Primary Tumor of the Ureter

Primary Tumor of the Ureter

PRIMARY TUMOR OF THE URETER END RESULTS IN THREE CASES 1 A. W. HUNTER Vancouver, British Columbia Primary tumors of the ureter are of infrequent occu...

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PRIMARY TUMOR OF THE URETER END RESULTS IN THREE CASES 1 A. W. HUNTER Vancouver, British Columbia

Primary tumors of the ureter are of infrequent occurrence. W. W. Scott (1) has published a very complete article on primary carcinoma of the ureter, showing 62 reported cases in his review of the literature. Melicon and Findlay (2) have reviewed the literature on primary benign papillary tumors of the ureter, and recorded 29 cases. I wish to report 2 cases of primary carcinoma of the ureter, and 1 case of benign papilloma.

Case 1. W. W. C., No. 48387, male, aged 50 years, referred by Dr. Day Smith, admitted March 28, 1929, complaining of pain in the perineum and bladder (when walking) for 1 year, and hematuria twice during the last 2 months. Lately he has developed increasing pain in the perineum and bladder, and dysuria, but no symptoms referable to the kidneys, and no increasing frequency of urination. The patient reported that x-rays showed no calculi in the urinary tract. Examination. General physical examination was essentially negative. Urinalysis showed a few red and white blood cells. On rectal examination the prostate was negative. On April 21, 1929, cystoscopy was performed. No residual urine was found. A white calculus, about¾ inch in diameter was seen. The right ureteral orifice was small. The bladder wall bulged in the region of the right ureter, and a foreign body was suspected. The vesical calculus was crushed and removed. The patient was advised to return in 2 weeks, but did not come back until September 9, 1929, after having passed blood without dysuria. On October 16, he still complained of symptomless hematuria. Cystoscopy revealed no prostatic enlargement, no calculus in the bladder, but a hemispherical fulness at the right ureteral orifice was seen. A small gray tag of tissue-like material appeared in the opening. Both ureters were easily catheterized, and no stone was detected in the right ureter, but hemorrhage followed the withdrawing of the catheter. The function was good on the left side, and less on the right. 1 Presented before the annual meeting of the American Urological Association, Atlantic City, N. J., May 22-24, 1934.

443 THE JOURNAL OF UROLOGY, VOL,

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The right renal pelvis held 40 cc. 12 per cent sodium iodide, while the left held 10 cc. A filling defect was seen in the lower right ureter and diagnosed tumor (fig. 1). Above it the ureter was markedly dilated, as was also the kidney pelvis (fig. 2). The left ureter functioned well, and the pyelogram was negative on that side. Examination. B. P. 110/70, W. B. C. 73,200. Urinalysis: S. G., 1017, acid, alb., pus, R. B. C., no sugar. On October 31, 1929, operation (Dr. Hunter) was carried out under ether anesthesia. An oblique incision was made in the right lower quadrant of the abdomen, and the lower third of the ureter exposed. It was dilated, and a fleshy-like mass, suggesting tumor, and not stone, was palpated within the

FIG. 2 FIG. 1 1. Case 1. Shows catheters were up either side and no stones showing. FIG. 2. Case 1. Showing normal left renal pelvis and ureter and right hydronephrotic kidney, and dilated ureter. The lower end of the ureter is not filled and the iodide solution extends down more on the central side of the ureter, and in the original ureterogram, a frayed filling defect can be seen. In an ordinary dilated ureter, the lower end should appear as a rounded sausage-like shadow. This photo is a negative, hence the right kidney is reversed. FIG.

ureter. This mass was found to extend almost to the bladder. The ureter having been freed, was ligated with catgut and divided at the upper third. Below this point the ureter, down to the bladder, was excised in one piece without being opened. After dividing the lower end of the ureter a cigarette drain was inserted. The muscles and fasciae were approximated, the skin closed, and a dressing applied. The patient was then placed on his left side, and the usual renal incision made, exposing the right ureter. The remaining portion of the ureter was delivered, and the hydronephrotic kidney removed. A cigarette drain was inserted, and the muscle, fascial and skin layers sutured. A catheter was inserted in the urethra for continuous drainage. On the day following operation the temperature was 100.3, pulse 82, and

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respirations 20. After the fourth day the temperature remained normal until his discharge on the fourteenth day. Pathological report (Dr. Allan Y. McNair). The kidney is enlarged, soft,
FIG. 3 Frn.4 3. Case 1. Shows the lower portion of the ureter. The lower end of the ureter is at the top. The broad based elevated tumor is visible. FIG. 4. Case 1. Shows the remainder of the ureter and the kidney pelvis free from tumor . involvement. FIG.

show no inflammatory processes. Within 1½ inches of the lower end of the ureter, there is a sausage-shaped swelling which measures approximately 2 inches in length, and¾ inch in diameter, moderately firm, and the bulk of this mass can be moved inside the ureter. The outer surface appears to be smooth and there is no point of ulceration or hemorrhage visible.

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The ureter was opened from end to end and revealed a villous tumor practically occluding the lumen, in places somewhat hemorrhagic, arising from the wall over an area ¾ inch in diameter, firmly and very definitely attached (fig. 3). It has the same appearance as that of a papilloma of the bladder. Microscopic study shows a series of branching tufts of stroma, which are very soft, delicate and vascular, covered by multiple layers of epithelium, resembling that of the bladder. Cells vary in shape from short spindle to almost round. Some of them are vacuolated. Villi are matted in many places. There is well marked variation in size of the cells and very extensive areas showing no regularity in arrangement. They are markedly hyperchromatic and pyknotic, and show a large number of mitotic figures. Round cell infiltration is seen in the wall of the ureter, and in places increased cellularity. Degeneration is seen in scattered areas. One point of attachment in the ureter wall is seen in which invasion of the muscle coat is noted. This tumor is malignant. Diagnosis. Primary papillary carcinoma of the ureter; secondary hydronephrosis. In April, 1934, the patient reported that he was free from symptoms. Examination showed no evidence of recurrence. Case 2. W. N., No. C15596. Patient, male, aged 52 years, referred by Dr. F. W. Andrews, was admitted on April 5, 1929. He gave a history of first noticing painless hematuria in 1928. He was advised to have cystoscopy performed, but delayed in doing so. The blood disappeared and recurred in March, 1929. The hematuria was more severe; clots were present, but otherwise he felt well. General examination revealed no gross lesion. The prostate was negative. B. P. was 136/78, T. 98°, P. 84, R. 20. The urine was acid, S. G. 1021, albumen plus 1, sugar 0, R. B. C. plus 2, W. B. C. plus 1, no casts. Cystoscopy was performed on April 6. The urethra and bladder were negative. The bladder urine was slightly turbid. The right ureteral catheter was easily inserted, and the urine obtained from that side was negative. It was impossible to pass a catheter up the left ureter. The catheter tip was found dissecting beneath the mucosa of the bladder. Repeated attempts to catheterize the left ureter were unsuccessful. No further examination was carried out, and the patient was advised to return in 2 weeks. On April 26 cystoscopy was again performed. Urethra, prostate and bladder were negative. The bladder urine was grossly free from blood. The right ureter was easily catheterized, and clear urine was obtained. Later urine became slightly blood-tinged. On the left side the catheter was easily passed for 5 to 8 cm., and then an obstruction was encountered. The catheter drained freely. The urine, which was obtained, was slightly turbid. An

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attempt was then made to pass the left ureteral catheter up near the kidney pelvis, but failed. No decided hemorrhage occurred. The right pyelogram (fig. S) shows the kidney well filled and apparently normal. On the left side the ureteral catheter reached to just above the upper border to the sacrum. Immediately above this, there is a small portion of the ureter which is somewhat dilated, containing sodium iodide solution. The outline of the kidney is well seen, and a considerable quantity of the solution appears in the calyces. These are dilated, rounded, and probably eroded. There is a definite hydronephrosis present. (See fig. 6.) The bladder urine was clear, yellow, acid, S. G., 1020, albumen plus 1, sugar 0, pus plus 1, epithelial cells plus 1, red blood cells plus 1.

FIG. 5 FIG.6 5. Case 2. Shows the ureteral catheters in place. The right is up to the renal pelvis. The left is up to the ilio-sacral notch and no calculus is seen. FIG. 6. Case 2. Shows a normal right renal pelvis and ureter. The left side shows a hydronephrotic pelvis and dilated ureter to the ilio-sacral notch. Then an absence of filling, showing the tip of the opaque catheter with some relaxation of the ureter below. The diagnostic point in this case is the filling defect where a calculus shadow did not previously exist. FIG.

Urine from the right ureter was straw-colored, slightly turbid, pus plus 1, R. B. C. plus 2, epithelial cells plus 2, bacteria 0, calcium oxalate crystals, casts 0, urea 2.2 per cent. Urine from the left ureter was straw-colored, slightly cloudy, pus plus 1, R. B. C. 0, epithelial cells plus 1, bacteria 0, casts 0, urea 0.85 per cent. My impressions were that there was an obstruction in the left ureter at the brim of the bony pelvis due to a tumor or a non-shadow producing stone associated with a moderate hydronephrosis. On April 29, 1929, a left lower quadrant incision was made. The muscles were split, and the peritoneum pushed inward. The ureter defined and found

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to be¾ to 1 inch in diameter. A rather firm mass was palpated. Attached to the inner side of the ureter was a small blood vessel that was ligated. The ureter was cut between ligatures below the mass. The upper end of the ureter was freed and pushed well up retroperitoneally. The muscle layers sutured and the skin closed. The patient turned on his right side and a left renal incision made. The ureter was defined, freed and delivered. The kidney

FIG. 7 FIG. 8 FIG. 7. Case 2. Shows the tumor mass bulging the lower end of the ureter. The upper portion of the ureter .is not so large. The general outward contour of the kidney is well preserved. FIG. 8. Case 2. Illustrates the opened ureter with the tumor in place and the remaining portion of the ureter and kidney pelvis show no tumor involvement.

was then removed with the ureter attached. A cigarette drain inserted and the muscle layers, fascia and skin sutured. The patient made an uneventful recovery, and was discharged on May 23, 1929. Pathological report (by Dr. Pitts). Specimen consists of left kidney, of normal size, with 15 cm. of ureter attached (fig. 7). The distal 5 cm. of ureter is dilated to 1.5 cm. in diameter. This dilated portion is quite firm and there is apparently a tumor mass either within the lumen or within the walls of the

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ureter. The upper extremity of this dilated portion is 8 cm. from the kidney; a date-stone shaped, grayish-red, papillomatous appearing growth 3 cm. long and 1 cm. in diameter is found, which is growing from a narrow pedicle attached to the wall of the ureter at the upper extremity of the growth, hanging downwards in the lumen of the ureter (fig. 8). The pelvis of the kidney and calyces are very slightly distended, and the wall of the ureter about the growth is somewhat thickened. There does not appear, however, to be any particular gross infiltration of the growth. Microscopic examination. Sections taken through the growth show a fairly definite papillomatous structure, composed of quite bulky and more slender papillary formations, with less well defined central stalk and many surmounting layers of rather spindle-shaped or elongated transitional epithelial cells. The borders of these masses are fairly regular and the cells appear fairly uniform throughout, and no definite infiltration at the base is noted; and though the blood vessels are congested, there is only a slight diffuse small round cell infiltration at the base. At one edge of the growth, however, there are numerous dilated channels filled with red blood cells and pus cells. This appears to be an area which has undergone degeneration and inflammation, as there are still less well-defined epithelial cells in these areas. Diagnosis. This is evidently a benign papilloma of the ureter. Dr. Andrews, who has examined the patient regularly, reported that on April, 1934, he was entirely free from symptoms. The following is a report of my first case of primary tumor of the ureter.

Case 3. E. G., B73563 male, aged 43 years, referred by Dr. MacLachlan, was admitted on May 27, 1926. He stated that in March, 1926 he had first noticed streaks of blood in his urine, which had recurred from time to time. A few days before admission, following a long automobile drive, he noticed that his urine was decidedly bloody. The history revealed that he had simply symptomless hematuria. At the age of 14, he had some hematuria, which had cleared up with rest in bed. There had been no recurrence of the hematuria until the present illness. There was no frequency of urination. General physical examination revealed an unusually well-developed man, weighing 200 pounds. Temperature, pulse and respirations were normal. No abdominal masses were made out; no renal, abdominal or rectal tenderness. The prostate was negative. On cystoscopy (under sacral anesthesia) the posterior urethra and bladder were negative. The bladder urine was clear. The right ureteral catheter met an obstruction within S cm. of the ureteral opening, and immediately a severe hemorrhage ensued. The catheter was removed, and an x-ray was taken. The following report was made by Dr. McIntosh.

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"The films show what I take to be a calcareous deposit, probably a calcified lymph gland, at the upper margin of the sacro-iliac joint on the right side. A smaller calcareous deposit is seen about half an inch higher up in the same plane. In the pelvis, on the right side and apparently close to the point of entry of the ureter into the bladder, a smoothly outlined shadow is seen, oval in shape with a transparent centre, thus presenting a ring-like appearance. A line of density stands across the middle of this oval shadow dividing the transparent circle into two parts. I would not be sure from the appearance of the shadow that it was definitely due to ureteral calculus."

Frn.10 FIG. 9 FIG. 9. Case 3. Shows the catheters in the ureters. The left goes up to the renal pelvis. The right appears turned backward in the ureter near the bladder end of the ureter. No calculus is seen. (This photo is a negative, hence the reversal of sides.) FIG. 10. Case 3. Shows the right ureter filled with iodide solution. It also shows the lack of filling in the lower end of the ureter due to the tumor at that area. The lower end of the ureterogram is irregular and ill-defined.

The following day the bleeding had largely subsided. The patient was discharged. The urine became clear and remained so. Cystoscopy was again performed on June 10. The bladder urine was clear. There was no evidence of acute or chronic inflammation in the bladder. The right ureteral orifice was a little fuller than normal, but soft and pliable. When peristalsis of the ureter was seen, there was a very small brownish mass protruding from the lower end of the ureter. This small projection disappeared on relaxation of the ureter. I tried to ascertain if this protrusion were organised vascular tissue, but was unable to do so.] An attempt was made to catherise the right ureter. A definite obstruction was encountered just within the opening. By rotating the catheter, it was possible to pass the obstruction

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and the catheter was inserted 15 cm. Blood issued from the ureteral opening about the catheter and persisted for some minutes. The urine from the right ureteral catheter was blood-tinged. The left ureter was easily catheterised all the way, and clear urine was passed in spasmodic drops. The cystoscope was removed. Five cubic centimeters of sterile water was injected into the right catheter as it had plugged with blood. This amount caused distension of the right kidney pelvis. It was allowed to drain 15 minutes. The distension pain disappeared. The right ureteral catheter then held 40 cc. of 12 per cent sodium iodide. There was no renal pain produced. The patient complained only of some suprapubic pain. A pyelogram was taken but the solution failed to fill the bottom of the ureter. The iodide solution was again injected and the same appearance was found.

FIG. 11. Case 3. Illustrates the upper portion of the ureterogram and the pyelogram. The right renal pelvis appears of normal size. You will note there is no hydronephrosis present.

The x-ray report (by Dr. H. H. McIntosh) is as follows: "Films of the urinary tract do not show any shadows suspicious of calculus, apart from an oval shadow in the pelvis on the right side which is half an inch away from the ureteral catheter. Opaque solution was injected through the ureteral catheter, the outline of the kidney is seen faintly filled with a normal pelvis and calices (fig. 11). The ureter is not unduly dilated. The catheter is seen curled upon itself evidently having reached the level of the brim of the pelvis and the point has then been deflected so that it is now lying pointing toward the bladder (fig. 9). Below this point no opaque solution is seen in the ureter (fig. 10). The ureter above is not dilated. A re-examination was made to visualise, if possible, the lower part of the ureter, but in this we were unsuccessful, no solution being seen in this part of the ureter."

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The laboratory report on the urines were: Bladder: clear, pale straw, specific gravity 1007, acid, albumen negative, sugar negative; crystals, fat, casts, bacteria, red blood cells and pus cells none. Eight ureter

Appearance ....................................... Color ........................................... . Crystals. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Casts ............................................ Fat .............................................. Bacteria. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Red blood cells ................................... Palys ............................................ Epithelial. ....................................... Albumen...... . ............................... Sugar ............................................ Urea........ . . . . ...........................

Left ureter

Bloody

Clear Pale straw None None None None None None None None Very many None None None None Plus 1 None None None None 0.8 per cent 0.94 per cent

My deductions from the above observations were (1) that the lower end of the right ureter contained a mass of irregular tissue that produced an obstruction, and caused the catheter to double in the ureter; that the lower end of the iodide shadow was irregular due to a tumor and hence the lack of filling in the bottom of the ureter; (2), a non-shadow producing sediment around a non-shadow producing stone could not definitely be excluded. I advised removal of the mass, ureter and kidney. June 16, 1926, the patient was prepared for operation. Temperature 98°, pulse 76, respirations 20. Chest and abdomen negative, blood pressure 132/74. Blood group 2. Kahn test negative. The pre-operative diagnosis: Primary tumor of right ureter. He was given ether anaesthesia, color maintained only by oxygen insufflation. Slight duskiness evident in face throughout anaesthesia. A right lower quadrant incision was made extraperitoneally and the ureter defined. No enlarged lymph glands were found. A fusiform enlargement was found in the lower portion of the ureter extending down to the junction of the ureter and the vas deferens. Beyond this point, it lessened in size until it became of normal calibre. The lower end was ligated and cut off at its entrance to the bladder. The upper end was ligated at the highest point possible and cut across. The intervening portion of ureter was removed (fig. 12). A cigarette drain was inserted and the wound closed. The excised ureter appeared to contract after removal. The condition of the patient did not justify the additional operation for removal of the right kidney. The following day his temperature was 100.3°, pulse 115, respirations 20. The succeeding days, his temperature gradually became normal. The patient complained of gas pains for 4 days and there was considerable distension.

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There was never any right renal pain, nor any tenderness in the right costovertebral angle. He was discharged on July 16, 1926. The patient was advised to have his right kidney removed, but refused. General physical examination was negative, until early in 1931. The following report of Dr. A. J. MacLachlan summarizes his case to the end. "On March 2, 1931 the patient complained of indigestion. Gastro-intestinal x-ray was negative for visible pathology. The patient left his phvsician and tried various dietetic treatments. "He returned to his doctor July 4, 1932, showing no improvement in his gastric symptoms. He had lost 20 pounds. He is anaemic and an epigastric mass is palpable. He refused further x-ray diagnosis. August 30, 1932, he

Frc. 12. Case 3. Shows the lower portion of the ureter laid open on a board. is visible. The lower portion is the bladder end.

The tumor

again consulted Dr. MacLachlan who demanded further x-ray examination. This revealed an obstruction at the pylorus. He was continually vomiting dark tenaceous fluid. His stomach was washed out for a few days. "On September 7, 1932 he was admitted to the hospital, his stomach being washed out with a Rehfuss tube, when he was seized with coughing and great pain in the abdomen. He developed severe shock. A gastric perforation was diagnosed. He was transfused and operated upon by Dr. A. J. MacLachlan and Dr. A. B. Schinbein. A colloid carcinoma of the stomach with perforation was diagnosed. There were metastases in the omentum. There was no mass in the right renal area. The patient died on the operating table." An autopsy was not obtained, but I examined the abdomen through the surgical incision and found a diffuse carcinoma infiltrating the pyloric end

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of the stomach. The stomach was much enlarged. The infiltration was along the muscle layers, rather than a heaping up mass of the mucosa. The liver was not palpably involved. The gastro-colic omentum was involved. The abdominal cavity was filled with dirty contents. The left renal area was negative. The right renal area revealed a well defined kidney and no other mass. The kidney and ureter were removed and photographed unopened after fixation. The mass was opened and photographed. The kidney shows a hydronephrosis and dilated ureter with no evidence of tumor involvement (fig. 13). This proves my case as one of primary carcinoma of the ureter after 6 years observation. Pathological note (by Dr. Pitts). "In 1926 Dr. A. W. Hunter resected the right ureter from this patient for what was apparently a primary tumor of

FIG. 14 FIG. 13 FrG. 13. Case 3. Shows the unopened upper portion of the ureter and renal mass. This was removed 6 years later. Note the hydronephrosis and distended ureter. FIG. 14. Case 3. Shows the hydronephrotic sac. There is no tumor involvement of the renal pelvis or of the ureter.

the ureter, which on microscopic section proved to be a primary papillary carcinoma of the ureter. In 1932 an exploratory laparotomy was done and a mass was found in the stomach with a perforation in its centre and large numbers of semi-gelatinous metastatic nodules were found in the omentum and mesentery. A portion of one of these was removed for microscopic examination. The operative wound was reopened and the right kidney and ureter removed. "This kidney had been converted into a markedly thin-walled hydronephrotic sac and there was no evidence of any primary growth in it (fig. 14). "The sections made through the mass from the omentum showed a typical adenocarcinomatous growth of the so-called colloid or mucoid type, bearing no resemblance whatever to the primary growth in the right ureter. "It seems reasonable to assume that there were two primary carcinomatous foci in this instance, i.e., the ureter and the stomach."

DISCUSSION

455

CONCLUSIONS Two of the 3 cases came with symptomless hematuria and the early diagnosis and treatment prolonged the life of one man for 6 years, when he died of another lesion; and the other patient is still well. The third case was complicated with a vesical calculus and his life was only saved after a complete renal study showing that the vesical calculus was of minor importance and that his symptomless hematuria was the warning symptom of his ureteral tumor, and he remains well 5 years after the removal of the tumor. REFERENCES (1) ScoT'r, W.W.: Surg., Gynecol. and Obstet., 1934, !viii, 215. (2) MELICON AND FINDLAY: Surg., Gynecol. and Obstet., 1932, liv, 680.

DISCUSSION PROF. LUIGI CA'.PORALE (Torino, Italy): I should like to refer to a case of mine, which was reported in the Urologic and Cutaneous Review, June, 1931. At that time there were 51 reported cases, and mine brought the total to 52. The tumor was supposedly found by urological examination and confirmed by histological study. The important points are these: In the majprity of cases, the tumor produces dilatation of the cavity above, with consequent decrease in the size of the kidney, but in my case, I found an atrophic epinephrosis. It is a rather rare disease. DR. HOMER G. HAMER (Indianapolis, Ind.): I was very much interested in Dr. Sprenger's report of his cases of bilateral carcinoma of the kidney, also in Dr. Bothe's splendid presentation of tissue changes in kidney tumors after x-ray therapy. In a review of the subject of primary tumors of the ureter, one is impressed with the difficulty of diagnosis; also that its rarity is decreasing with the improvement in our diagnostic methods. The early cases were found at autopsy or operation for surgical diseases of the kidney, most commonly some obstructive condition such as pyonephrosis or hydronephrosis. Since Albarran's report of a preoperative diagnosis in 1896, more and more cases have been reported in which at least a presumptive diagnosis of tumor of the ureter was made. Search for the cause of obscure hematuria has become more painstaking and the interpretation of the significance of obstructed ureter has led to a correct diagnosis more and more frequently.