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Primary Carcinoma of Residual Ureter
THE JOURNAL OF UROLOGY
Vol. 72, No. 5, November 1954 Printed in U.S.A.
PRIMARY CARCINOMA OF RESIDUAL URETER ALEXANDER TAYLOR
AND
J. VICTOR BERRY
From the Section of Urology, Ottawa Civic Hospital, Ottawa, Canada
Primary carcinoma of a ureteral stump, residual to an infected hydronephrosis of congenital origin, is one of the rarest tumors encountered. The first incidence of such a condition was reported by Loef and Casella in 1952. We believe that the present report deals with two separate clinical entities and that this carcinoma of a residual ureter is the second case on record. CASE REPORT
H. B., a 57-year-old railroad engineer, was first seen by us in August 1947, complaining of severe intermittent lancinating pains in his right loin and right lower quadrant, radiating into his right testicle. Physical examination revealed rigidity of the muscles of the right upper quadrant with tenderness in the loin. Under sedation there was a suggestion of fullness in the affected loin and a mass in the renal area. There were no other abnormal findings. His previous history was interesting if not relevant. Nephritis had been diagnosed in 1918. An appendectomy had been performed in 1933 following an atypical attack of pain in the right lower quadrant with radiation to the right loin. Latent syphilis was also diagnosed at this time and inadequately treated. In 1945 neurological syphilis was diagnosed and apparently cured, his Wasserman reaction being reversed. A thyroidectomy for simple goiter was also performed in 1945. At this time (1947) numerous red blood cells and a few pus cells were noted in his urine. All other laboratory data were within normal limits. Cystoscopic examination revealed a normal bladder and prostatic urethra. Obstruction was encountered with a 5F ureteral catheter in the upper third of the right ureter at the 25 cm. mark. This was, however, successfully negotiated with a 4F catheter and the renal pelvis was drained for 24 hours. A skiogram revealed no calculi in relation to the right ureteral catheter (fig. 1, A). The patient refused further treatment and left the hospital. He was re-admitted in March 1948, complaining of marked aggravation of an intermittent dull ache which had been present in his right loin since he had left the hospital, and associated with anorexia and indigestion. Obstruction in the upper third of the right ureter was again encountered at the 25 cm. mark and found impossible to negotiate. Two days later a 4F catheter was successfully passed and a pyelogram was taken which revealed marked hydronephrosis of the right kidney (fig. 1, B). The left pyelogram was normal. A few red blood cells and pus cells were present in the urine from the right kidney. All urine smears and cultures were negative. At operation marked perinephritis was noted with an enormous extrarenal pelvis which was pressing on and adherent to the duodenum. Immediately below Read at annual meeting of Northeastern Section, American Urological Association, at Shawnee Inn, Pa. September 15, 1953. 817
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A. TAYLOR AND J. V. BERRY
the renal pelvis the first 2 cm. of the ureter were tortuous and cord-like and suggested a congenital stricture with marked peri-ureteritis. The ureter below the lesion appeared normal and a nephrectomy and partial ureterectomy were performed. Gross hydronephrosis and chronic pyelonephritis were the pathological diagnoses. An uneventful recovery was made, the patient expressing complete relief of indigestion as the most gratifying result of the operation. Thereafter, he enjoyed good health for 3½ years. In September 1951 he was again admitted to the hospital complaining of a dull ache in the right lower quadrant with an occasional sharp stabbing pain. Urgency and hematuria had also been noted. Cystoscopic examination revealed no bleeding areas in the bladder. A bulge of the right ureteral ridge was noted and a small fimbriated growth was seen protruding from the right meatus. The introduction of a ureteral catheter for a few centimeters resulted in profuse bleeding. A ureterogram revealed only minimal evidence of an intrinsic lesion (fig. 1, C). A number of deep tissue sections were taken from the region of the right ureteral ostia and a pathological diagnosis of transitional cell carcinoma of the ureter made. A ureterectomy was accordingly performed. The external surface of the specimen was not remarkable. When it was opened, however, numerous greyish-white soft implants of abnormal tissue were noted along the entire length of the specimen. Microscopic examination of these excrescences revealed that they were composed of a delicate, scant, vascular, fibrous stroma clothed by an exceedingly thick layer of transitional epithelial cells. These cells varied in size, shape and staining characteristics and a few
Fm. 1. A, pyelogram showing normal left renal pelvis. Right catheter is at point of obstruction with regurgitation of opaque medium down ureter. B, pyelogram demonstrating marked hydronephrosis with destruction of calyceal pattern. C, ureterogram of residual stump. Catheter introduced 10 cm.
CARCINOMA OF URETER
819
mitotic figures were noted. The tumors appeared to be relatively superficial, no invasion beyond the submucosa being demonstrable. Postoperative recovery was uneventful. The resection of the bladder wall proved inadequate but the recurrent growth was apparently controlled by transurethral resection. In March 1952 the patient complained of pain in the sternum and back and biopsy of a raised area of the sternum revealed metastatic carcinoma of the bone. He then received roentgen therapy which resulted in temporary relief but in June 1952 a generalized icterus was noted with vomiting and abdominal distention. On July 8, 1952, he died. Permission for an autopsy was refused. COMMENT
In the case here reported there was every reason to believe that the patient's first symptom of renal disease developed in 1933. The diagnosis of acute appendicitis was not confirmed at operation, and the history revealed that the patient had similar acute episodes during the next 14 years. In August 1947 an acute ureteral obstruction occurred due to congenital stenosis which had produced a pronounced peripelvi-ureteritis and complete occlusion. N ephrectomy and partial ureterectomy resulted in complete relief of a troublesome chronic gastric complaint. A 3½ year period of good health was suddenly interrupted by a bout of hematuria. A diagnosis of carcinoma of the ureteral stump was established by transurethral biopsy and the residual ureter was then removed. Metastatic lesions were discovered 6 months later and 4 months thereafter he died. The increasing number of case reports of carcinoma in the literature is due to an awareness on the part of the urologist of the importance of this entity and to improved diagnostic measures. It is believed that carcinoma of the ureter, both primary and metastatic, occurs more frequently than is yet appreciated. Soloway states that in 50 per cent of cases the condition is discovered at autopsy. The incidence of this tumor has been reported in the lower third of the right ureter in males of the sixth and seventh decade in 50 per cent of cases. The reports by Holmes, Long and many others indicate that hematuria is the symptom most frequently encountered. As in most intrinsic obstructive lesions of the ureter, pain is an associated symptom and hydronephrosis on the affected side is an inevitable sequelum. It is reasonable to conclude, therefore, that intrinsic obstructions of the ureter with resultant hydronephrosis, unless caused by stones, or strictures of various types, are due to tumors. These tumors are predominantly malignant. The treatment of these conditions where the clinical and radiological findings point to carcinoma of the ureter is obviated by the ability to perform a biopsy. It cannot be too strongly emphasized that a liberal portion of the bladder should be removed along with the ureter particularly where the intramural portion of the ureter is involved. In retrospect a cystotomy should have been performed in this case. Surgical treatment of primary carcinoma of the ureter has so far been de-
820
A. TAYLOR AND J. V. BERRY
cidedly discouraging, largely due to the fact that the growth has already metastasized although in the gross it appears well circumscribed. Soloway states that of the 149 reported cases in which operation was performed there are only 32 patients who were followed and reported as well. It would appear, therefore, that considerably more time and effort should be exerted in the direction of early diagnosis and treatment of a condition which presents such a high degree of malignancy and where the reported incidences of cures is so very low. SUMMARY
A case of primary carcinoma of a ureteral stump, presenting 3½ years after a nephrectomy and partial ureterectomy, is recorded. The diagnostic criteria of this condition are also discussed. This is the second case on record. Medical Arts Bldg., Ottawa 4, Ontario, Canada REFERENCES BROOKS, W. H.: J. Urol., 61: 29, 1949. FooRn, A. G. AND FERRIER, P. A.: J.A.M.A., 112: 596, 1939. GALBRAITH, W.W.: Brit. J. Urol., 22: 195, 1950. HARRISON, F. G., WARRES, H. L. AND FUST, J. A.: Ann. Surg., 130: 902, 1949. HOLMES, R. B.: Radiology, 66: 520, 1951 LAZARUS, J. A. AND MARKS, M. S.: J. Urol., 64: 140, 1945. LoEF, J. A. AND CASELLA, P.A.: J. Urol., 67: 159, 1952. LONG, J. H.: J. Urol., 61: 23, 1949. O'BRIEN, H. A.: J. Urol., 37: 49, 1937. SoLOWAY, H. M.: J. Internat. Coll. Surg., 16: 141, 1951. TAYLOR, J. A.: J. Urol., 66: 797, 1951. RAYER, P.: Traite de maladies des reins. Paris :J.B. Bailliere, 1841.
Vol. 72, No. 5, November 1954 Printed in U.S.A.
PRIMARY CARCINOMA OF RESIDUAL URETER ALEXANDER TAYLOR
AND
J. VICTOR BERRY
From the Section of Urology, Ottawa Civic Hospital, Ottawa, Canada
Primary carcinoma of a ureteral stump, residual to an infected hydronephrosis of congenital origin, is one of the rarest tumors encountered. The first incidence of such a condition was reported by Loef and Casella in 1952. We believe that the present report deals with two separate clinical entities and that this carcinoma of a residual ureter is the second case on record. CASE REPORT
H. B., a 57-year-old railroad engineer, was first seen by us in August 1947, complaining of severe intermittent lancinating pains in his right loin and right lower quadrant, radiating into his right testicle. Physical examination revealed rigidity of the muscles of the right upper quadrant with tenderness in the loin. Under sedation there was a suggestion of fullness in the affected loin and a mass in the renal area. There were no other abnormal findings. His previous history was interesting if not relevant. Nephritis had been diagnosed in 1918. An appendectomy had been performed in 1933 following an atypical attack of pain in the right lower quadrant with radiation to the right loin. Latent syphilis was also diagnosed at this time and inadequately treated. In 1945 neurological syphilis was diagnosed and apparently cured, his Wasserman reaction being reversed. A thyroidectomy for simple goiter was also performed in 1945. At this time (1947) numerous red blood cells and a few pus cells were noted in his urine. All other laboratory data were within normal limits. Cystoscopic examination revealed a normal bladder and prostatic urethra. Obstruction was encountered with a 5F ureteral catheter in the upper third of the right ureter at the 25 cm. mark. This was, however, successfully negotiated with a 4F catheter and the renal pelvis was drained for 24 hours. A skiogram revealed no calculi in relation to the right ureteral catheter (fig. 1, A). The patient refused further treatment and left the hospital. He was re-admitted in March 1948, complaining of marked aggravation of an intermittent dull ache which had been present in his right loin since he had left the hospital, and associated with anorexia and indigestion. Obstruction in the upper third of the right ureter was again encountered at the 25 cm. mark and found impossible to negotiate. Two days later a 4F catheter was successfully passed and a pyelogram was taken which revealed marked hydronephrosis of the right kidney (fig. 1, B). The left pyelogram was normal. A few red blood cells and pus cells were present in the urine from the right kidney. All urine smears and cultures were negative. At operation marked perinephritis was noted with an enormous extrarenal pelvis which was pressing on and adherent to the duodenum. Immediately below Read at annual meeting of Northeastern Section, American Urological Association, at Shawnee Inn, Pa. September 15, 1953. 817
818
A. TAYLOR AND J. V. BERRY
the renal pelvis the first 2 cm. of the ureter were tortuous and cord-like and suggested a congenital stricture with marked peri-ureteritis. The ureter below the lesion appeared normal and a nephrectomy and partial ureterectomy were performed. Gross hydronephrosis and chronic pyelonephritis were the pathological diagnoses. An uneventful recovery was made, the patient expressing complete relief of indigestion as the most gratifying result of the operation. Thereafter, he enjoyed good health for 3½ years. In September 1951 he was again admitted to the hospital complaining of a dull ache in the right lower quadrant with an occasional sharp stabbing pain. Urgency and hematuria had also been noted. Cystoscopic examination revealed no bleeding areas in the bladder. A bulge of the right ureteral ridge was noted and a small fimbriated growth was seen protruding from the right meatus. The introduction of a ureteral catheter for a few centimeters resulted in profuse bleeding. A ureterogram revealed only minimal evidence of an intrinsic lesion (fig. 1, C). A number of deep tissue sections were taken from the region of the right ureteral ostia and a pathological diagnosis of transitional cell carcinoma of the ureter made. A ureterectomy was accordingly performed. The external surface of the specimen was not remarkable. When it was opened, however, numerous greyish-white soft implants of abnormal tissue were noted along the entire length of the specimen. Microscopic examination of these excrescences revealed that they were composed of a delicate, scant, vascular, fibrous stroma clothed by an exceedingly thick layer of transitional epithelial cells. These cells varied in size, shape and staining characteristics and a few
Fm. 1. A, pyelogram showing normal left renal pelvis. Right catheter is at point of obstruction with regurgitation of opaque medium down ureter. B, pyelogram demonstrating marked hydronephrosis with destruction of calyceal pattern. C, ureterogram of residual stump. Catheter introduced 10 cm.
CARCINOMA OF URETER
819
mitotic figures were noted. The tumors appeared to be relatively superficial, no invasion beyond the submucosa being demonstrable. Postoperative recovery was uneventful. The resection of the bladder wall proved inadequate but the recurrent growth was apparently controlled by transurethral resection. In March 1952 the patient complained of pain in the sternum and back and biopsy of a raised area of the sternum revealed metastatic carcinoma of the bone. He then received roentgen therapy which resulted in temporary relief but in June 1952 a generalized icterus was noted with vomiting and abdominal distention. On July 8, 1952, he died. Permission for an autopsy was refused. COMMENT
In the case here reported there was every reason to believe that the patient's first symptom of renal disease developed in 1933. The diagnosis of acute appendicitis was not confirmed at operation, and the history revealed that the patient had similar acute episodes during the next 14 years. In August 1947 an acute ureteral obstruction occurred due to congenital stenosis which had produced a pronounced peripelvi-ureteritis and complete occlusion. N ephrectomy and partial ureterectomy resulted in complete relief of a troublesome chronic gastric complaint. A 3½ year period of good health was suddenly interrupted by a bout of hematuria. A diagnosis of carcinoma of the ureteral stump was established by transurethral biopsy and the residual ureter was then removed. Metastatic lesions were discovered 6 months later and 4 months thereafter he died. The increasing number of case reports of carcinoma in the literature is due to an awareness on the part of the urologist of the importance of this entity and to improved diagnostic measures. It is believed that carcinoma of the ureter, both primary and metastatic, occurs more frequently than is yet appreciated. Soloway states that in 50 per cent of cases the condition is discovered at autopsy. The incidence of this tumor has been reported in the lower third of the right ureter in males of the sixth and seventh decade in 50 per cent of cases. The reports by Holmes, Long and many others indicate that hematuria is the symptom most frequently encountered. As in most intrinsic obstructive lesions of the ureter, pain is an associated symptom and hydronephrosis on the affected side is an inevitable sequelum. It is reasonable to conclude, therefore, that intrinsic obstructions of the ureter with resultant hydronephrosis, unless caused by stones, or strictures of various types, are due to tumors. These tumors are predominantly malignant. The treatment of these conditions where the clinical and radiological findings point to carcinoma of the ureter is obviated by the ability to perform a biopsy. It cannot be too strongly emphasized that a liberal portion of the bladder should be removed along with the ureter particularly where the intramural portion of the ureter is involved. In retrospect a cystotomy should have been performed in this case. Surgical treatment of primary carcinoma of the ureter has so far been de-
820
A. TAYLOR AND J. V. BERRY
cidedly discouraging, largely due to the fact that the growth has already metastasized although in the gross it appears well circumscribed. Soloway states that of the 149 reported cases in which operation was performed there are only 32 patients who were followed and reported as well. It would appear, therefore, that considerably more time and effort should be exerted in the direction of early diagnosis and treatment of a condition which presents such a high degree of malignancy and where the reported incidences of cures is so very low. SUMMARY
A case of primary carcinoma of a ureteral stump, presenting 3½ years after a nephrectomy and partial ureterectomy, is recorded. The diagnostic criteria of this condition are also discussed. This is the second case on record. Medical Arts Bldg., Ottawa 4, Ontario, Canada REFERENCES BROOKS, W. H.: J. Urol., 61: 29, 1949. FooRn, A. G. AND FERRIER, P. A.: J.A.M.A., 112: 596, 1939. GALBRAITH, W.W.: Brit. J. Urol., 22: 195, 1950. HARRISON, F. G., WARRES, H. L. AND FUST, J. A.: Ann. Surg., 130: 902, 1949. HOLMES, R. B.: Radiology, 66: 520, 1951 LAZARUS, J. A. AND MARKS, M. S.: J. Urol., 64: 140, 1945. LoEF, J. A. AND CASELLA, P.A.: J. Urol., 67: 159, 1952. LONG, J. H.: J. Urol., 61: 23, 1949. O'BRIEN, H. A.: J. Urol., 37: 49, 1937. SoLOWAY, H. M.: J. Internat. Coll. Surg., 16: 141, 1951. TAYLOR, J. A.: J. Urol., 66: 797, 1951. RAYER, P.: Traite de maladies des reins. Paris :J.B. Bailliere, 1841.