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Leiomyosarcoma of the Ureter: Case Report and Review of Literature
THE JOURNAL OF UROLOGY
Vol. 82, No. 1, July 1959
Printed in U.S.A.
LEIOMYOSARCOMA OF THE URETER: CASE REPORT AND REVIEW OF LITERATURE JAN R. WERi',ER, WILLIAM KLINGENSMITH
ANn
JOHN V. DENKO
From the Departments of Siirgery, Urology and Pathology, Northwest Texas Hospital, ,1marillo, Texas
Malignant tumors of smooth muscle can occur anywhere in the body where smooth muscle is found, but are most commonly encountered in the gastrointestinal tract and in the uterus. In the genitourinary tract they are found more commonly in the kidney than the ureter. Leiomyosarcoma of the ureter is an extremely rare condition reported 7 times in the world literature. This report is a case of leiomyosarcoma of the ureter substantiated by urograms and photomicrographs of the tumor. The first instance of a leiomyosarcoma arising in the genitourinary tract was reported by Ribbert in 1886. This was a leiomyosarcoma of the renal pelvis in a 4-year-old girl. In 1928, N ewmirth reported a leiomyosarcoma arising in the lower third of the ureter in a 51-year-old man. G. G. Smith reported a case of leiomyosarcoma of the genitourinary tract but was unable to determine whether it arose in the ureter or in the kidney. Rademaker, in 1943, reported a leiomyosarcoma of the ureter in a 59-year-old woman with a history of no recurrence for 14 months after surgery. In 1944, John E. Kraus reported a leiomyosarcoma of the ureter with metastasis to the pituitary gland. Rossien and Russel, in 1946, reported a case of leiomyosarcoma of the ureter in a 55-year-old woman who died 2 years after surgery from generalized metastases. In 1955, Alznauer reported a case of leiomyosarcoma of the ureter in a 60-year-old woman, in whom generalized metastases developed 9 months after surgery. Death was due to myocardial infarction and cerebral emboli.
fever that responded promptly to antibiotic therapy. She had had some slight nausea during the past 4 months, but no change in bowel habit or any other abdominal complaints. A vaginal hysterectomy for uterine prolapse had been performed two years previously; there was no familial history of malignancy. On physical examination the patient did not appear acutely ill. The neck was supple and without masses, and the heart, lungs and breasts showed no abnormalities. The blood pressure, temperature, and pulse were normal. The uterus and cervix were absent on bimanual examination and no abdominal masses were felt. Palpation of the abdomen elicited tenderness in the left lower quadrant with the presence of a sense of resistance in that area. The left flank was tender posteriorly. A blood count showed 3.8 million red blood cells, 11.3 gm. hemoglobin, 13,800 white blood cells, with 93 per cent neutrophils and 31 per
CASE REPORT
Mrs. F. R., a 60-year-old white woman, was admitted to Northwest Texas Hospital on January 5, 1958, complaining of left epigastric pain radiating into the left flank and groin of about 4 months in duration. There was no history of weight loss or urinary difficulty. But 10 days prior to admission she had a chill with Read at annual meeting of South Central Section, American Urological Association, Inc., Dallas, Texas, October 12-15, 1958.
FIG. 1
68
LEI0MYOSARC0ll1A OF' URE'l'ER
FIG. 2. A, leiomyosarcoma shows atypical smooth muscle bundles and whorling pattern. (H X 100.) B, mitosis and pleomorphism are evident in smooth muscle bundle infiltrating uret,er. space is lymphatic chanrrnl. (H & E, X 430.)
cent stabs. The urine contained 2 plus albumin and 5 to 15 white blood cells per high power field. The sedimentation rate (\Vestergren) was 26 mm. per hour. The nonprotein nitrogen determination was 39 mg. per cent. The chest x-ray showed no abnormalities. A barium enema showed sigmoid diverticulosis with no displacement of the bowel, and a barium meal revealed a hiatus hernia. A preliminary excretory urogram showed moderate hydronephrosis of the left renal pelvis and dilatation of all calycc"s. No definite point of obstruction could be visualized. The outline of the right kidney showed normal visualization within fivr) minutes after injection and with a normal anatomical pattern of the calyceal system. Cystoscopy demonstrated the lower urinary tract to he normal. On cathc,terization of the left ureter some difficulty was encountered approximately 14 cm. above thr, bladder. The catheter was withdrawn and a pycloureterogram was obtained (fig. l). The left mid ureter showed evidence of medial displacement in this area. The ureter appeared to be suddenly compressed by a soft tissue mass whieh could be plainly visualized. A lateral film showed sonw anterior displacement of the ureter in this area. The ureter above
this n1ass was dilated, and also 1.he entire: and all calyces. Five days after admission an exploratory operation was undertaken. F pon opening tbc peritoneal cavity a hard fix(;c[ mass just medial and below the lower pole of the lPft kidney wa8 noted. This mass was becneath the peritom·um but not attached to it. It was fixed to the muscleR posteriorly. It was also attached nwdiaLly to th(: aorta and to the superior mescnteric artery. The parietal peritoneum was opened and the inferim and superior margins of this mass and the ureter were identified. The ureter was found to br: con1· plctcly surrounded by the firm mass. A combination of sharp and blunt dissection freed the tumor from the aorta and from the-'. superior nwsc·nteric artery. There was a definite line of cle.a.vage between the tumor and the large vessels. The tumor was easily dissected from the psoas muscle posteriorly. An attempt was made to dissect the tumor from the ureter but the ureternl wall wm, in continuity with the tumor mass. Furthr·r exploration of the retropccritom:al space sl1mvPd the left ovarian vein to be infiltrated with tumor as it trnnsvc)rsed the mass. The kidney, the entire ureter and the tumor mass were then removed in one bloek.
70
Wl-
because of a suspected rctroperitoncal abscess. Purulent material with a fecal odor was obtained and a free segment of necrotic large bowel was removed. Two days later a right transverse colostomy was done to divert the focal stream, and the patient was greatly improved. She was discharged from the hospital 1 week later with a satisfactory functioning colostomy. We did not have occasion to sec this patient thereafter but learned that she expired 6 months postoperatively from multiple metastasis. COMMI£NT
FIG. 3. More aggressive portion of tumor with some giunt cells. (H & E, X430.)
The gross ·specimen consisted of a kidney with attached ureter and with tumor mass about the ureter. The fleshy gray-pink tumor mass measured 8 by 9 by 5 cm. and lay 6 cm. below the urcteropclvic junction. On cut surface the lumen of the ureter ,vas patent but narrowed by surrounding tumor tissue. :Microscopic sections of the tumor tissue were examined at many different levels. The cellular pattern consisted of elongated, plump, strapshapcd, spindle cells grouped in interlacing bundles with a slight degree of nuclear variation (fig. 2). The nuclear pattern was that of smooth muscle bundles with blunt end nuclei and rcticulin fibers. Other areas showed a more bizarre pattern with some pleomorphism, giant cells, fair numbers of which showed abnormal mitosis (fig. 3). These cells were found infiltrating and rn.erging with the ureter that had an intact transitional mucosa. Some portions of the turn.or had foci of necrosis. The leiomyosarcoma also infiltrated the ovarian vein. The kidney parenchyma showed focal infiltrations of lymphocytes and neutrophils in the stroma. The postoperative course was completely satisfactory until the seventh day when the patient became nauseated and left flank pain and fever developed. A daily spiking fever persisted and on the fourteenth day, the left flank was opened
A review of syrn.ptoms described in the previous case reports, as well as in our case, does not show any characteristic clinical picture. The symptoms can be traced to the obstruction of the ureter and the secondary changes in the proximal ureter and kidney. Hcmaturia is not an early sign. Secondary pyelonephritis had ensued in our patient. Preliminary roentgcnographic examination did not reveal pulmonary metastasis even though we found evidence of gross infiltration into the surrounding venous system during surgery. The postoperative necrosis of the bowel was probably due to some surgical trauma, thrombosis or invasion of blood vessels by tumor. In the differential diagnosis of this tumor we had to consider the possibility of the sarcoma originating in the retropcritoneal area and secondarily encroaching upon and invading the ureter. This particular tumor was associated with and surrounded the ureter. It also could be easily dissected from all other surrounding structures. The tumor grew around the ureter in a concentric fashion producing partial obstruction of the lumen. A review ot the treatment of reported cases as well as in our case consisted of radical excision and nephro-ureterectomy. No irradiation therapy was given to our patient. The prognosis is poor in all established instances. Our patient died within six months after diagnosis from generalized metastases. Unfortunately we were unable to obtain a postmortem examination. SUMMARY
A case report of a leiomyosarcoma of the ureter is presented in detail with urograms and
LEIOMYOSARCOMA OF URETER
photomicrographs. It is the eighth case added to the world literature. Photomicrographs were made by the Armed Forces Institute of Pathology, Washington, D. C. REFERENCES ALZNAUER, R. L.: Leiomyosarcoma of right ureter: Report of a case. Arch. Path., 59: 94-95, 1955. KRAUS, J. E.: Primary sarcoma of the ureter. Urol. & Cutan. Rev., 48: 522, 1944.
71
NEWMIRTH: (Quoted by Rademaker.) RADEMAKER, L.: Primary sarcoma of ureter: Case report and review of literature. Am. J. Surg. 61 : 402-406, 1943. ' RrnBERT: Ueber ein Myosarcoma atriocellular des Nierenbecken und des Ureters. Virch. Arch., 106: 282, 1886. RossIEN, A. K. AND RussELL, TH. H.: Leiomyosarcoma involving right ureter. Arch. Path., 41: 655-660, 1946. SMITH, G. G.: Neoplasms of kidney and ureter. Am. J. Surg., 30: 130, 1935.
Vol. 82, No. 1, July 1959
Printed in U.S.A.
LEIOMYOSARCOMA OF THE URETER: CASE REPORT AND REVIEW OF LITERATURE JAN R. WERi',ER, WILLIAM KLINGENSMITH
ANn
JOHN V. DENKO
From the Departments of Siirgery, Urology and Pathology, Northwest Texas Hospital, ,1marillo, Texas
Malignant tumors of smooth muscle can occur anywhere in the body where smooth muscle is found, but are most commonly encountered in the gastrointestinal tract and in the uterus. In the genitourinary tract they are found more commonly in the kidney than the ureter. Leiomyosarcoma of the ureter is an extremely rare condition reported 7 times in the world literature. This report is a case of leiomyosarcoma of the ureter substantiated by urograms and photomicrographs of the tumor. The first instance of a leiomyosarcoma arising in the genitourinary tract was reported by Ribbert in 1886. This was a leiomyosarcoma of the renal pelvis in a 4-year-old girl. In 1928, N ewmirth reported a leiomyosarcoma arising in the lower third of the ureter in a 51-year-old man. G. G. Smith reported a case of leiomyosarcoma of the genitourinary tract but was unable to determine whether it arose in the ureter or in the kidney. Rademaker, in 1943, reported a leiomyosarcoma of the ureter in a 59-year-old woman with a history of no recurrence for 14 months after surgery. In 1944, John E. Kraus reported a leiomyosarcoma of the ureter with metastasis to the pituitary gland. Rossien and Russel, in 1946, reported a case of leiomyosarcoma of the ureter in a 55-year-old woman who died 2 years after surgery from generalized metastases. In 1955, Alznauer reported a case of leiomyosarcoma of the ureter in a 60-year-old woman, in whom generalized metastases developed 9 months after surgery. Death was due to myocardial infarction and cerebral emboli.
fever that responded promptly to antibiotic therapy. She had had some slight nausea during the past 4 months, but no change in bowel habit or any other abdominal complaints. A vaginal hysterectomy for uterine prolapse had been performed two years previously; there was no familial history of malignancy. On physical examination the patient did not appear acutely ill. The neck was supple and without masses, and the heart, lungs and breasts showed no abnormalities. The blood pressure, temperature, and pulse were normal. The uterus and cervix were absent on bimanual examination and no abdominal masses were felt. Palpation of the abdomen elicited tenderness in the left lower quadrant with the presence of a sense of resistance in that area. The left flank was tender posteriorly. A blood count showed 3.8 million red blood cells, 11.3 gm. hemoglobin, 13,800 white blood cells, with 93 per cent neutrophils and 31 per
CASE REPORT
Mrs. F. R., a 60-year-old white woman, was admitted to Northwest Texas Hospital on January 5, 1958, complaining of left epigastric pain radiating into the left flank and groin of about 4 months in duration. There was no history of weight loss or urinary difficulty. But 10 days prior to admission she had a chill with Read at annual meeting of South Central Section, American Urological Association, Inc., Dallas, Texas, October 12-15, 1958.
FIG. 1
68
LEI0MYOSARC0ll1A OF' URE'l'ER
FIG. 2. A, leiomyosarcoma shows atypical smooth muscle bundles and whorling pattern. (H X 100.) B, mitosis and pleomorphism are evident in smooth muscle bundle infiltrating uret,er. space is lymphatic chanrrnl. (H & E, X 430.)
cent stabs. The urine contained 2 plus albumin and 5 to 15 white blood cells per high power field. The sedimentation rate (\Vestergren) was 26 mm. per hour. The nonprotein nitrogen determination was 39 mg. per cent. The chest x-ray showed no abnormalities. A barium enema showed sigmoid diverticulosis with no displacement of the bowel, and a barium meal revealed a hiatus hernia. A preliminary excretory urogram showed moderate hydronephrosis of the left renal pelvis and dilatation of all calycc"s. No definite point of obstruction could be visualized. The outline of the right kidney showed normal visualization within fivr) minutes after injection and with a normal anatomical pattern of the calyceal system. Cystoscopy demonstrated the lower urinary tract to he normal. On cathc,terization of the left ureter some difficulty was encountered approximately 14 cm. above thr, bladder. The catheter was withdrawn and a pycloureterogram was obtained (fig. l). The left mid ureter showed evidence of medial displacement in this area. The ureter appeared to be suddenly compressed by a soft tissue mass whieh could be plainly visualized. A lateral film showed sonw anterior displacement of the ureter in this area. The ureter above
this n1ass was dilated, and also 1.he entire: and all calyces. Five days after admission an exploratory operation was undertaken. F pon opening tbc peritoneal cavity a hard fix(;c[ mass just medial and below the lower pole of the lPft kidney wa8 noted. This mass was becneath the peritom·um but not attached to it. It was fixed to the muscleR posteriorly. It was also attached nwdiaLly to th(: aorta and to the superior mescnteric artery. The parietal peritoneum was opened and the inferim and superior margins of this mass and the ureter were identified. The ureter was found to br: con1· plctcly surrounded by the firm mass. A combination of sharp and blunt dissection freed the tumor from the aorta and from the-'. superior nwsc·nteric artery. There was a definite line of cle.a.vage between the tumor and the large vessels. The tumor was easily dissected from the psoas muscle posteriorly. An attempt was made to dissect the tumor from the ureter but the ureternl wall wm, in continuity with the tumor mass. Furthr·r exploration of the retropccritom:al space sl1mvPd the left ovarian vein to be infiltrated with tumor as it trnnsvc)rsed the mass. The kidney, the entire ureter and the tumor mass were then removed in one bloek.
70
Wl-
because of a suspected rctroperitoncal abscess. Purulent material with a fecal odor was obtained and a free segment of necrotic large bowel was removed. Two days later a right transverse colostomy was done to divert the focal stream, and the patient was greatly improved. She was discharged from the hospital 1 week later with a satisfactory functioning colostomy. We did not have occasion to sec this patient thereafter but learned that she expired 6 months postoperatively from multiple metastasis. COMMI£NT
FIG. 3. More aggressive portion of tumor with some giunt cells. (H & E, X430.)
The gross ·specimen consisted of a kidney with attached ureter and with tumor mass about the ureter. The fleshy gray-pink tumor mass measured 8 by 9 by 5 cm. and lay 6 cm. below the urcteropclvic junction. On cut surface the lumen of the ureter ,vas patent but narrowed by surrounding tumor tissue. :Microscopic sections of the tumor tissue were examined at many different levels. The cellular pattern consisted of elongated, plump, strapshapcd, spindle cells grouped in interlacing bundles with a slight degree of nuclear variation (fig. 2). The nuclear pattern was that of smooth muscle bundles with blunt end nuclei and rcticulin fibers. Other areas showed a more bizarre pattern with some pleomorphism, giant cells, fair numbers of which showed abnormal mitosis (fig. 3). These cells were found infiltrating and rn.erging with the ureter that had an intact transitional mucosa. Some portions of the turn.or had foci of necrosis. The leiomyosarcoma also infiltrated the ovarian vein. The kidney parenchyma showed focal infiltrations of lymphocytes and neutrophils in the stroma. The postoperative course was completely satisfactory until the seventh day when the patient became nauseated and left flank pain and fever developed. A daily spiking fever persisted and on the fourteenth day, the left flank was opened
A review of syrn.ptoms described in the previous case reports, as well as in our case, does not show any characteristic clinical picture. The symptoms can be traced to the obstruction of the ureter and the secondary changes in the proximal ureter and kidney. Hcmaturia is not an early sign. Secondary pyelonephritis had ensued in our patient. Preliminary roentgcnographic examination did not reveal pulmonary metastasis even though we found evidence of gross infiltration into the surrounding venous system during surgery. The postoperative necrosis of the bowel was probably due to some surgical trauma, thrombosis or invasion of blood vessels by tumor. In the differential diagnosis of this tumor we had to consider the possibility of the sarcoma originating in the retropcritoneal area and secondarily encroaching upon and invading the ureter. This particular tumor was associated with and surrounded the ureter. It also could be easily dissected from all other surrounding structures. The tumor grew around the ureter in a concentric fashion producing partial obstruction of the lumen. A review ot the treatment of reported cases as well as in our case consisted of radical excision and nephro-ureterectomy. No irradiation therapy was given to our patient. The prognosis is poor in all established instances. Our patient died within six months after diagnosis from generalized metastases. Unfortunately we were unable to obtain a postmortem examination. SUMMARY
A case report of a leiomyosarcoma of the ureter is presented in detail with urograms and
LEIOMYOSARCOMA OF URETER
photomicrographs. It is the eighth case added to the world literature. Photomicrographs were made by the Armed Forces Institute of Pathology, Washington, D. C. REFERENCES ALZNAUER, R. L.: Leiomyosarcoma of right ureter: Report of a case. Arch. Path., 59: 94-95, 1955. KRAUS, J. E.: Primary sarcoma of the ureter. Urol. & Cutan. Rev., 48: 522, 1944.
71
NEWMIRTH: (Quoted by Rademaker.) RADEMAKER, L.: Primary sarcoma of ureter: Case report and review of literature. Am. J. Surg. 61 : 402-406, 1943. ' RrnBERT: Ueber ein Myosarcoma atriocellular des Nierenbecken und des Ureters. Virch. Arch., 106: 282, 1886. RossIEN, A. K. AND RussELL, TH. H.: Leiomyosarcoma involving right ureter. Arch. Path., 41: 655-660, 1946. SMITH, G. G.: Neoplasms of kidney and ureter. Am. J. Surg., 30: 130, 1935.