- Email: [email protected]
Primary Carcinoma of the Ureter1
THE JOURNAL OF UROLOGY
Vol. 61, No. 1, January, 1949
Printed in U.S.A.
PRIJ\1IARY CARCINOMA OF THE URETER1 .JOHN HERMAN LONG
The increased frequency with ·which reports of primary carcinoma of the ureter have appeared in the medical literature during the past 15 years probably does not indicate an actual increase in the frequency of the condition, but rather increased frequency of recognition of the disease. As Scott has pointed out, this is due to improvements in our diagnostic armamentarium and methods, and to the greater number of well trained urologists in practice throughout the country. One great fault in most of the reported cases and summaries of the cases in the literature is the lack of sufficient data regarding the follow-up, so that it is difficult to compile definite statistics concerning the condition. Another difficulty in correlating the reported cases has been the ·wide variation in the pathological nomenclature. Scott reported 22 different tumor types described in the literature. Lazarus reported 15 pathological types. It would seem more logical to limit the type of malignant tumors to two: epidermoid or solid, and papillary carcinoma. In 1943 Scott summarized the 182 cases on record at that time, and presented. an excellent review of the literature with a comprehensive bibliography. Since that time there have been reports of 19 other cases, bringing the total to 201 cases. In analyzing the various reports the following points have been brought out: 1) History: The first reported case was by Rayer in 1841. The first correct preoperative diagnosis ·was made by Albarran in 1902. 2) Sex: The condition is twice as frequent in male as in female patients. 3) Age: More than half the cases occur in the sixth and seventh decades of life. The youngest case was 22, the oldest 89 years of age. 4) Location: The right ureter is affected about twice as often as the left. The lower third of the ureter is the site of the growth twice as frequently as the upper two-thirds. 5) Symptoms: Characteristic triad: A. Hematuria, in 70 per cent; may be profuse, microscopic, early, late, recurrent, or constant. Ureteral blood clot casts may be passed. B. Pain, in 64 per cent; due to (a) ureteral obstruction from tumor, or blood clot; or (b) involvement of nearby or remote organs by metastases or extension. C. Tumor, in 40 per cent; due to (a) enlarged kidney secondary to obstruction by tumor; (b) ureteral tumor felt abdominally, rectally, or vaginally; or (c) enlarged lymph nodes. 6) Urine: May be grossly bloody or clear, even microscopically. May contain tumor cells. 7) Cystoscopy may reveal: 1 Read at annual meeting, Mid-Atlantic Section American Urological Association, Hot Springs, Va., March 18, 1948.
23
24
JOHN HERMAN LONG
A. B. C. D. E.
8)
9)
10) 11) 12)
13)
Tumor projecting from ureteral orifice in about 25 per cent. Bulge in ureteral ridge. Edema and congestion of ureteral orifice. Trickle of blood from orifice after vaginal or rectal manipulation. Obstruction, either complete, or a mushy resiliency on attempted passage of catheter. F. Decrease in function on involved side. Roentgenographic studies may reveal: A. Hydronephrosis on affected side. B. Goblet shaped filling defect in region of tumor. C. Metastases in bones or chest. Treatment: Treatment of choice is extraperitoneal nephro-ureterectomy with segmental resection of bladder. If necessary to do operation in 2 stages, remove tumor at first operation. Be certain tumor is malignant (frozen section if in doubt) before radical resection is done. Pre- and postoperative radiation probably of little value. Pathology: There is great confusion over nomenclature but probably 60 per cent of the tumors are papillary and 40 per cent are solid carcinomata. Metastases: 34 per cent. Most frequent sites are retroperitoneal lymph nodes, liver, bones, and lungs. Results: Confusing because of inadequate follow-up. A. Postoperative deaths, about 25 per cent. B. No patient living more than 4 years postoperative died of metastases. C. Of 40 patients reported well only 14 were followed 4 or more years. One case was well 13 years; one, 12; one, 11; one, 9; two, 8; two, 7; and one, 6 years after operation. Follow-up: The patient should be examined every 3 months the first year after operation, every 6 months the second, third and fourth years, and yearly thereafter. The examination should consist of a general physical examination, pelvic or rectal examination, cystoscopic examination, and x-ray of lumbar spine and pelvis. CASE REPORT
An unmarried 61 year old woman vrns first seen in consultation on May 12, 1941, complaining of hematuria. She had never had any urinary complaints until the sudden appearance 24 hours previously of painless, gross hematuria which had persisted, and was unaccompanied by other urinary symptoms. The past history was relatively unimportant. There had never been any urinary disturbances. The menstrual periods had ceased in about 1920 after a normal menstrual life. She had had moderately advanced hypertension for 30 years, but had never had symptoms from it. The physical examination was essentially normal. The patient was moderately obese. The blood pressure was 200/100. There was no abdominal or costovertebral angle tenderness and no masses. Rectal examination revealed no abnormalities. The catheterized urine was grossly bloody; the culture was sterile. The 2 hour phenolsulphonphthalein function
PRIMARY CARCINOMA OF URE'l'ER
25
test revealed only 50 per cent excretion. The excretion curve showed 15, 10, 10 and 15 per cent in 15, 30, 60 and 120 minutes. The blood nonprotein nitrogen was 32 mg. per cent. An intravenous urogram taken on May 13, 1941, showed a normal right, and an imperfectly filled left kidney. The ureters were not visualized. Cystoscopic examination on May 14, 1941 revealed grossly bloody urine and a normal bladder. Neither urine nor blood was seen emerging from either ureteral orifice. The left orifice was catheterized with a No. 7 pointed tip catheter. Mild obstruction ,vas encountered just inside the ureteral orifice and the catheter passed all the way up the ureter with some difficulty. Clear urine drained from the catheter, a culture of which was negative. A plain x-ray was normaL A
FIG. 1
pyelo-ureterogram using 37 cc of opaque medium showed a large hydronephrosis with dilated, blunted calyces. The ureter was moderately dilated to a point just above the bladder where there was an acute narrowing for a few centimeters; then a moderately dilated area below this (fig. 1). A diagnosis of ureteral tumor was made. There was no gross hematuria after this examination. On May 23, and June 3, 1941 attempts were made to catheterize the left ureteral orifice to obtain a differential phenolsulphonphthalein function test, but each time the tip of the catheter obstructed 1~2 cm. inside the orifice and did not drain. It was thought that there was enough evidence of a ureteral tumor to warrant an exploratory operation. On June 10, 1941 the lower ureter was explored through a left gridiron, extraperitoneal incision. The ureter was normal as it emerged from the bladder. Approximately 1.0 cm. above this point the ureter became dilated and for about 5.0 cm. was filled with a solid tumor. There was no evidence of extension of the tumor through the ureteral wall. Above the tumor area the ureter became dilated and thin walled, and the lumen was free of palpable tumor mass. The ureter was resected from the bladder with a small margin of bladder wall; then freed upward to the kidney. The kidney was
FIG. 2
FIG. 3
26
PRIMARY CARCINOMA OF URETER
Frn.4
27
28
JOHN HERMAN LONG
only slightly enlarged; the dilated pelvis was extrarenal, and the kidney pedicle was unusually long. After the kidney was freed by blunt dissection it was brought down into the gridiron incision so that the pedicle was exposed, clamped, ligated, and cut, and the entire kidney and ureter removed. The postoperative course was uneventful. The blood pressure fell to near normal after the operation, but as soon as the patient became ambulatory it rose to the former level, where it has remained since. It has not increased since the operation, however. The gross specimen revealed the tumor mass occupying a space of about 5.0 cm. in the lower ureter. The ureter was about 2.0 cm. in diameter at this point and above this area was dilated and thin walled, but the mucosa above the tumor was normal. The kidney pelvis was dilated. The renal substance showed some scarring. Microscopic sections of the tumor showed a papillary carcinoma. Numerous sections through the tumor revealed no invasion of the ureteral wall (figs. 2, 3, 4). For the first year following the operation the patient was examined every 3 months, and the next 2 years at 6 month intervals. The last examination, until recently, was 4 years after the operation. The last examination was on February 28, 1948. There has been no evidence of recurrence of the tumor as evidenced by abdominal mass, pelvic mass or induration, and cystoscopic examination has revealed a normal bladder. In conclusion, a case of primary carcinoma of the ureter has been presented. The preoperative x-ray findings suggested a ureteral tumor. At the time of operation there was no gross evidence of extension or metastases. The entire ureter and kidney were removed through an extraperitoneal lower gridiron inc1s10n. The patient has remained well and has had no evidence of recurrence of the carcinoma after being followed 6 years and 10 months. SUMMARY
A case of primary carcinoma of the ureter has been presented. It is unusual in only one respect. The patient has been followed closely for nearly 7 years after the original operation and is alive and well, with no evidence of recurrence. This is the tenth case that has been followed more than 6 years. The literature on the condition has been reviewed and summarized and the bibliography is brought up to date.
11 East Chase St., Baltimore 2, Md. REFERENCES Bowrn, C. F. AND BoRs, E.: J. Urol., 54: 434, 1945. CARAVEN, J.: J. d'urol., 52: 11, 1944. CASE REPORT: New Eng. J. Med.: 235: 337, 1946. CouNSELLER, V. S., CooK, E. N. AND SEEFIELD, P.H.: J. Urol., 51: 606, 1944. DE LA PENA, A. AND DE LA PENA, E.: Urol. and Cutan. Rev., 50: 667, 1946. GUALTIERI, T., HAYES, J. AND SEGAL, A., J. Urol., 59: 1083, 1948. HUNDLEY, J.M. AND HUNTER, J. S., J. Urol., 58: 176, 1947. JAFFE, S. A. AND MENDILLO, A. J.: Am. J. Surg., 62: 126, 1943. LAZARUS, J. A. AND MARKS, M. S.: J. Urol., 54: 140, 1945. LEPOUTRE, C.: J. d'urol., 52: 143, 1944-45. McCLELLAND, J.C.: J. Urol., 52: 522, 1944. REGAN, J. R.: J. Urol., 50: 304, 1943. ScoTT, W.W.: J. Urol., 50: 45, 1943. VAN GuLIK, F. H.: Nederl. tijdschr. v. geneesk., 90: 503, 1946. VEST, S. A.: J. Urol., 53: 97, 1945.
Vol. 61, No. 1, January, 1949
Printed in U.S.A.
PRIJ\1IARY CARCINOMA OF THE URETER1 .JOHN HERMAN LONG
The increased frequency with ·which reports of primary carcinoma of the ureter have appeared in the medical literature during the past 15 years probably does not indicate an actual increase in the frequency of the condition, but rather increased frequency of recognition of the disease. As Scott has pointed out, this is due to improvements in our diagnostic armamentarium and methods, and to the greater number of well trained urologists in practice throughout the country. One great fault in most of the reported cases and summaries of the cases in the literature is the lack of sufficient data regarding the follow-up, so that it is difficult to compile definite statistics concerning the condition. Another difficulty in correlating the reported cases has been the ·wide variation in the pathological nomenclature. Scott reported 22 different tumor types described in the literature. Lazarus reported 15 pathological types. It would seem more logical to limit the type of malignant tumors to two: epidermoid or solid, and papillary carcinoma. In 1943 Scott summarized the 182 cases on record at that time, and presented. an excellent review of the literature with a comprehensive bibliography. Since that time there have been reports of 19 other cases, bringing the total to 201 cases. In analyzing the various reports the following points have been brought out: 1) History: The first reported case was by Rayer in 1841. The first correct preoperative diagnosis ·was made by Albarran in 1902. 2) Sex: The condition is twice as frequent in male as in female patients. 3) Age: More than half the cases occur in the sixth and seventh decades of life. The youngest case was 22, the oldest 89 years of age. 4) Location: The right ureter is affected about twice as often as the left. The lower third of the ureter is the site of the growth twice as frequently as the upper two-thirds. 5) Symptoms: Characteristic triad: A. Hematuria, in 70 per cent; may be profuse, microscopic, early, late, recurrent, or constant. Ureteral blood clot casts may be passed. B. Pain, in 64 per cent; due to (a) ureteral obstruction from tumor, or blood clot; or (b) involvement of nearby or remote organs by metastases or extension. C. Tumor, in 40 per cent; due to (a) enlarged kidney secondary to obstruction by tumor; (b) ureteral tumor felt abdominally, rectally, or vaginally; or (c) enlarged lymph nodes. 6) Urine: May be grossly bloody or clear, even microscopically. May contain tumor cells. 7) Cystoscopy may reveal: 1 Read at annual meeting, Mid-Atlantic Section American Urological Association, Hot Springs, Va., March 18, 1948.
23
24
JOHN HERMAN LONG
A. B. C. D. E.
8)
9)
10) 11) 12)
13)
Tumor projecting from ureteral orifice in about 25 per cent. Bulge in ureteral ridge. Edema and congestion of ureteral orifice. Trickle of blood from orifice after vaginal or rectal manipulation. Obstruction, either complete, or a mushy resiliency on attempted passage of catheter. F. Decrease in function on involved side. Roentgenographic studies may reveal: A. Hydronephrosis on affected side. B. Goblet shaped filling defect in region of tumor. C. Metastases in bones or chest. Treatment: Treatment of choice is extraperitoneal nephro-ureterectomy with segmental resection of bladder. If necessary to do operation in 2 stages, remove tumor at first operation. Be certain tumor is malignant (frozen section if in doubt) before radical resection is done. Pre- and postoperative radiation probably of little value. Pathology: There is great confusion over nomenclature but probably 60 per cent of the tumors are papillary and 40 per cent are solid carcinomata. Metastases: 34 per cent. Most frequent sites are retroperitoneal lymph nodes, liver, bones, and lungs. Results: Confusing because of inadequate follow-up. A. Postoperative deaths, about 25 per cent. B. No patient living more than 4 years postoperative died of metastases. C. Of 40 patients reported well only 14 were followed 4 or more years. One case was well 13 years; one, 12; one, 11; one, 9; two, 8; two, 7; and one, 6 years after operation. Follow-up: The patient should be examined every 3 months the first year after operation, every 6 months the second, third and fourth years, and yearly thereafter. The examination should consist of a general physical examination, pelvic or rectal examination, cystoscopic examination, and x-ray of lumbar spine and pelvis. CASE REPORT
An unmarried 61 year old woman vrns first seen in consultation on May 12, 1941, complaining of hematuria. She had never had any urinary complaints until the sudden appearance 24 hours previously of painless, gross hematuria which had persisted, and was unaccompanied by other urinary symptoms. The past history was relatively unimportant. There had never been any urinary disturbances. The menstrual periods had ceased in about 1920 after a normal menstrual life. She had had moderately advanced hypertension for 30 years, but had never had symptoms from it. The physical examination was essentially normal. The patient was moderately obese. The blood pressure was 200/100. There was no abdominal or costovertebral angle tenderness and no masses. Rectal examination revealed no abnormalities. The catheterized urine was grossly bloody; the culture was sterile. The 2 hour phenolsulphonphthalein function
PRIMARY CARCINOMA OF URE'l'ER
25
test revealed only 50 per cent excretion. The excretion curve showed 15, 10, 10 and 15 per cent in 15, 30, 60 and 120 minutes. The blood nonprotein nitrogen was 32 mg. per cent. An intravenous urogram taken on May 13, 1941, showed a normal right, and an imperfectly filled left kidney. The ureters were not visualized. Cystoscopic examination on May 14, 1941 revealed grossly bloody urine and a normal bladder. Neither urine nor blood was seen emerging from either ureteral orifice. The left orifice was catheterized with a No. 7 pointed tip catheter. Mild obstruction ,vas encountered just inside the ureteral orifice and the catheter passed all the way up the ureter with some difficulty. Clear urine drained from the catheter, a culture of which was negative. A plain x-ray was normaL A
FIG. 1
pyelo-ureterogram using 37 cc of opaque medium showed a large hydronephrosis with dilated, blunted calyces. The ureter was moderately dilated to a point just above the bladder where there was an acute narrowing for a few centimeters; then a moderately dilated area below this (fig. 1). A diagnosis of ureteral tumor was made. There was no gross hematuria after this examination. On May 23, and June 3, 1941 attempts were made to catheterize the left ureteral orifice to obtain a differential phenolsulphonphthalein function test, but each time the tip of the catheter obstructed 1~2 cm. inside the orifice and did not drain. It was thought that there was enough evidence of a ureteral tumor to warrant an exploratory operation. On June 10, 1941 the lower ureter was explored through a left gridiron, extraperitoneal incision. The ureter was normal as it emerged from the bladder. Approximately 1.0 cm. above this point the ureter became dilated and for about 5.0 cm. was filled with a solid tumor. There was no evidence of extension of the tumor through the ureteral wall. Above the tumor area the ureter became dilated and thin walled, and the lumen was free of palpable tumor mass. The ureter was resected from the bladder with a small margin of bladder wall; then freed upward to the kidney. The kidney was
FIG. 2
FIG. 3
26
PRIMARY CARCINOMA OF URETER
Frn.4
27
28
JOHN HERMAN LONG
only slightly enlarged; the dilated pelvis was extrarenal, and the kidney pedicle was unusually long. After the kidney was freed by blunt dissection it was brought down into the gridiron incision so that the pedicle was exposed, clamped, ligated, and cut, and the entire kidney and ureter removed. The postoperative course was uneventful. The blood pressure fell to near normal after the operation, but as soon as the patient became ambulatory it rose to the former level, where it has remained since. It has not increased since the operation, however. The gross specimen revealed the tumor mass occupying a space of about 5.0 cm. in the lower ureter. The ureter was about 2.0 cm. in diameter at this point and above this area was dilated and thin walled, but the mucosa above the tumor was normal. The kidney pelvis was dilated. The renal substance showed some scarring. Microscopic sections of the tumor showed a papillary carcinoma. Numerous sections through the tumor revealed no invasion of the ureteral wall (figs. 2, 3, 4). For the first year following the operation the patient was examined every 3 months, and the next 2 years at 6 month intervals. The last examination, until recently, was 4 years after the operation. The last examination was on February 28, 1948. There has been no evidence of recurrence of the tumor as evidenced by abdominal mass, pelvic mass or induration, and cystoscopic examination has revealed a normal bladder. In conclusion, a case of primary carcinoma of the ureter has been presented. The preoperative x-ray findings suggested a ureteral tumor. At the time of operation there was no gross evidence of extension or metastases. The entire ureter and kidney were removed through an extraperitoneal lower gridiron inc1s10n. The patient has remained well and has had no evidence of recurrence of the carcinoma after being followed 6 years and 10 months. SUMMARY
A case of primary carcinoma of the ureter has been presented. It is unusual in only one respect. The patient has been followed closely for nearly 7 years after the original operation and is alive and well, with no evidence of recurrence. This is the tenth case that has been followed more than 6 years. The literature on the condition has been reviewed and summarized and the bibliography is brought up to date.
11 East Chase St., Baltimore 2, Md. REFERENCES Bowrn, C. F. AND BoRs, E.: J. Urol., 54: 434, 1945. CARAVEN, J.: J. d'urol., 52: 11, 1944. CASE REPORT: New Eng. J. Med.: 235: 337, 1946. CouNSELLER, V. S., CooK, E. N. AND SEEFIELD, P.H.: J. Urol., 51: 606, 1944. DE LA PENA, A. AND DE LA PENA, E.: Urol. and Cutan. Rev., 50: 667, 1946. GUALTIERI, T., HAYES, J. AND SEGAL, A., J. Urol., 59: 1083, 1948. HUNDLEY, J.M. AND HUNTER, J. S., J. Urol., 58: 176, 1947. JAFFE, S. A. AND MENDILLO, A. J.: Am. J. Surg., 62: 126, 1943. LAZARUS, J. A. AND MARKS, M. S.: J. Urol., 54: 140, 1945. LEPOUTRE, C.: J. d'urol., 52: 143, 1944-45. McCLELLAND, J.C.: J. Urol., 52: 522, 1944. REGAN, J. R.: J. Urol., 50: 304, 1943. ScoTT, W.W.: J. Urol., 50: 45, 1943. VAN GuLIK, F. H.: Nederl. tijdschr. v. geneesk., 90: 503, 1946. VEST, S. A.: J. Urol., 53: 97, 1945.