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Primary Papillary Carcinoma of the Ureter1
PRIMARY PAPILLARY CARCINOMA OF THE URETER 1 WM. N. TAYLOR
AND
C. A. KUEHN
From the Department of Urology, Ohio State University
The rarity with which tumors of the ureter occur, the lack of a definite symptomatology, and the difficulty of diagnosis justify the reporting of all cases encountered. The literature at the present time records about 87 cases of maligrrant tumors of the ureter. Of these approximately 60 per cent are recorded as papillary growths. It is to this group that we wish to add two cases. Case 1. E. P., white male, 54 years of age, entered the University Hospital August 7, 1934. He stated that he had an attack of hematuria on April 6, 1934. The blood appeared in the urine without previous bladder symptoms or other provocation. Bleeding continued throughout the day and an acute retention developed due to a clot in the bladder. The patient was removed to a neighboring hospital where the retention was relieved and the clots removed. He remained in bed 5 days, at the end of which time the urine was clear and he was voiding normally. Six weeks later, he had another attack of hematuria which lasted 5 days. He was not inconvenienced by it in any way except for a moderate frequency of urination day and night. He did not consult his physician at this time. Two months following this second attack of bleeding, the urine was clear and his bladder symptoms had subsided. The day before he entered the hospital, he had a moderate hemorrhage associated with frequency of urination. He has only suffered frequency of urination during the attacks of bleeding. He has never complained of chills, fever, back pain, or abdominal discomfort and he considered himself in good health although he had lost about 10 pounds in wdght. The family and past history were irrelevant except for gonorrhea at the age of 29. Physical examination was negative except for many carious teeth and the surgical absence of the left radius, which was removed because of osteomyelitis. A moderate arteriosclerosis was present with a moderate hypertension. The blood pressure was 160 systolic and 82 diastolic. Examination of the abdomen revealed no tenderness or other abnormalities. Urinalysis showed a few red blood cells; otherwise it was normal. The blood count was within normal limits with a hemoglobin of 98 per cent. 1 Read before the North Central Branch of the American Urological Association, Cincinnati, Ohio, September 25-26, 1936.
504
PRIMARY PAPILLARY CARCINOMA OF URETER
505
Nonprotein nitrogen was 25 mgm. The blood sedimentation rate was moderately rapid. The blood Wassermann and Kahn gave negative reactions. Cystoscopic examination revealed a normal bladder except for a tumor covering or protruding from the left ureteral orifice. The tumor was conical in shape with the base over the orifice. In size, shape, and color it resembled a small ripe strawberry. The presence of the tumor made catheterization of the left ureter impossible. Indigo carmine did not appear at the left orifice and intravenous urography with a pyelogram did not show the left kidney. A plain film showed the left kidney to be slightly enlarged without evidence of calculus in the kidney or ureter. The urine, kidney function, and pyelograms on the right side were normal. Preoperatively, our impressions were that we were dealing with a tumor of the bladder that originated very close to the left ureteral meatus, or in the ureter and protruded through the ureteral meatus. However, the complete destruction of the kidney insofar as function and urograms were concerned led us to believe that there probably was more tumor in the ureter than that which was seen on cystoscopic examination. Operation was performed under spinal anesthesia on July 12, 1934. The bladder was first opened to determine the site of origin of the tumor. It originated in the ureter and protruded through the orifice. The lower part of the ureter was then investigated extraperitoneally. The tumor proved to be a markedly indurated and irregularly elongated mass firmly attached to the surrounding structures and extending from the base of the bladder to a point 2 inches above the bifurcation of the iliac artery. There was no apparent infiltration of tumor tissue into the surrounding structures, but it was densely adherent to them by what apparently was a chronic inflammatory process. With the cutting current, a wide section of bladder was taken about the tumor in the ureteral orifice. The ureter was freed as high as possible through the abdominal wound. The tumor mass was then securely wrapped in alcohol gauze and dropped back into the wound. The patient was then turned on his side and the left kidney together with the remainder of the ureter removed (fig. 1). Pathological report (Dr. H. L. Reinhart): "Upon gross examination the specimen consisted of a kidney, ureter, and a section of urinary bladder. The kidney was hydronephrotic and the upper half of the ureter was moderately dilated. The lower half of the ureter consisted of an irregularly elongated mass of firm consistency which measured 14 cm. in length and 3 cm. in diameter. Protruding from the ureteral orifice into the bladder was a firm meaty papillary tumor. "On cut section the hydronephrotic kidney and upper ureter were filled with a thin hemorrhagic fluid. The kidney tissue averaged 2 mm. in thick-
506
WM. N. TAYLOR AND C. A. KUEHN
ness. The mucosa of the kidney and upper ureter was thin and showed no evidence of neoplasm. The lower half of the ureter was the seat of a marked
FIG. 1
FIG.
2
Fm. 1. Case 1. Kidney removed at operation FIG. 2. Case 1. Cross section of kidney
papillary proliferation with considerable aseptic creamy exudate bathing the entire neoplasm. The two portions of the ureter were divided by sharp angulation, separating the neoplastic portion from the hydro-ureter (figs. 2 and 3).
PRIMARY PAPILLARY CARCINOMA OF URETER
507
"Microscopic examination of the tumor of the ureter revealed a papillary type of growth in which the connective tissue stalk was very delicate with areas of distortion of the vascular supply. There were numerous areas of necrosis apparently clue to the interrupted blood supply. There was also a subacute inflammatory exudate present. This, with the general conformation of the papillary stalks, gave the impression of an inflammatory papilloma; but there was a moderate degree of anaplasia of the epithelial cells with from 3 to 5 mitotic figures present per thousand cells, occasional areas exhibiting invasive characteristics. Microscopic examination of the portion of the tumor protruding from the ureteral orifice into the bladder revealed the same type of tumor but it is more malignant (fig. 4).
Frc. 3
Frc. 4
Frc. 3. Case 1. Low power photomicrograph of tumor in ureteral lumen FIG. 4. Case 1. High power photomicrograph of tumor in ureteral orifice
"Diagnosis: Papillary carcinoma of the ureter, Grade 2, malignancy." The convalescence was uneventful and the patient was discharged in excellent condition 27 days later. In the meantime he was given a course of deep x-ray therapy over the bladder, left kidney region, and spine. In reply to an inquiry June 1, 1936, two years after his operation, he stated that he had no urinary difficulties and that he was still able to work some, but was suffering from what his physician diagnosed as "heart trouble." The lower half of the ureter in this case presented a papillomatosis with low grade malignant changes. The tumor in the lower end of the ureter which protruded into the bladder presented a much higher grade
508
WM. N. TAYLOR AND C. A. KUEHN
of malignancy. Stoerk refers to papillomatosis due to a chronic inflammatory hyperplasia, which he believes should not be considered neoplastic. However, it is reasonable that malignant changes may occur in these benign papillary growths. We believe that the original growth in the ureter was a benign papillomatosis, the more malignant tumor at the orifice represented a more advanced carcinomatous transformation occurring in these originally benign tumors. Glas maintains that, not infrequently in primary carcinoma of the ureter, the metastases may be simple papillomata that may grow and continue to exist as much, and then later degenerate into carcinom.atous formation. Renner in discussing cases presented by Israel, Poll and Player in which both malignant and non-malignant papillary growths were present states, "It is evident that, in the cases of which the authors speak, it must have been a question, either of primary multiple papillomata (papillomatosis), in which several of the villous tumors have already become cancerous, while in others their benign character has been retained; or it is also possible that subsequently and quite independent of the carcinoma, benign papilloma have developed. The process, assumed by Israel, Glas and Player, could not possibly be brought into agreement with the views which we hold in regard to malignant tumors." Case 2. H. B., a white female, sculptress, aged 76 years, entered the University Hospital September 30, 1935, complaining of recurrent pain in the left lower quadrant of the abdomen and in the left costovertebral angle. She stated that about August 1, 1935, she first noticed a backache on the left side in the region of the kidney. A few days later she suffered a severe colic on the same side and voided bloody urine. She entered a hospital elsewhere where a diagnosis of stricture of the ureter with calculus was made. About ten days later, following a series of attacks of colic, she thought she passed a small stone. Roentgen examination following these attacks did not reveal any evidence of a calculus. She had been comparatively comfortable except for backache on the left side, which has been controlled by mild sedatives until five days before she entered the hospital, when she experienced a severe colic, which was referred to the left lower quadrant and associated with nausea and vomiting. The patient did not notice blood in the urine during this attack and she entered the hospital for the specific purpose of having the stricture of the ureter dilated. She had lost about 10 pounds in weight.
PRIMARY PAPILLARY CARCINOMA OF URETER
509
The past medical and surgical history were negative. The physical examination throughout was essentially negative except for tenderness which was elicited in the left costovertebral angle and in the left lower quadrant about two inches from the left anterior superior spine of the ilium. A moderate cystocele was present. The blood pressure was 132 systolic, 68 diastolic. Urinalysis was negative except for a few red blood cells. A blood count revealed hemoglobin, 80 per cent, red blood cells, 4,195,000, white blood cells .3,950, polymorphonuclears 70 per cent, lymphocytes 27, monocytes 2, eosinophiles 1. A phenosulphonphthalein test showed 70 per cent excretion in two hours. Nonprotein nitrogen 22 mgm. The blood sedimentation rate was moderately rapid. Cystoscopic examination of the bladder showed some coarse trabeculation posterior to the trigone and a moderate cystocele. Both ureteral orifices were normal. The left ureter was catheterized easily for 15 cm. where an obstruction was met and passed with great difficulty. After repeated manipulations to pass the catheter no free blood or urine escaped from the catheter. The obstruction was finally passed and the fluid withdrawn from the kidney pelvis revealed a few pus cells and many red blood cells. No bacteria were found. The right ureter was catheterized easily. The urine from the right side was normal. Fhenosulphonphthalein given intravenously appeared in the right side in 4 minutes, and 22 per cent was recovered in 15 minutes. On the left side it appeared in 17 minutes and 5 per cent was recovered in 15 minutes. A pyelogram of the left side showed a very definite hydronephrosis and marked dilatation of the ureter in its upper half. At this point a very definite obstruction was evident. The column of sodium iodide in the_ upper part of the ureter seemed to fit over the dome of the obstructing mass in the ureter (fig. 5). Another x-ray examination did not reveal any evidence that this obstruction was due to stone. There was marked spasm which tightly gripped the catheter and a great deal of pain was experienced by the patient on attempts to move the catheter. The catheter was allowed to remain in place for drainage. Intravenous urography gave a normal pyelogram on the right side and a very faint shadow down to the point of obstruction on the left side. Our impressions following the examination of this patient were, first, that she had a functionless left kidney with a very marked obstruction in the ureter which we did not believe was due to a non shadow-casting stone, although the previous history indicated that she had passed a small calculus. Our attempts to pass a catheter did not give the impression that we were dealing with a stone. Secondly, the filling defect did give the impression that the column of sodium iodide in the upper part of the ureter was resting upon a smooth,
510
WM. N. TAYLOR AND C. A. KUEHN
dome-shaped surface. Although there was no marked free bleeding following our manipulations to pass a ureteral catheter, we made a preoperative diagnosis of a tumor of the ureter. At operation on August 8, 1935, a left nephrectomy was performed and the ureter was freed to the point of obstruction were a very definite nodule was
A FIG. 5. Case 2. moved.
B A, Showing filling defect; and B, gross specimen, one-half of tumor re-
palpated within the ureter. The ureter was doubly clamped below the nodule and severed. The specimen (fig. 5, B) was sent to the laboratory for diagnosis. A frozen section at this time revealed that it was malignant, and that our forceps had caught a portion of the tumor in the wall of the ureter. The patient was immediately turned upon her back and through a right rectus incision the remainder of the ureter was removed flush with the bladder.
PRIMARY PAPILLARY CARCINOMA OF URETER
511
Pathological report (Dr. H. L. Reinhart): Gross examination showed the specimen to consist of a kidney and ureter. The kidney was moderately hydronephrotic and the ureter is moderately dilated. The ureter measured 14 cm. in length. There is an S-shaped kink of the ureter at the ureteropelvic juncture. At the severed end of the ureter there was a smooth, firm mass of tissue broadly attached to the inner surface of the ureter which measures 1.75 cm. in length and 10 mm. in diameter. Cross section of the ureter at the site of the tumor showed the lumen to be practically obliterated. There was no evidence of ulceration or hemorrhage (fig. 6). Microscopic examination revealed a papillary carcinoma of the ureter
FIG. 6 FIG. 7 FIG. 6. Case 2. Low power photomicrograph of tumor in ureteral lumen FIG. 7. Case 2. Showing infiltration of ureteral wall
exhibiting marked anaplasia of the epithelial cells. Four mitotic figures per thousand cells and marked invasion of the ureteral wall were noted (fig. 7). Diagnosis: Papillary carcinoma of the ureter, Grade 3 malignancy. This patient made an excellent recovery following her operation. She left the hospital in good condition 14 days later. She has spent the summer as hostess to a tourist party in Alaska. This patient was not given x-ray therapy. This case presented a symptomatology of short duration (two months), in which a diagnosis of stricture of the ureter with stone had been made previously. She did not have the free bleeding through the ureteral catheter which has been noted by Chevassu and Mock as of diagnostic importance in tumors of the ureter. The tumor was firm, smooth with a broad base, definitely papillary, and invaded the ureteral wall. The diagnosis was suggested by the filling defect disclosed by the ureterogram. We believe that the firmness with which the ureteral catheter was grasped and the pain produced by attempts to move it are worthy of note and were of diagnostic significance.
512
WM. N. TAYLOR AND C. A. KUEHN
A review of the literature on tumors of the ureter is unnecessary, as contributions by Player, D'Aunoy and Zoeller, Rousselot and Lamon, Scott, and Lazarus have fully covered the subject and presented complete bibliographies and case abstracts. Five references are added which are not included in these reports. The incidence of the lesion is low. Renner found 4 primary tumors of the ureter in 13,854 autopsies at the Pathological Institute of Vienna. The records of 7,701 autopsies at Ohio State University do not record a case. Usually the tumor gives rise to symptoms that are severe enough to demand surgical interference regardless of a correct diagnosis before operation. These symptoms usually consist of pain, hematuria and occasionally a palpable mass. In the great majority of cases, the palpable mass is a hydronephrotic kidney. Since the symptomatology is in no way typical of the lesion, the diagnosis depends upon urological data. Three findings suggest the lesion: (1), tumors in or about the ureteral orifices; (2), ureteral obstruction which bleeds profusely on manipulation of the catheter; and (3), the most important, a filling defect shown on the ureterogram. The treatment is nephro-ureterectomy followed by x-ray therapy to the spine, liver, and lungs. SUMMARY
Two cases of primary papillary carcinoma 0f the ureter are recorded. A probable diagnosis was made in the first case because of the presence of a tumor in the ureteral orifice. In the second case, it was made because of a filling defect shown on the ureterogram. Both patients had complete nephro-ureterectomies at one operation. Both recovered and are living one and two years respectively. REFERENCES ARMITAGE, G. L.: Discussion of Hunter's case. Jour. Urol., 33: 460, 1935. COLSTON, J. A. C.: Primary tumors of the ureter with presentation of new method at complete nephro-ureterectomy. Trans. Amer. Assoc. Genito. Urin., Surg., 26: 41-66, 1933. D'AuNOY, R., AND ZOELLER, A.: Primary carcinoma of the ureter. Arch. Path., 9: 17-30, January, 1930. HARRAH, F. W.: Primary carcinoma of the ureter. Amer. Jour. Surg., 26: 550-560, 1934. HUNTER, A. W.: Primary tumor of the ureter. Jour. Urol., 33: 443-455, 1935. LAZARUS, J. A.: Primary tumors of the ureter with special reference to the malignant tumors. Ann. Surg., 99: 769-795, 1934. PAPIN, M.: Epithelioma of the ureter after nephrectomy for renal tuberculosis. Bull. Soc. Franc d Urol., pp. 258-264, June 17, 1935. PLAYER, L. P.: Primary ureteral carcinoma. Urol. and Cutan. Rev., 32: 438-444, 1928. RENNER, M. J.: Primary malignant tumors of the ureter. Surg., Gynec. and Obstet., 52: No. 4, 793-803, April, 1931. RoussELOT, L. M., AND LAMON, J. D.: Primary carcinoma of the ureter, case and review of literature. Surg., Gynec. and Obstet., 50: 17-28, 1930. ScoTT, A. W.: Primary carcinoma of the ureter. Surg., Gynec. and Obst., 58: 215-227, 1934.
AND
C. A. KUEHN
From the Department of Urology, Ohio State University
The rarity with which tumors of the ureter occur, the lack of a definite symptomatology, and the difficulty of diagnosis justify the reporting of all cases encountered. The literature at the present time records about 87 cases of maligrrant tumors of the ureter. Of these approximately 60 per cent are recorded as papillary growths. It is to this group that we wish to add two cases. Case 1. E. P., white male, 54 years of age, entered the University Hospital August 7, 1934. He stated that he had an attack of hematuria on April 6, 1934. The blood appeared in the urine without previous bladder symptoms or other provocation. Bleeding continued throughout the day and an acute retention developed due to a clot in the bladder. The patient was removed to a neighboring hospital where the retention was relieved and the clots removed. He remained in bed 5 days, at the end of which time the urine was clear and he was voiding normally. Six weeks later, he had another attack of hematuria which lasted 5 days. He was not inconvenienced by it in any way except for a moderate frequency of urination day and night. He did not consult his physician at this time. Two months following this second attack of bleeding, the urine was clear and his bladder symptoms had subsided. The day before he entered the hospital, he had a moderate hemorrhage associated with frequency of urination. He has only suffered frequency of urination during the attacks of bleeding. He has never complained of chills, fever, back pain, or abdominal discomfort and he considered himself in good health although he had lost about 10 pounds in wdght. The family and past history were irrelevant except for gonorrhea at the age of 29. Physical examination was negative except for many carious teeth and the surgical absence of the left radius, which was removed because of osteomyelitis. A moderate arteriosclerosis was present with a moderate hypertension. The blood pressure was 160 systolic and 82 diastolic. Examination of the abdomen revealed no tenderness or other abnormalities. Urinalysis showed a few red blood cells; otherwise it was normal. The blood count was within normal limits with a hemoglobin of 98 per cent. 1 Read before the North Central Branch of the American Urological Association, Cincinnati, Ohio, September 25-26, 1936.
504
PRIMARY PAPILLARY CARCINOMA OF URETER
505
Nonprotein nitrogen was 25 mgm. The blood sedimentation rate was moderately rapid. The blood Wassermann and Kahn gave negative reactions. Cystoscopic examination revealed a normal bladder except for a tumor covering or protruding from the left ureteral orifice. The tumor was conical in shape with the base over the orifice. In size, shape, and color it resembled a small ripe strawberry. The presence of the tumor made catheterization of the left ureter impossible. Indigo carmine did not appear at the left orifice and intravenous urography with a pyelogram did not show the left kidney. A plain film showed the left kidney to be slightly enlarged without evidence of calculus in the kidney or ureter. The urine, kidney function, and pyelograms on the right side were normal. Preoperatively, our impressions were that we were dealing with a tumor of the bladder that originated very close to the left ureteral meatus, or in the ureter and protruded through the ureteral meatus. However, the complete destruction of the kidney insofar as function and urograms were concerned led us to believe that there probably was more tumor in the ureter than that which was seen on cystoscopic examination. Operation was performed under spinal anesthesia on July 12, 1934. The bladder was first opened to determine the site of origin of the tumor. It originated in the ureter and protruded through the orifice. The lower part of the ureter was then investigated extraperitoneally. The tumor proved to be a markedly indurated and irregularly elongated mass firmly attached to the surrounding structures and extending from the base of the bladder to a point 2 inches above the bifurcation of the iliac artery. There was no apparent infiltration of tumor tissue into the surrounding structures, but it was densely adherent to them by what apparently was a chronic inflammatory process. With the cutting current, a wide section of bladder was taken about the tumor in the ureteral orifice. The ureter was freed as high as possible through the abdominal wound. The tumor mass was then securely wrapped in alcohol gauze and dropped back into the wound. The patient was then turned on his side and the left kidney together with the remainder of the ureter removed (fig. 1). Pathological report (Dr. H. L. Reinhart): "Upon gross examination the specimen consisted of a kidney, ureter, and a section of urinary bladder. The kidney was hydronephrotic and the upper half of the ureter was moderately dilated. The lower half of the ureter consisted of an irregularly elongated mass of firm consistency which measured 14 cm. in length and 3 cm. in diameter. Protruding from the ureteral orifice into the bladder was a firm meaty papillary tumor. "On cut section the hydronephrotic kidney and upper ureter were filled with a thin hemorrhagic fluid. The kidney tissue averaged 2 mm. in thick-
506
WM. N. TAYLOR AND C. A. KUEHN
ness. The mucosa of the kidney and upper ureter was thin and showed no evidence of neoplasm. The lower half of the ureter was the seat of a marked
FIG. 1
FIG.
2
Fm. 1. Case 1. Kidney removed at operation FIG. 2. Case 1. Cross section of kidney
papillary proliferation with considerable aseptic creamy exudate bathing the entire neoplasm. The two portions of the ureter were divided by sharp angulation, separating the neoplastic portion from the hydro-ureter (figs. 2 and 3).
PRIMARY PAPILLARY CARCINOMA OF URETER
507
"Microscopic examination of the tumor of the ureter revealed a papillary type of growth in which the connective tissue stalk was very delicate with areas of distortion of the vascular supply. There were numerous areas of necrosis apparently clue to the interrupted blood supply. There was also a subacute inflammatory exudate present. This, with the general conformation of the papillary stalks, gave the impression of an inflammatory papilloma; but there was a moderate degree of anaplasia of the epithelial cells with from 3 to 5 mitotic figures present per thousand cells, occasional areas exhibiting invasive characteristics. Microscopic examination of the portion of the tumor protruding from the ureteral orifice into the bladder revealed the same type of tumor but it is more malignant (fig. 4).
Frc. 3
Frc. 4
Frc. 3. Case 1. Low power photomicrograph of tumor in ureteral lumen FIG. 4. Case 1. High power photomicrograph of tumor in ureteral orifice
"Diagnosis: Papillary carcinoma of the ureter, Grade 2, malignancy." The convalescence was uneventful and the patient was discharged in excellent condition 27 days later. In the meantime he was given a course of deep x-ray therapy over the bladder, left kidney region, and spine. In reply to an inquiry June 1, 1936, two years after his operation, he stated that he had no urinary difficulties and that he was still able to work some, but was suffering from what his physician diagnosed as "heart trouble." The lower half of the ureter in this case presented a papillomatosis with low grade malignant changes. The tumor in the lower end of the ureter which protruded into the bladder presented a much higher grade
508
WM. N. TAYLOR AND C. A. KUEHN
of malignancy. Stoerk refers to papillomatosis due to a chronic inflammatory hyperplasia, which he believes should not be considered neoplastic. However, it is reasonable that malignant changes may occur in these benign papillary growths. We believe that the original growth in the ureter was a benign papillomatosis, the more malignant tumor at the orifice represented a more advanced carcinomatous transformation occurring in these originally benign tumors. Glas maintains that, not infrequently in primary carcinoma of the ureter, the metastases may be simple papillomata that may grow and continue to exist as much, and then later degenerate into carcinom.atous formation. Renner in discussing cases presented by Israel, Poll and Player in which both malignant and non-malignant papillary growths were present states, "It is evident that, in the cases of which the authors speak, it must have been a question, either of primary multiple papillomata (papillomatosis), in which several of the villous tumors have already become cancerous, while in others their benign character has been retained; or it is also possible that subsequently and quite independent of the carcinoma, benign papilloma have developed. The process, assumed by Israel, Glas and Player, could not possibly be brought into agreement with the views which we hold in regard to malignant tumors." Case 2. H. B., a white female, sculptress, aged 76 years, entered the University Hospital September 30, 1935, complaining of recurrent pain in the left lower quadrant of the abdomen and in the left costovertebral angle. She stated that about August 1, 1935, she first noticed a backache on the left side in the region of the kidney. A few days later she suffered a severe colic on the same side and voided bloody urine. She entered a hospital elsewhere where a diagnosis of stricture of the ureter with calculus was made. About ten days later, following a series of attacks of colic, she thought she passed a small stone. Roentgen examination following these attacks did not reveal any evidence of a calculus. She had been comparatively comfortable except for backache on the left side, which has been controlled by mild sedatives until five days before she entered the hospital, when she experienced a severe colic, which was referred to the left lower quadrant and associated with nausea and vomiting. The patient did not notice blood in the urine during this attack and she entered the hospital for the specific purpose of having the stricture of the ureter dilated. She had lost about 10 pounds in weight.
PRIMARY PAPILLARY CARCINOMA OF URETER
509
The past medical and surgical history were negative. The physical examination throughout was essentially negative except for tenderness which was elicited in the left costovertebral angle and in the left lower quadrant about two inches from the left anterior superior spine of the ilium. A moderate cystocele was present. The blood pressure was 132 systolic, 68 diastolic. Urinalysis was negative except for a few red blood cells. A blood count revealed hemoglobin, 80 per cent, red blood cells, 4,195,000, white blood cells .3,950, polymorphonuclears 70 per cent, lymphocytes 27, monocytes 2, eosinophiles 1. A phenosulphonphthalein test showed 70 per cent excretion in two hours. Nonprotein nitrogen 22 mgm. The blood sedimentation rate was moderately rapid. Cystoscopic examination of the bladder showed some coarse trabeculation posterior to the trigone and a moderate cystocele. Both ureteral orifices were normal. The left ureter was catheterized easily for 15 cm. where an obstruction was met and passed with great difficulty. After repeated manipulations to pass the catheter no free blood or urine escaped from the catheter. The obstruction was finally passed and the fluid withdrawn from the kidney pelvis revealed a few pus cells and many red blood cells. No bacteria were found. The right ureter was catheterized easily. The urine from the right side was normal. Fhenosulphonphthalein given intravenously appeared in the right side in 4 minutes, and 22 per cent was recovered in 15 minutes. On the left side it appeared in 17 minutes and 5 per cent was recovered in 15 minutes. A pyelogram of the left side showed a very definite hydronephrosis and marked dilatation of the ureter in its upper half. At this point a very definite obstruction was evident. The column of sodium iodide in the_ upper part of the ureter seemed to fit over the dome of the obstructing mass in the ureter (fig. 5). Another x-ray examination did not reveal any evidence that this obstruction was due to stone. There was marked spasm which tightly gripped the catheter and a great deal of pain was experienced by the patient on attempts to move the catheter. The catheter was allowed to remain in place for drainage. Intravenous urography gave a normal pyelogram on the right side and a very faint shadow down to the point of obstruction on the left side. Our impressions following the examination of this patient were, first, that she had a functionless left kidney with a very marked obstruction in the ureter which we did not believe was due to a non shadow-casting stone, although the previous history indicated that she had passed a small calculus. Our attempts to pass a catheter did not give the impression that we were dealing with a stone. Secondly, the filling defect did give the impression that the column of sodium iodide in the upper part of the ureter was resting upon a smooth,
510
WM. N. TAYLOR AND C. A. KUEHN
dome-shaped surface. Although there was no marked free bleeding following our manipulations to pass a ureteral catheter, we made a preoperative diagnosis of a tumor of the ureter. At operation on August 8, 1935, a left nephrectomy was performed and the ureter was freed to the point of obstruction were a very definite nodule was
A FIG. 5. Case 2. moved.
B A, Showing filling defect; and B, gross specimen, one-half of tumor re-
palpated within the ureter. The ureter was doubly clamped below the nodule and severed. The specimen (fig. 5, B) was sent to the laboratory for diagnosis. A frozen section at this time revealed that it was malignant, and that our forceps had caught a portion of the tumor in the wall of the ureter. The patient was immediately turned upon her back and through a right rectus incision the remainder of the ureter was removed flush with the bladder.
PRIMARY PAPILLARY CARCINOMA OF URETER
511
Pathological report (Dr. H. L. Reinhart): Gross examination showed the specimen to consist of a kidney and ureter. The kidney was moderately hydronephrotic and the ureter is moderately dilated. The ureter measured 14 cm. in length. There is an S-shaped kink of the ureter at the ureteropelvic juncture. At the severed end of the ureter there was a smooth, firm mass of tissue broadly attached to the inner surface of the ureter which measures 1.75 cm. in length and 10 mm. in diameter. Cross section of the ureter at the site of the tumor showed the lumen to be practically obliterated. There was no evidence of ulceration or hemorrhage (fig. 6). Microscopic examination revealed a papillary carcinoma of the ureter
FIG. 6 FIG. 7 FIG. 6. Case 2. Low power photomicrograph of tumor in ureteral lumen FIG. 7. Case 2. Showing infiltration of ureteral wall
exhibiting marked anaplasia of the epithelial cells. Four mitotic figures per thousand cells and marked invasion of the ureteral wall were noted (fig. 7). Diagnosis: Papillary carcinoma of the ureter, Grade 3 malignancy. This patient made an excellent recovery following her operation. She left the hospital in good condition 14 days later. She has spent the summer as hostess to a tourist party in Alaska. This patient was not given x-ray therapy. This case presented a symptomatology of short duration (two months), in which a diagnosis of stricture of the ureter with stone had been made previously. She did not have the free bleeding through the ureteral catheter which has been noted by Chevassu and Mock as of diagnostic importance in tumors of the ureter. The tumor was firm, smooth with a broad base, definitely papillary, and invaded the ureteral wall. The diagnosis was suggested by the filling defect disclosed by the ureterogram. We believe that the firmness with which the ureteral catheter was grasped and the pain produced by attempts to move it are worthy of note and were of diagnostic significance.
512
WM. N. TAYLOR AND C. A. KUEHN
A review of the literature on tumors of the ureter is unnecessary, as contributions by Player, D'Aunoy and Zoeller, Rousselot and Lamon, Scott, and Lazarus have fully covered the subject and presented complete bibliographies and case abstracts. Five references are added which are not included in these reports. The incidence of the lesion is low. Renner found 4 primary tumors of the ureter in 13,854 autopsies at the Pathological Institute of Vienna. The records of 7,701 autopsies at Ohio State University do not record a case. Usually the tumor gives rise to symptoms that are severe enough to demand surgical interference regardless of a correct diagnosis before operation. These symptoms usually consist of pain, hematuria and occasionally a palpable mass. In the great majority of cases, the palpable mass is a hydronephrotic kidney. Since the symptomatology is in no way typical of the lesion, the diagnosis depends upon urological data. Three findings suggest the lesion: (1), tumors in or about the ureteral orifices; (2), ureteral obstruction which bleeds profusely on manipulation of the catheter; and (3), the most important, a filling defect shown on the ureterogram. The treatment is nephro-ureterectomy followed by x-ray therapy to the spine, liver, and lungs. SUMMARY
Two cases of primary papillary carcinoma 0f the ureter are recorded. A probable diagnosis was made in the first case because of the presence of a tumor in the ureteral orifice. In the second case, it was made because of a filling defect shown on the ureterogram. Both patients had complete nephro-ureterectomies at one operation. Both recovered and are living one and two years respectively. REFERENCES ARMITAGE, G. L.: Discussion of Hunter's case. Jour. Urol., 33: 460, 1935. COLSTON, J. A. C.: Primary tumors of the ureter with presentation of new method at complete nephro-ureterectomy. Trans. Amer. Assoc. Genito. Urin., Surg., 26: 41-66, 1933. D'AuNOY, R., AND ZOELLER, A.: Primary carcinoma of the ureter. Arch. Path., 9: 17-30, January, 1930. HARRAH, F. W.: Primary carcinoma of the ureter. Amer. Jour. Surg., 26: 550-560, 1934. HUNTER, A. W.: Primary tumor of the ureter. Jour. Urol., 33: 443-455, 1935. LAZARUS, J. A.: Primary tumors of the ureter with special reference to the malignant tumors. Ann. Surg., 99: 769-795, 1934. PAPIN, M.: Epithelioma of the ureter after nephrectomy for renal tuberculosis. Bull. Soc. Franc d Urol., pp. 258-264, June 17, 1935. PLAYER, L. P.: Primary ureteral carcinoma. Urol. and Cutan. Rev., 32: 438-444, 1928. RENNER, M. J.: Primary malignant tumors of the ureter. Surg., Gynec. and Obstet., 52: No. 4, 793-803, April, 1931. RoussELOT, L. M., AND LAMON, J. D.: Primary carcinoma of the ureter, case and review of literature. Surg., Gynec. and Obstet., 50: 17-28, 1930. ScoTT, A. W.: Primary carcinoma of the ureter. Surg., Gynec. and Obst., 58: 215-227, 1934.