and we were unable to prove or disprove the presence of tumor. In5 of the 8 patients the tumor was in the lower third of the ureter (Fig. 201), in one almost all of the ureter was involved, in one the tumor was in the upper third and in the case of bilateral ureteral papillomas the tumors were in the lower and middle thirds. Five of the 8 patients had gross hematuria. In one case the tumor was detected by routine intravenous pyelographic study. This patient (Case 7) had no symptoms whatsoever related to the genito-urinary tract, but because of his age, was subjected to excretory urograms as part of the general examination. Routine urinalysis revaled no red cells. 623
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EARL E. EWERT AND ROBERT J. DUNCAN
REPORT OF THREE CASES
CASE 5. This 66 year old man first appeared at the clinic August 29, 1945, relating a history of fatigue and pain on the right side. The previous year an excretory urogram done elsewhere for the same complaint revealed a nonfunctioning kidney on the right side, but further investigation was not deemed necessary. In February 1945, a small tumor of the bladder was fulgurated; the pathologist's diagnosis was papillary carcinpma of the bladder. At that time
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Fig. 201. Location of the tumors in Cases 1-4, 6 and 7. transurethral resection was carried out and in August, just before he came to the clinic, a small recurrence was noted. Palpation of the abdomen revealed a questionable enlargement of the right kidney. The excretory urogram demonstrated a nonfunctioning right kidney. Cystoscopic examination disclosed a suspicious bulging of the right ureteral ridge, and the catheter could be passed only a few centimeters up this ureter. A pyelo-ureterogram (Figs. 202 and 203) revealed a typical filling defect of the lower and middle thirds of the ureter consistent with papillary masses. Nephroureterectomy was carried out immediately. The right kidney was composed of a hydronephrotic sac and small papillary masses were found, beginning at the middle third of the ureter, which was greatly dilated, and involving the entire lower third. A cuff of the bladder was removed with the ureter. The microscopic report of the excised ureter was papillomatosis (Fig. 204) of the ureter with
PRIMARY TUMORS OF URETER
Fig. 202.
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Fig. 203.
Fig. 204. Fig. 202 (Case 5). Right ureterogram showing the type of filling defect and massive size of neoplasm. Fig. 203 (Case 5). The right ureter is involved by a continuous solid mass of papillomas, producing complete destruction of the kidney. Fig. 204 (Case 5). Papilloma of the ureter (X45).
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EARL E. EWERT AND ROBERT J. DUNCAN
hydronephrosis. This patient had frequent cystoscopic examinations and in April 1946, a small recurrence was found on the posterior bladder wall. In August 1948, a small papillary tumor was located just inside the bladder neck in the prostatic urethra; on section it was found to be an actively growing papillary tumor, probably epidermoid carcinoma. In May 1949, endoscopy revealed more papillary masses. The pathologic diagnosis was carcinoma arising in the papilloma and invading the prostate. On September 10, 1949, radical perineal prostatectomy was performed including excision of the bulbous urethra, prostate, seminal vesicles and a cuff of the bladder, and a permanent suprapubic cystostomy was instituted. On May 31, 1950, cystoscopic examination through the sinus demonstrated a recurrence at the site of excision of the bladder neck. By this time the region of the prostate and base of the bladder were involved in a large, hard, infiltrating mass extending into the pelvis. Supervoltage deep x-ray therapy was given over this site and the infiltrated areas completely disappeared. During this time repeated excretory urograms and blood chemistry studies demonstrated a normally functioning kidney. This case illustrates the seeding of the bladder from a tumor in the ureter and the increase in virulence from Grade 1, or cytologically benign, to frank carcinoma of a higher degree of malignancy. This patient also related a history of urinary tract infection for a period of about thirty years. CASE 7. A man, 52 years of age, registered at the clinic on July 22, 1949, for a routine physical examination. He had no urologic complaints. On physical examination a mass was palpated in the right flank and an excretion urogram revealed a non functioning right kidney with an enlarged renal shadow. The urine sediment showed from 0 to 3 leukocytes per high power field. Blood studies were negative; the blood nonprotein nitrogen was 28 mg. per 100 cc. On August 8, panendoscopy revealed a moderate trilobar prostatic enlargement. On attempting to catheterize the right ureter an impassable obstruction was met in the lower third and the catheter was found to curl in the ureter at the upper border of the sacrum on the right side. An attempt to make the ureterogram was unsuccessful. A transverse flank incision was made and a large hydronephrotic kidney found. The large dilated ureter was then exposed. It was excised below the point where it narrowed to near normal size. On opening the gross specimen the tumor was found to extend 3 cm. up into the upper ureter. Through a groin incision the remainder of the ureter was then excised with a cuff of the bladder. The pathologic report was as follows: the tumor, 4 by 3.5 cm., extended 6 cm. from the pelvis. It was composed of friable, soft, white tissue. The serosal surface did not appear to be invadedby the tumor. The microscopic diagnosis was papilloma of the ureter (Fig. 205), hydronephrosis, pyelonephritis and hydro-ureter.
The only comment that can be made is that a serious lesion of the ureter producing slow obstruction of the kidney which gives no evidence of function, may not produce symptoms. This case also illustrates the·
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value of excretion urography and indicates that it should be used more frequently in a complete examination of a patient. CASE 8. This 45 year old white man registered at the clinic on September 19, 1949, relating a history that in the Orient the previous May cystoscopic
examination was carried out because of a painless, causeless hematuria. He was in doubt as to the true diagnosis. While in this country, he consulted us because of hematuria which had again appeared in July. On urologic examination the urine was found microscopically to contain blood and on culture was sterile. He was admitted to the hospital for study. Cystoscopic examination revealed the bladder to be entirely normal. Urinary function tests demonstrated normal
Fig. 205 (Case 7). "Silent" papilloma of the right ureter (X45). excretion of dye from both sides and bilateral specimens were collected for study, including tests for ova, particularly relating to the Schistosoma haematobium. Bilateral pyelo-ureterograms (Fig. 206, a) revealed filling defects in both ureters with a normal pyelographic pattern above the defects. Because of the presence of bilateral filling defects which were consistent with papillomas of both ureters or with some granulomatous lesion, cystoscopic examination and the same procedures as in the first examination were carried out again, with the same report of no ova or organisms in the urine. On the right side, the middle and lower thirds of the ureter on both retrograde pyelograms revealed filling defects consistent with small papillary masses. On the left side there were also characteristic filling defects; one defect, at least 1.5 cm. in diameter, was seen in the ureter overlying the lower margin of the sacro-iliac joint. Daily urine specimens were examined for ova and no evidence of any parasitic infection was found. On September 28, the left ureter was exposed through a
b
a
c .Fig. 206 (Case 8). a, Bilateral retrograde pyelograms showing bilateral ureteral filling d~fects with characteristics common to each side. b, Left ureterogram before removal of a large papillary neoplasm. Smaller defects cimalso be seen on the right side. c', Typical papilloma of the ureter (X45).
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Gibson type incision and the large mass noted in the roentgenogram, which caused a decided bulging of the ureter, was easily seen. On palpating the ureter above and below the mass, it was thought that other small masses could be felt. On incising the ureter the large papillary neoplasm protruded; 3.5 cm. of the entire ureter was excised (Figs. 206, b, and 207) and an end-to-end anastomosis performed, splinting the ureter by means of a T tube, the long arm of the tube being brought out about 2 cm. from the anastomosis. On examination of a frozen section the pathologist reported the tumor to be a benign papilloma. and this
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Fig. 207 (Case 8). Diagram shows the location of the papillomas and site of .the ureteral anastomosis. diagnosis was substantiated by permanent sections a few days later (Fig. 206, c). The fistula ceased to drain in twenty-four hours. The T tube was removed sixteen days after operation. Unfortunately, the patient, a British subject, had to return immediately to England and we asked him to see Mr. Terrence Millin. We were gratified to receive a report from Mr. G. M. Lewis, Mr. Millin's associate, on December 13, 1950, fourteen months later, which stated that at cystoscopy the bladder was clear, and on retrograde pyelography there was no evidence of any abnormality in either ureter or renal pelvis.
The disappearance of the papilloma, which at operation could be palpated in the left ureter, and which gave a typical filling defect, is difficult to explain.
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COMMENT
Three of the 8 patients did not have the finding most often associated with a urologic tumor, namely, hematuria. Blood in the urine, either on gross or microscopic examination, is generally recognized among urologists as an unreliable sign in the early diagnosis of tumors of the urinary TABLE 1 CLINICAL FINDINGS
Age, years
Symptoms
Infected Urine
Other Genito-urinary Disease
2
55 74
Hematuria; prostatism Hematuria; prostatism
Yes Yes
3
49
4 5
31 66
No Yes Yes
6
59 52 45
Pain in left flank Hematuria Pain in right flank; fatigue Hematuria No symptoms Hematuria
Prostatitis Benign prostatic hyper trophy None Papillomas of bladder Previous papilloma 0 bladder None None None
Case 1
7 8
No No No
TABLE 2. PATHOY,OGIC DIAGNOSIS AND SURVIVAL DATA
Case 1
2 3
4 5 6
7 8
Treatment
Pathologic Diagnosis
Papilloma Epidermoid carcinoma, Grade 2 Epidermoid carciN ephro-ureterectomy noma, Grade 3 Papilloma Nephro-ureterectomy Papillomatosis N ephro-ureterectomy Epidermoid carciN ephro-ureterectomy noma, Grade 3 Papilloma N ephro-ureterectomy Segmental resection of Papilloma ureter
N ephro-ureterectomy N ephro-ureterectomy
Survival, Living years
2
Yes No
2l
No
2i 51 I}
Yes Yes No
1;
Yes Yes
8
Ii
tract (Table 1). We were disturbed that one patient had no symptoms. The more frequent use of excretion urograms in adult individuals will help to solve this problem. Two patients complained. of flank pain and the kidney seemed to be enlarged on palpation. If the patient is obese, however, it is impossible to palpate a kidney and here again urograms must be advised. Hematuria, either gross or microscopic, necessitates a
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complete examination. In those patients with unexplained bleeding from a kidney the presence of a tumor of the ureter must always be considered if the pyelographic pattern is normal. If tumors are present in the bladder the urologist must prove that the upper urinary tract is not also a site of tumors. Furthermore, a patient who has had papillomas of the bladder should have a study of the upper urinary tract made at least once a year. In the presence of multiple papillomas in the bladder (Table 2), which are now generally recognized as Grade 1 malignant tumors, the entire excretory tract should be considered the possible site of malignant disease. Preliminary endoscopy of the urethra from the external urinary meatus to the bladder neck is a prerequisite of the patient's periodic check-up examination. It seems more and more evident that when the urethra becomes involved from a Grade 1 malignancy of the bladder, the involvement is of a higher grade of malignancy and the prostatic gland quickly becomes involved. This has happened to 2 of our patients, one of whom is included in this series. The rare occurrence, as in Case 8, of involvement of both ureters with the complete disappearance of the lesions fourteen months later is noteworthy. The fact that ureteral tumors tend to extend to other portions of the urinary tract makes it imperative that patients with these lesions must have complete urologic investigation and must be carefully followed. All urologists are in agreement that nephroureterectomy with removal of a cuff of the bladder should be carried out if the opposite kidney is normal.
REFERENCES 1. Barnes, R. W. and Kawaichi, G. K.: Primary neoplasms of ureters; report of 6 cases. Urol. & Cutan. Rev. 48:430-436 (Sept.) 1944. 2. Counseller, V. S., Cook, E. N. and Seefeld, P. H.: Primary epithelioma of ureter; follow-up study of 18 cases with addition of 9 new cases. J. Urol. 61 :606-615 (June) 1944. 3. Hamm, F. D. and Lavalle, L. L.: Tumors of the ureter. J. Urol. 61:493-505 (Mar.) 1949. 4. Lazarus, J. A. and Marks, M. S.: Primary carcinoma of ureter with special reference to hydronephrosis; review of literature and report of unusual case. J. Urol. 64:140-157 (Aug.) 1945. 5. Lewis, G. M.: Personal communication. 6. O'Conor, V. J.: The treatment and prognosis of papillary tumors of the renal pelvis and ureter. J. Urol. 61:488-492 (Mar.) 1949.