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Unusual Causes of Extrinsic Ureteral Obstruction, Part II
THE JOURNAL OF UROLOGY
Vol. 87, No. 3 March 1962 Copyright © 1962 by The Williams & Wilkins Co.
Ptinted in U.S.A.
UNUSUAL CAUSES OF EXTRINSIC URETERAL OBSTRUCTION, PAllT II JOSEPH J. KAUFMA~ From the Department of Surgery, Division of Urology, University of California il1eclical Center, Los Angeles, and the Urological Service, Wadsworth General Hospital, Veterans Administration Center, Los Angeles 24, Cal.
In part I (p. 319 of this issue) uncommon non-neoplastic conditions which can produce obstruction of the ureters ,vere discussed. This paper will deal with examples of primary and secondary retroperitoneal tumors involving or compressing the ureter ancl causing hydronephrosis. PRIMARY RETROPERITONEAL TUMORS INVOLVING THE URETER
Retroperitoneal reticulum cell sarcoma producing amiria; case report. G. G. (GSH Ko. 60-7621), a patient of Dr. Elmer Belt, was a 62-year-old white man who was admitted to the hospital with anuria of 4 days' duration. Cholecystectomy 6 weeks previously was followed by an uneventful course except for oliguria, beginning approximately 2 weeks following the operation. At that time his nonprotein nitrogen was 178. On a large fluid intake this dropped to 94, and he was sent home. However, 4 days prior to admission to the hospital he became totally anuric, which was followed by peripheral edema, orthopnea, dysnea, and mental confusion. He appeared cachectic. Pitting edema was present over both ankles and sacrum. A hard, nontender mass filled the entire right upper quadrant and did not appear to be related to the wound. Ureteral catheters were passed and immediate diuresis occurred with outputs averaging 3,000 cc daily. Retrograde pyelograms done subsequently showed bilateral hydronephrosis and hydroureter down to the pelvic brim (fig. 1). Despite his clinical improvement from urinary drainage, he died and at autopsy, an extensive retroperitoneal reticulum cell sarcoma was found with fixation to the aorta, to the inferior vena cava, and to the ureters. There was no direct involvement of the ureteral muscular wall by tumor, but there was acute interstitial ureteritis and localized stenosis of the ureters,
Accepted for publication July 21, 1961. Read at annual meeting of Western Section of American Urological Association, Inc., Las Vegas, Nevada, February 6-9, 1961.
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with angulation of the upper portion of the left ureter. The ureters were easily dissected free from the retroperitoneal sarcoma. Microscopic study of the retroperitoneal tumor showed a densely cellular, malignant neoplasm in which the dominant cell type was the reticulum cell. The ureters at multiple levels examined were partly found to be completely surrounded by tumor with only occasional microscopic foci of involvement of the ureteral musculature proper. LYMPHOSARCOMA PRODUCING URETERAL OBSTRUCTION
Case report. E. K. (GH No. 2270), a 65-yearold white man, was admitted to the Veterans Administration Hospital, Los Angeles, complaining of right flank pain of 1 week's duration. For several weeks there had been nausea, fever, and chills. There had been a 15 to 20 pound weight loss in the preceding 3 months despite a good appetite. Examination showed a chronically ill man, who had a questionable palpable right flank mass and definite tenderness of the right costovertebral angle. The hemoglobin was 13.1 and the creatinine ,ms 2.2. Intravenous urography revealed a moderately dilated right urinary collecting system down to the bony pelvis where there was an abrupt cut-off (fig. 2). Cystoscopy and ureteral catheterization were attempted but the catheter could not be advanced beyond 6 cm. on the right. Retrograde injection of contrast medium failed to fill the ureter beyond this point. The presumptive diagnosis was an extrinsic lesion compressing the right ureter. The lower ureter was explored through a Gibson incision, and a fusiform firm swelling of the ureter vms found 6 cm. above the bladder. The swelling measured approximately 6 cm. in length and 3 to 4 cm. in diameter. With only slight difficulty, the ureter with the bulbous swelling was dissected from the surrounding structures. The ureter distal to the swelling was normal, whereas above the swelling it was dilated and tense. Nephro-ureterectomy was done
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Fm. 1. Retrograde pyelograms show bilateral hydronephrosis caused by ureteral compression and invasion secondary to primary retroperitoneal reticulum cell sarcoma. (fig. 3, A). Section of the ureter at the area of the bulbous swelling showed it to be encased by a discrete periureteral tumor (lymphosarcoma). The wall of the ureter was not directly invaded by the lymphosarcoma, but the tumor was closely applied to the outside of the ureter (fig. 3, B). This patient had a satisfactory postoperative course and was subsequently treated with deep radiation therapy to the right lower quadrant through 4 portals. He was doing well 5 months postoperatively and 4 months after radiation therapy was completed. HODGKIN'S DISEASE WITH LEFT URETERAL OBSTRUCTION
This patient is presented through the courtesy of Dr. Robert 0. Pearman. Case report. A 36-year-old white man entered the hospital complaining of a left flank tumor of 3 months' duration. A diagnosis of diffuse Hodgkin's disease was made 4 years earlier from nodes excised from the left axilla. He had been previously treated with x-ray therapy and was then well until 3 months prior to admission, at which time he noted a dull aching pain in the left flank with subsequent appearance of a bulging mass in the left flank. On physical examination, the abdomen was moderately tender and a mass the size of a grapefruit in the left flank was found to extend to the midline. Urinalysis was negative, but an intravenous
Fm. 2. Intravenous urogram shows ureteral obstruction causing right hydronephrosis. urogram disclosed no function of the left kidney. A retrograde pyelogram was made showing hydronephrosis and hydroureter on the left extending down to a point several centimeters above the bladder (fig. 4). There was marked lateral displacement of the left kidney and upper
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FIG. 3. A, specimen (resected kidney and ureter) shows bulbous swelling of lower ureter. B, crosssection of ureter at level of swelling shows lymphosarcoma.
ureter, with medial displacement of the lower ureter. The patient was treated with deep x-ray therapy, but failed to respond. He died 6 months later and at autopsy, diffuse Hodgkin's disease was found involving the retropcritoneum, abdominal viscera and lungs. DISCUSSIOK
A comprehensive treatise on primary retroperitoneal tumors was recently written by Pack and Tabah.1 In this article, the authors estimated that approximately 750 cases of primary retroperitoneal tumors had been reported to that time (1954). They found an incidence of primary retroperitoneal tumors at the Memorial Cancer Center over a 26-year period from 1926 to 1951 was 0.2 per cent (120 cases). Of these 120 cases, 85.8 per cent were diagnosed as malignant while the remainder were benign. The most common malignant primary retroperitoneal tumor was 1 Pack, G. T. and Tabah, E. J.: Primary retroperitoneal tumors. A study of 120 cases. Coll. Review, Int. Abstr. Surg .. 99: 209-231, 313-341,
1954.
FIG. 4. Left ureteral obstruction and displacement seen in retrograde pyelogram of patient with disseminated Hodgkin's disease.
UNUSUAL CAUSES OF EXTRINSIC URETERAL OBSTRUCTION. II
the lymphoma group with lymphosarcoma and Hodgkin's disease comprising 24 cases. Next in frequency were rhabdomyosarcoma (22 cases) and liposarcoma (17 cases). The remainder of the primary malignant retroperitoneal tumors were the rarer varieties such as leiomyosarcoma, neuroblastoma, fibrosarcoma and mesothelioma. It is surprising that in this survey, while the authors mention that 39 per cent of the cases showed invasion of major blood vessels, including the aorta, vena cava or renal vessels, they fail to make any mention of ureteral involvement by retroperitoneal tumors. URETERAL OBSTRUCTION BY SECONDARY RETROPERITONEAL TUMORS
Metastatic or by direct spread. The first documented case of metastatic disease to the ureter was reported by Stow in 1909. 2 In 1948, Presman and Ehrlich summarized 35 cases of metastatic tumor to the ureter which they found in the literature and added 2 cases of their own. 3 Hydroureter and hydronephrosis were present in 70 per cent of the cases and in 50 per cent there was bilateral ureteral involvement. MacLean and Fowler found 39 cases of metastatic tumor involving the ureter in 10,223 successive autopsies at the Royal Victoria Hospital. 4 In 18 of these cases, there was an actual lesion in the ureter, and in 21 a lesion surrounded the ureter and occluded it. The primary sites of tumors in those cases where the metastases were to the ureteral wall proper included the prostate, rectum, pancreas, breast, thyroid and melanoma of the skin. Of the cases where the ureter was occluded by tumor surrounding it, the primary lesion was in the breast in two, ovary in two, cervix in 10, rectum and sigmoid in four, jejunum in one and penis in one. They state that extrinsic occlusion of the ureter by metastases from a primary carcinoma elsewhere occurred in 0.2 per cent of the autopsies. Ureteral obstruction by metastatic gastric carcinoma to the ureteral wall; case report. D. G., a 76-year-old white clerk, was admitted to the 2 Stow, B.: Fibrolymphosarcomata of both ureters, metastatic to a primary lymphosarcoma of the anterior mediastinum of thymus origin. Ann. Surg., 50: 901-906, 1909. 3 Presman, E. and Ehrlich, L.: Metastatic tumors of the ureter. J. Urol., 59: 312-325, 1948. 4 MacLean, J. T. and Fowler, V. B.: Tumors of renal pelvis and ureter. J. Urol., 75: 384-415, 1956.
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Wadsworth Veterans Hospital, Los Angeles, complaining of severe pain in the left flank of 18 hours' duration. Two years previously, he had been hospitalized because of epigastric pain, which occurred in relation to meals. An upper gastrointestinal series at that time was reported as normal. Shortly after the patient's admission an intravenous urogram with delayed films showed extravasation of opaque material about the left renal pelvis (fig. 5). Because of this unexpected finding of spontaneous rupture of the kidney, surgical exploration was carried out, at which time the left perinephric area was found to be edematous. The renal pelvis was dilated and there was a kink in the upper third of the ureter. A biopsy taken from the thickened periureteral tissue showed metastatic adenocarcinoma. The primary site was thought to be the gastrointestinal tract. During the postoperative period an upper gastrointestinal series demonstrated a carcinoma of the stomach. The patient died approximately 2}i months later; at autopsy, there was moderate bilateral hydronephrosis. On the left side the obstruction was due to tumor infiltration in the wall of the upper third of the ureter and on the right side there was a periureteral tumor near the bladder. An infiltrating tumor in the antrum on the stomach involved the anterior and posterior walls of the greater curvature and lymph nodes of the gastrocolic ligament. The periportal, periaortic and mesenteric lymph nodes were densely infiltrated with tumor. This case has been reported previously by the author as an example of ureteral and lymphatic obstruction producing a "closed compartment" rupture of the kidney. 5 Fergusson 6 described a case similar to this in which the patient had a carcinoma of the pyloric antrum with widespread metastases which obstructed the right ureter at the pelvic brim and also produced spontaneous rupture of the kidney. URETERAL COMPRESSION BY METASTATIC PANCREATIC CARCINOMA
A nuria caused by periureteral pancreatic carcinoma; case report. M. G., a 56-year-old 5 Kaufman, J. J. and Goodwin, W. E.: Renal lymphatics III. Clinical implications and experiments of nature. Ann. Int. Med., 49: 109-110, 1958. 6 Fergusson, J. D.: Ureter al stricture with perinephric urinary extravasation caused by metastases from a silent carcinoma of the stomach. Brit. J. Surg., 31: 283-286, 1943-1944.
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JOSEPH J. KAUFMAN
FIG. 5. Spontaneous rupture of left renal pelvis secondary to upper ureteral compression by metastatic gastric carcinoma. (Kaufman, J ..J. and Goodwin, W. E.: Ann. Int. Med., 49: 109-119, 1958.)
white male janitor, was admitted to Mount Sinai Hospital, Los )u1gdes, complaining of back pain, anorexia and weight loss of 4 months' duration. He had been admitted to the hospital 1 month earlier for similar complaints. At that time his blood and urinalysis were normal, and an upper gastrointestinal series revealed a duodenal ulcer. The patient improved somewhat on his ulcer regimen, but was readmitted because of severe back pain. On examination he appeared chronically ill. The lungs were clear; the liver was smooth and palpable 2 fingerbreadtbs below the costal margin. The pain became progressively worse and he continued to lose weight. An intravenous urogram showed slight left bydronephrosis with apparent partial obstruction (fig. 6). There was no obstruction on the right. However, 1 month later, the patient became totally anuric. An intravenous urogram showed no function and on the following day attempts to pass ureteral catheters were made without success. The right flank was explored and the ureter was found to be encased by a hard tumor whose limits could not be defined. A portion of the ureter and the attached mass were taken for biopsy, and a nephrostomy was performed. The peritoneal cavity was opened and the head of the pancreas was pal-
pated and found to be stony hard. This induration spread retroperitoneally to involve the midureters on both sides. The patient's immediate postoperative rnurse was satisfactory, the creatininc dropping from 8.8 mg. per cent preoperativcly to 1.4 mg. per cent 1 week later. However, he died 4 months later. The pathology report was pcriureteral and infiltrative pleomorphic adenocarcinoma of pancreatic origin. URETERAL COMPRESSION BY MBTASTATIC RECTAL CARCINOMA
Case report 1. YV. L., (CLH No. 59-06102), a 41-year-old man, was admitted to Cedars of Lebanon Hospital, Los Angeles, complaining of right flank pain, and a presumptive diagnosis of ureteral calculus was made. An intravenous urogram showed grade 1 caliectasia with ureterectasia on the right down to the level of the sacrum. The distal portion of the ureter was not visualized. The patient had chills and fever several days later, and at this time there was marked costovertebral angle tenderness on the right. Cystoscopy was done and catheters were passed with ease into the right renal pelvis. No evidence of ureteral calculus was seen on x-ray, but there was moderate pyelectasia and ureterectasia do,vn to the level of the third sacral
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Fm. 6. Intravenous urogram and retrograde pyelograms of patient with metastatic pancreatic carcinoma obstructing left ureter and which subsequently obstructed both ureters completely. segment. Below this point, the ureter narrowed sharply and showed irregularity of its margins which suggested extrinsic constriction (fig. 7). The impression from these roentgenograms was periureteritis plastica (retroperitoneal fibrosis). An indwelling, size 7 ureteral catheter was left in place, and the patient was given corticosteroid therapy for 5 days. After removal of the catheter however, the right flank pain recurred, and the lower right ureter was then explored through a lateral rectus inc1s10n and extraperitoneal approach. The ureter was found to be surrounded by extensive, hard, periureteral tissue which also fanned out retrovesically into the iliac areas. Multiple biopsies were done. The ureter was finally freed from its fibrous sheath and, since frozen section did not answer the question as to whether this was tumor or retroperitoneal fibrosis, a ureteroneocystostomy was done. The permanent sections showed carcinoma typical of rectal cancer involving the periureteral tissue. A barium enema, performed postoperatively, disclosed a constricting lesion which extended from 4 inches to approximately 8 inches above the anal margin. Sigmoidoscopy showed an annular carcinoma of the rectosigmoid. The patient subsequently had x-ray
therapy, but had a gradual downhill course and died in December 1959, of widespread metastases. URETERAL OBSTRUCTION BY METASTATIC TESTIS TUMOR
Case report.* J. R. (GH No. 11312), a 35-yearold white man, had right orchiectomy in 1949 for seminoma. Postoperatively, he was treated with radiation therapy to the retroperitoneal area and to the mediastinum. He did well for 4½ years, but then began to have pain in the right flank and right lower quadrant. On admission there was evidence of chest metastases, but excretory urography revealed mild hydronephrosis with medial deviation of the midureter suggestive of extrinsic pressure. An abdominal venogram was done, and no ascent of dye was seen in the vena cava. The accompanying film shows the combined excretory urogram and venogram revealing ureteral and ca val obstruction (fig. 8). Surgical exploration corroborated the radiographic findings of inoperable
*Case previously reported by the author. 7 7 Kaufman, J. J., Burke, D. E. and Goodwin, W. E.: Abdominal venography in urological diagnosis. J. Urol., 75: 160-168, 1956.
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Fm. 7. Intravenous urogram and retrograde pyelogram show stricture of distal third of right ureter produced by retroperitoneal extension of rectal carcinoma. retroperitoneal lymph node metastases with ureteral obstruction by external compression. The patient was given additional radiation therapy. URETERAL COMPRESSION BY METASTATIC BRONCHOGENIC CARCINOMA
Case report. J. D. (GH No. 54802), a 66-yearold white policeman, entered the vVads worth General Hospital, Veterans Administration Center on April 8, 1955, in a semi-stuporous condition. A chest film showed pulmonary congestion and possible pneumonitis at the left base. He had a rapid downhill course and died April 14, 6 days after admission. At autopsy he was found to have a bronchogenic carcinoma originating in the left lower lobe with metastases to the liver, to the retroperitoneal area, to the prostate and adrenal. _Moderate right hydronephrosis and ureterectasia were caused by a retroperitoneal metastatic tumor enveloping the mid-ureter (fig. 9). URETERAL OBSTRUCTION BY EXTRINSIC ENDOMETRIOSIS
Case report. (Courtesy of Drs. Joseph Kaplan and Harold Kudish.) S. S., a 33-year-old single
Fm. 8. Combined intravenou8 urogram and venacavagram show obstruction of right midureter and vena cava by metastatic seminoma of right testis. (Kaufman, J. J., Burke, D. E. and Goodwin, W. E.: J. Urol., 75: 160-168, 1956.)
UNUSUAL CAUSES OF EXTRINSIC URETERAL OBSTRUCTION. II
,
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·'t:' \,.,
Fw. 9. Low and high power photomicrographs show periureteral metastatic bronchogenic carcinoma
woman, had left flank pain, chills and fever and was admitted to Mount Sinai Hospital (No. 60-2922) with a diagnosis of acute pyelonephritis. She was treated with antibiotics without response. Because of a past history of cyclic flank pain associated with menses, a presumptive diagnosis of endometriosis was made. However, in the past none of these episodes had been associated with chills or fever. On examination, she was found to have definite left flank tenderness. Her temperature was 102F. An intravenous urogram showed a slight delay of dye appearance on the left side, and on subsequent films there was hydronephrosis and hydroureter with tortuosity of the ureter and dilatation down to a point approximately 7 cm. above the ureterovesical juncture (fig. 10, ,{). A retrograde pyelogram was attempted, but the catheter could not be passed beyond 4 cm., and the injection of a Foley cone catheter disclosf'd an area of narrowing of the lower ureter and filling of only 6 cm. of the ureter above this constricted area. On the following day, an operation was performed. A dense fibrous envelope was found around the distal 7 cm. of the ureter. Ureterotomy was done in the dilated pelvic portion of the ureter, and the ureter was then dissected out of the sheath. A size 8 Foley ureteral splint was placed in the
ureter, and the area was drained. At the same time, a hysterectomy and bilateral oophorectomy were done for obvious endometriosis. She had a chocolate cyst of the left ovary; the ovary had dropped down into the pouch of Douglas. Postoperatively, the patient made an uneventful recovery, and subsequent pyelograms showed a return to normal of the dilated ureter (fig. 10, B). DISCUSSION
Over half of the cases of ureteral obstruction by metastatic tumor reported in the literature have been characterized by periureteral involvement of the ureteral wall. 3 The ureter is not uncommonly involved by direct extension from a primary malignant tumor. This is frequently seen in carcinoma of the cervix which characteristically spreads along the broad ligament to entrap the lower ureter. It is also a frequent finding in carcinoma of the prostate. Beach states that 60 to 80 per cent of women afflicted with cancer of the cervix who succumb to this disease die of uremia, secondary to occlusion of the pelvic ureters. 8 The treatment of secondary tumors of the 8 Beach, E. W.: Urologic complications of cancer of the uterine cervix. J. Urol., 68: 178-189,
1952.
33G
JOSEPH J. KAUFMAN
Frn. 10. Pre- and postoperative intravenous urograms of patient with right ureteral obstruction by extrinsic endometriosis.
ureter is entirely palliative. Some urologists alleviate ureteral obstruction by nephrostomy, cutaneous ureterostomy, or other supravesical urinary diversion. Others adopt the philosophy that in widespread cancer, uremia is a blessing Endometriosis is not to be included in this category, since treatment by surgical radiation or hormonal means is curative. The first case of ureteral obstruction due to endometriosis was described by Randall in 1941. 9 Ratliff and Crenshaw10 added 3 cases of ureteral obstruction to five which they found in the literature. They state that the diagnosis of this condition is sometimes difficult, but there are several aids: Usually the ureter shows obstruction in the lower third; there is almost concurrent pelvic endometriosis; and the pain is frequently of cyclic nature. The cause of endometriosis involving the ureter is unknown, but in six of their 8 patients, pelvic surgery had been performed previously lending weight to the theory of traumatic displacement or "spill" of endometrial tissue. This was supported by the finding
of peritoneal endometriosis overlying the stricture site of the ureter in 5 cases. Occasionally, endometrial tissue involves the lumen of the ureter as in the case reported by Chin, Horton and Rusche. 11 The treatment of ureteral endometriosis consists of local excision of the endometrial implant in young patients with a small lesion in the hope of retaining fertility, and by castration (surgical, x-rays or hormonal) for those patients in whom the kidney can be saved. Brock states that the treatment of intrinsic ureteral endometriosis is surgical with local excision if feasible and that extrinsic endometriosis with ureteral obstruction is treated by castration.12
s Randall, A.: Endometrioma of the ureter. J. Urol., 46: 419-422, 1941. 10 Ratliff, R. K. and Crenshaw, W. B.: Ureteral obstruction from endometriosis. Surg., Gynec. & Obst., 100: 414-418, 1955.
11 Chinn, J., Horton, R. and Rusche, C.: Unilateral ureteral obstruction as sole manifestations of endometriosis. J. Urol., 77: 144-150, 1959. 12 Brock, D.R.: Ureteral obstruction from endometriosis. J. Urol., 83: 100-102, 1960.
SUMMARY
A variety of cases of extrinsic ureteral obstruction from primary or secondary retroperitoneal tumors has been presented. The ureters can be compressed or directly involved by metastatic tumors originating in widely separated organs. Several points are worthy of emphasis: Many times extrinsic ureteral compression can produce
UNUSUAL CAUSES OF EXTRINSIC URETERAL OBSTRUCTION. II
anuria. However, not infrequently, ureteral catheterization can be done with no resistance being met and with at least temporary relief of the obstruction. In other cases, the ureteral catheter cannot be advanced beyond an impassable obstruction. In selected cases, antegrade pyelography may give a clue to the site and nature of the ureteral obstruction.13 The history 13 Casey, W. C. and Goodwin, W. E.: Percutaneous antegrade pyelography and hydronephrosis. J. Urol., 74: 164-173, 1955.
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is extremely important. A background of carcinoma of the prostate, cervix, stomach, pancreas, uterus and ovary, rectum, pancreas and testes should alert the urologist and radiologist. Even such distant sites as the lung should be kept in mind. It will often be found that diagnosis will depend upon surgical exploration and adequate biopsy. The treatment depends upon the nature of the problem. Some methods of treatment have been suggested in the cases presented here.
Vol. 87, No. 3 March 1962 Copyright © 1962 by The Williams & Wilkins Co.
Ptinted in U.S.A.
UNUSUAL CAUSES OF EXTRINSIC URETERAL OBSTRUCTION, PAllT II JOSEPH J. KAUFMA~ From the Department of Surgery, Division of Urology, University of California il1eclical Center, Los Angeles, and the Urological Service, Wadsworth General Hospital, Veterans Administration Center, Los Angeles 24, Cal.
In part I (p. 319 of this issue) uncommon non-neoplastic conditions which can produce obstruction of the ureters ,vere discussed. This paper will deal with examples of primary and secondary retroperitoneal tumors involving or compressing the ureter ancl causing hydronephrosis. PRIMARY RETROPERITONEAL TUMORS INVOLVING THE URETER
Retroperitoneal reticulum cell sarcoma producing amiria; case report. G. G. (GSH Ko. 60-7621), a patient of Dr. Elmer Belt, was a 62-year-old white man who was admitted to the hospital with anuria of 4 days' duration. Cholecystectomy 6 weeks previously was followed by an uneventful course except for oliguria, beginning approximately 2 weeks following the operation. At that time his nonprotein nitrogen was 178. On a large fluid intake this dropped to 94, and he was sent home. However, 4 days prior to admission to the hospital he became totally anuric, which was followed by peripheral edema, orthopnea, dysnea, and mental confusion. He appeared cachectic. Pitting edema was present over both ankles and sacrum. A hard, nontender mass filled the entire right upper quadrant and did not appear to be related to the wound. Ureteral catheters were passed and immediate diuresis occurred with outputs averaging 3,000 cc daily. Retrograde pyelograms done subsequently showed bilateral hydronephrosis and hydroureter down to the pelvic brim (fig. 1). Despite his clinical improvement from urinary drainage, he died and at autopsy, an extensive retroperitoneal reticulum cell sarcoma was found with fixation to the aorta, to the inferior vena cava, and to the ureters. There was no direct involvement of the ureteral muscular wall by tumor, but there was acute interstitial ureteritis and localized stenosis of the ureters,
Accepted for publication July 21, 1961. Read at annual meeting of Western Section of American Urological Association, Inc., Las Vegas, Nevada, February 6-9, 1961.
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with angulation of the upper portion of the left ureter. The ureters were easily dissected free from the retroperitoneal sarcoma. Microscopic study of the retroperitoneal tumor showed a densely cellular, malignant neoplasm in which the dominant cell type was the reticulum cell. The ureters at multiple levels examined were partly found to be completely surrounded by tumor with only occasional microscopic foci of involvement of the ureteral musculature proper. LYMPHOSARCOMA PRODUCING URETERAL OBSTRUCTION
Case report. E. K. (GH No. 2270), a 65-yearold white man, was admitted to the Veterans Administration Hospital, Los Angeles, complaining of right flank pain of 1 week's duration. For several weeks there had been nausea, fever, and chills. There had been a 15 to 20 pound weight loss in the preceding 3 months despite a good appetite. Examination showed a chronically ill man, who had a questionable palpable right flank mass and definite tenderness of the right costovertebral angle. The hemoglobin was 13.1 and the creatinine ,ms 2.2. Intravenous urography revealed a moderately dilated right urinary collecting system down to the bony pelvis where there was an abrupt cut-off (fig. 2). Cystoscopy and ureteral catheterization were attempted but the catheter could not be advanced beyond 6 cm. on the right. Retrograde injection of contrast medium failed to fill the ureter beyond this point. The presumptive diagnosis was an extrinsic lesion compressing the right ureter. The lower ureter was explored through a Gibson incision, and a fusiform firm swelling of the ureter vms found 6 cm. above the bladder. The swelling measured approximately 6 cm. in length and 3 to 4 cm. in diameter. With only slight difficulty, the ureter with the bulbous swelling was dissected from the surrounding structures. The ureter distal to the swelling was normal, whereas above the swelling it was dilated and tense. Nephro-ureterectomy was done
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Fm. 1. Retrograde pyelograms show bilateral hydronephrosis caused by ureteral compression and invasion secondary to primary retroperitoneal reticulum cell sarcoma. (fig. 3, A). Section of the ureter at the area of the bulbous swelling showed it to be encased by a discrete periureteral tumor (lymphosarcoma). The wall of the ureter was not directly invaded by the lymphosarcoma, but the tumor was closely applied to the outside of the ureter (fig. 3, B). This patient had a satisfactory postoperative course and was subsequently treated with deep radiation therapy to the right lower quadrant through 4 portals. He was doing well 5 months postoperatively and 4 months after radiation therapy was completed. HODGKIN'S DISEASE WITH LEFT URETERAL OBSTRUCTION
This patient is presented through the courtesy of Dr. Robert 0. Pearman. Case report. A 36-year-old white man entered the hospital complaining of a left flank tumor of 3 months' duration. A diagnosis of diffuse Hodgkin's disease was made 4 years earlier from nodes excised from the left axilla. He had been previously treated with x-ray therapy and was then well until 3 months prior to admission, at which time he noted a dull aching pain in the left flank with subsequent appearance of a bulging mass in the left flank. On physical examination, the abdomen was moderately tender and a mass the size of a grapefruit in the left flank was found to extend to the midline. Urinalysis was negative, but an intravenous
Fm. 2. Intravenous urogram shows ureteral obstruction causing right hydronephrosis. urogram disclosed no function of the left kidney. A retrograde pyelogram was made showing hydronephrosis and hydroureter on the left extending down to a point several centimeters above the bladder (fig. 4). There was marked lateral displacement of the left kidney and upper
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FIG. 3. A, specimen (resected kidney and ureter) shows bulbous swelling of lower ureter. B, crosssection of ureter at level of swelling shows lymphosarcoma.
ureter, with medial displacement of the lower ureter. The patient was treated with deep x-ray therapy, but failed to respond. He died 6 months later and at autopsy, diffuse Hodgkin's disease was found involving the retropcritoneum, abdominal viscera and lungs. DISCUSSIOK
A comprehensive treatise on primary retroperitoneal tumors was recently written by Pack and Tabah.1 In this article, the authors estimated that approximately 750 cases of primary retroperitoneal tumors had been reported to that time (1954). They found an incidence of primary retroperitoneal tumors at the Memorial Cancer Center over a 26-year period from 1926 to 1951 was 0.2 per cent (120 cases). Of these 120 cases, 85.8 per cent were diagnosed as malignant while the remainder were benign. The most common malignant primary retroperitoneal tumor was 1 Pack, G. T. and Tabah, E. J.: Primary retroperitoneal tumors. A study of 120 cases. Coll. Review, Int. Abstr. Surg .. 99: 209-231, 313-341,
1954.
FIG. 4. Left ureteral obstruction and displacement seen in retrograde pyelogram of patient with disseminated Hodgkin's disease.
UNUSUAL CAUSES OF EXTRINSIC URETERAL OBSTRUCTION. II
the lymphoma group with lymphosarcoma and Hodgkin's disease comprising 24 cases. Next in frequency were rhabdomyosarcoma (22 cases) and liposarcoma (17 cases). The remainder of the primary malignant retroperitoneal tumors were the rarer varieties such as leiomyosarcoma, neuroblastoma, fibrosarcoma and mesothelioma. It is surprising that in this survey, while the authors mention that 39 per cent of the cases showed invasion of major blood vessels, including the aorta, vena cava or renal vessels, they fail to make any mention of ureteral involvement by retroperitoneal tumors. URETERAL OBSTRUCTION BY SECONDARY RETROPERITONEAL TUMORS
Metastatic or by direct spread. The first documented case of metastatic disease to the ureter was reported by Stow in 1909. 2 In 1948, Presman and Ehrlich summarized 35 cases of metastatic tumor to the ureter which they found in the literature and added 2 cases of their own. 3 Hydroureter and hydronephrosis were present in 70 per cent of the cases and in 50 per cent there was bilateral ureteral involvement. MacLean and Fowler found 39 cases of metastatic tumor involving the ureter in 10,223 successive autopsies at the Royal Victoria Hospital. 4 In 18 of these cases, there was an actual lesion in the ureter, and in 21 a lesion surrounded the ureter and occluded it. The primary sites of tumors in those cases where the metastases were to the ureteral wall proper included the prostate, rectum, pancreas, breast, thyroid and melanoma of the skin. Of the cases where the ureter was occluded by tumor surrounding it, the primary lesion was in the breast in two, ovary in two, cervix in 10, rectum and sigmoid in four, jejunum in one and penis in one. They state that extrinsic occlusion of the ureter by metastases from a primary carcinoma elsewhere occurred in 0.2 per cent of the autopsies. Ureteral obstruction by metastatic gastric carcinoma to the ureteral wall; case report. D. G., a 76-year-old white clerk, was admitted to the 2 Stow, B.: Fibrolymphosarcomata of both ureters, metastatic to a primary lymphosarcoma of the anterior mediastinum of thymus origin. Ann. Surg., 50: 901-906, 1909. 3 Presman, E. and Ehrlich, L.: Metastatic tumors of the ureter. J. Urol., 59: 312-325, 1948. 4 MacLean, J. T. and Fowler, V. B.: Tumors of renal pelvis and ureter. J. Urol., 75: 384-415, 1956.
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Wadsworth Veterans Hospital, Los Angeles, complaining of severe pain in the left flank of 18 hours' duration. Two years previously, he had been hospitalized because of epigastric pain, which occurred in relation to meals. An upper gastrointestinal series at that time was reported as normal. Shortly after the patient's admission an intravenous urogram with delayed films showed extravasation of opaque material about the left renal pelvis (fig. 5). Because of this unexpected finding of spontaneous rupture of the kidney, surgical exploration was carried out, at which time the left perinephric area was found to be edematous. The renal pelvis was dilated and there was a kink in the upper third of the ureter. A biopsy taken from the thickened periureteral tissue showed metastatic adenocarcinoma. The primary site was thought to be the gastrointestinal tract. During the postoperative period an upper gastrointestinal series demonstrated a carcinoma of the stomach. The patient died approximately 2}i months later; at autopsy, there was moderate bilateral hydronephrosis. On the left side the obstruction was due to tumor infiltration in the wall of the upper third of the ureter and on the right side there was a periureteral tumor near the bladder. An infiltrating tumor in the antrum on the stomach involved the anterior and posterior walls of the greater curvature and lymph nodes of the gastrocolic ligament. The periportal, periaortic and mesenteric lymph nodes were densely infiltrated with tumor. This case has been reported previously by the author as an example of ureteral and lymphatic obstruction producing a "closed compartment" rupture of the kidney. 5 Fergusson 6 described a case similar to this in which the patient had a carcinoma of the pyloric antrum with widespread metastases which obstructed the right ureter at the pelvic brim and also produced spontaneous rupture of the kidney. URETERAL COMPRESSION BY METASTATIC PANCREATIC CARCINOMA
A nuria caused by periureteral pancreatic carcinoma; case report. M. G., a 56-year-old 5 Kaufman, J. J. and Goodwin, W. E.: Renal lymphatics III. Clinical implications and experiments of nature. Ann. Int. Med., 49: 109-110, 1958. 6 Fergusson, J. D.: Ureter al stricture with perinephric urinary extravasation caused by metastases from a silent carcinoma of the stomach. Brit. J. Surg., 31: 283-286, 1943-1944.
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FIG. 5. Spontaneous rupture of left renal pelvis secondary to upper ureteral compression by metastatic gastric carcinoma. (Kaufman, J ..J. and Goodwin, W. E.: Ann. Int. Med., 49: 109-119, 1958.)
white male janitor, was admitted to Mount Sinai Hospital, Los )u1gdes, complaining of back pain, anorexia and weight loss of 4 months' duration. He had been admitted to the hospital 1 month earlier for similar complaints. At that time his blood and urinalysis were normal, and an upper gastrointestinal series revealed a duodenal ulcer. The patient improved somewhat on his ulcer regimen, but was readmitted because of severe back pain. On examination he appeared chronically ill. The lungs were clear; the liver was smooth and palpable 2 fingerbreadtbs below the costal margin. The pain became progressively worse and he continued to lose weight. An intravenous urogram showed slight left bydronephrosis with apparent partial obstruction (fig. 6). There was no obstruction on the right. However, 1 month later, the patient became totally anuric. An intravenous urogram showed no function and on the following day attempts to pass ureteral catheters were made without success. The right flank was explored and the ureter was found to be encased by a hard tumor whose limits could not be defined. A portion of the ureter and the attached mass were taken for biopsy, and a nephrostomy was performed. The peritoneal cavity was opened and the head of the pancreas was pal-
pated and found to be stony hard. This induration spread retroperitoneally to involve the midureters on both sides. The patient's immediate postoperative rnurse was satisfactory, the creatininc dropping from 8.8 mg. per cent preoperativcly to 1.4 mg. per cent 1 week later. However, he died 4 months later. The pathology report was pcriureteral and infiltrative pleomorphic adenocarcinoma of pancreatic origin. URETERAL COMPRESSION BY MBTASTATIC RECTAL CARCINOMA
Case report 1. YV. L., (CLH No. 59-06102), a 41-year-old man, was admitted to Cedars of Lebanon Hospital, Los Angeles, complaining of right flank pain, and a presumptive diagnosis of ureteral calculus was made. An intravenous urogram showed grade 1 caliectasia with ureterectasia on the right down to the level of the sacrum. The distal portion of the ureter was not visualized. The patient had chills and fever several days later, and at this time there was marked costovertebral angle tenderness on the right. Cystoscopy was done and catheters were passed with ease into the right renal pelvis. No evidence of ureteral calculus was seen on x-ray, but there was moderate pyelectasia and ureterectasia do,vn to the level of the third sacral
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Fm. 6. Intravenous urogram and retrograde pyelograms of patient with metastatic pancreatic carcinoma obstructing left ureter and which subsequently obstructed both ureters completely. segment. Below this point, the ureter narrowed sharply and showed irregularity of its margins which suggested extrinsic constriction (fig. 7). The impression from these roentgenograms was periureteritis plastica (retroperitoneal fibrosis). An indwelling, size 7 ureteral catheter was left in place, and the patient was given corticosteroid therapy for 5 days. After removal of the catheter however, the right flank pain recurred, and the lower right ureter was then explored through a lateral rectus inc1s10n and extraperitoneal approach. The ureter was found to be surrounded by extensive, hard, periureteral tissue which also fanned out retrovesically into the iliac areas. Multiple biopsies were done. The ureter was finally freed from its fibrous sheath and, since frozen section did not answer the question as to whether this was tumor or retroperitoneal fibrosis, a ureteroneocystostomy was done. The permanent sections showed carcinoma typical of rectal cancer involving the periureteral tissue. A barium enema, performed postoperatively, disclosed a constricting lesion which extended from 4 inches to approximately 8 inches above the anal margin. Sigmoidoscopy showed an annular carcinoma of the rectosigmoid. The patient subsequently had x-ray
therapy, but had a gradual downhill course and died in December 1959, of widespread metastases. URETERAL OBSTRUCTION BY METASTATIC TESTIS TUMOR
Case report.* J. R. (GH No. 11312), a 35-yearold white man, had right orchiectomy in 1949 for seminoma. Postoperatively, he was treated with radiation therapy to the retroperitoneal area and to the mediastinum. He did well for 4½ years, but then began to have pain in the right flank and right lower quadrant. On admission there was evidence of chest metastases, but excretory urography revealed mild hydronephrosis with medial deviation of the midureter suggestive of extrinsic pressure. An abdominal venogram was done, and no ascent of dye was seen in the vena cava. The accompanying film shows the combined excretory urogram and venogram revealing ureteral and ca val obstruction (fig. 8). Surgical exploration corroborated the radiographic findings of inoperable
*Case previously reported by the author. 7 7 Kaufman, J. J., Burke, D. E. and Goodwin, W. E.: Abdominal venography in urological diagnosis. J. Urol., 75: 160-168, 1956.
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Fm. 7. Intravenous urogram and retrograde pyelogram show stricture of distal third of right ureter produced by retroperitoneal extension of rectal carcinoma. retroperitoneal lymph node metastases with ureteral obstruction by external compression. The patient was given additional radiation therapy. URETERAL COMPRESSION BY METASTATIC BRONCHOGENIC CARCINOMA
Case report. J. D. (GH No. 54802), a 66-yearold white policeman, entered the vVads worth General Hospital, Veterans Administration Center on April 8, 1955, in a semi-stuporous condition. A chest film showed pulmonary congestion and possible pneumonitis at the left base. He had a rapid downhill course and died April 14, 6 days after admission. At autopsy he was found to have a bronchogenic carcinoma originating in the left lower lobe with metastases to the liver, to the retroperitoneal area, to the prostate and adrenal. _Moderate right hydronephrosis and ureterectasia were caused by a retroperitoneal metastatic tumor enveloping the mid-ureter (fig. 9). URETERAL OBSTRUCTION BY EXTRINSIC ENDOMETRIOSIS
Case report. (Courtesy of Drs. Joseph Kaplan and Harold Kudish.) S. S., a 33-year-old single
Fm. 8. Combined intravenou8 urogram and venacavagram show obstruction of right midureter and vena cava by metastatic seminoma of right testis. (Kaufman, J. J., Burke, D. E. and Goodwin, W. E.: J. Urol., 75: 160-168, 1956.)
UNUSUAL CAUSES OF EXTRINSIC URETERAL OBSTRUCTION. II
,
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·'t:' \,.,
Fw. 9. Low and high power photomicrographs show periureteral metastatic bronchogenic carcinoma
woman, had left flank pain, chills and fever and was admitted to Mount Sinai Hospital (No. 60-2922) with a diagnosis of acute pyelonephritis. She was treated with antibiotics without response. Because of a past history of cyclic flank pain associated with menses, a presumptive diagnosis of endometriosis was made. However, in the past none of these episodes had been associated with chills or fever. On examination, she was found to have definite left flank tenderness. Her temperature was 102F. An intravenous urogram showed a slight delay of dye appearance on the left side, and on subsequent films there was hydronephrosis and hydroureter with tortuosity of the ureter and dilatation down to a point approximately 7 cm. above the ureterovesical juncture (fig. 10, ,{). A retrograde pyelogram was attempted, but the catheter could not be passed beyond 4 cm., and the injection of a Foley cone catheter disclosf'd an area of narrowing of the lower ureter and filling of only 6 cm. of the ureter above this constricted area. On the following day, an operation was performed. A dense fibrous envelope was found around the distal 7 cm. of the ureter. Ureterotomy was done in the dilated pelvic portion of the ureter, and the ureter was then dissected out of the sheath. A size 8 Foley ureteral splint was placed in the
ureter, and the area was drained. At the same time, a hysterectomy and bilateral oophorectomy were done for obvious endometriosis. She had a chocolate cyst of the left ovary; the ovary had dropped down into the pouch of Douglas. Postoperatively, the patient made an uneventful recovery, and subsequent pyelograms showed a return to normal of the dilated ureter (fig. 10, B). DISCUSSION
Over half of the cases of ureteral obstruction by metastatic tumor reported in the literature have been characterized by periureteral involvement of the ureteral wall. 3 The ureter is not uncommonly involved by direct extension from a primary malignant tumor. This is frequently seen in carcinoma of the cervix which characteristically spreads along the broad ligament to entrap the lower ureter. It is also a frequent finding in carcinoma of the prostate. Beach states that 60 to 80 per cent of women afflicted with cancer of the cervix who succumb to this disease die of uremia, secondary to occlusion of the pelvic ureters. 8 The treatment of secondary tumors of the 8 Beach, E. W.: Urologic complications of cancer of the uterine cervix. J. Urol., 68: 178-189,
1952.
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JOSEPH J. KAUFMAN
Frn. 10. Pre- and postoperative intravenous urograms of patient with right ureteral obstruction by extrinsic endometriosis.
ureter is entirely palliative. Some urologists alleviate ureteral obstruction by nephrostomy, cutaneous ureterostomy, or other supravesical urinary diversion. Others adopt the philosophy that in widespread cancer, uremia is a blessing Endometriosis is not to be included in this category, since treatment by surgical radiation or hormonal means is curative. The first case of ureteral obstruction due to endometriosis was described by Randall in 1941. 9 Ratliff and Crenshaw10 added 3 cases of ureteral obstruction to five which they found in the literature. They state that the diagnosis of this condition is sometimes difficult, but there are several aids: Usually the ureter shows obstruction in the lower third; there is almost concurrent pelvic endometriosis; and the pain is frequently of cyclic nature. The cause of endometriosis involving the ureter is unknown, but in six of their 8 patients, pelvic surgery had been performed previously lending weight to the theory of traumatic displacement or "spill" of endometrial tissue. This was supported by the finding
of peritoneal endometriosis overlying the stricture site of the ureter in 5 cases. Occasionally, endometrial tissue involves the lumen of the ureter as in the case reported by Chin, Horton and Rusche. 11 The treatment of ureteral endometriosis consists of local excision of the endometrial implant in young patients with a small lesion in the hope of retaining fertility, and by castration (surgical, x-rays or hormonal) for those patients in whom the kidney can be saved. Brock states that the treatment of intrinsic ureteral endometriosis is surgical with local excision if feasible and that extrinsic endometriosis with ureteral obstruction is treated by castration.12
s Randall, A.: Endometrioma of the ureter. J. Urol., 46: 419-422, 1941. 10 Ratliff, R. K. and Crenshaw, W. B.: Ureteral obstruction from endometriosis. Surg., Gynec. & Obst., 100: 414-418, 1955.
11 Chinn, J., Horton, R. and Rusche, C.: Unilateral ureteral obstruction as sole manifestations of endometriosis. J. Urol., 77: 144-150, 1959. 12 Brock, D.R.: Ureteral obstruction from endometriosis. J. Urol., 83: 100-102, 1960.
SUMMARY
A variety of cases of extrinsic ureteral obstruction from primary or secondary retroperitoneal tumors has been presented. The ureters can be compressed or directly involved by metastatic tumors originating in widely separated organs. Several points are worthy of emphasis: Many times extrinsic ureteral compression can produce
UNUSUAL CAUSES OF EXTRINSIC URETERAL OBSTRUCTION. II
anuria. However, not infrequently, ureteral catheterization can be done with no resistance being met and with at least temporary relief of the obstruction. In other cases, the ureteral catheter cannot be advanced beyond an impassable obstruction. In selected cases, antegrade pyelography may give a clue to the site and nature of the ureteral obstruction.13 The history 13 Casey, W. C. and Goodwin, W. E.: Percutaneous antegrade pyelography and hydronephrosis. J. Urol., 74: 164-173, 1955.
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is extremely important. A background of carcinoma of the prostate, cervix, stomach, pancreas, uterus and ovary, rectum, pancreas and testes should alert the urologist and radiologist. Even such distant sites as the lung should be kept in mind. It will often be found that diagnosis will depend upon surgical exploration and adequate biopsy. The treatment depends upon the nature of the problem. Some methods of treatment have been suggested in the cases presented here.