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Fibroepithelial polyp of ureter
FIBROEPITHELIAL
F. M. J. DEBRUYNE, W. A. MOONEN,
POLYP OF URETER
M.D.
M.D.
A. A. DAENEKINDT, K. P. J. DELAERE,
M.D. M.D.
From the Departments of Urology, Radboud University Hospital Nijmegen, and Catharina City Hospital, Eindhoven, The Netherlands
__________~__
________
-__
ABSTRACT - Fibroepithelial polyp of the ureter, a mesodermal benign tumor, is extremely rare. Four cases of our own prompted us to review the world literature on this subject (108 documented cases since 1950). The mode of presentation, etiology, clinical features, and treatment modalities of this lesion are discussed.
The most recent review concerning fibroepithelial benign polyps of the ureter was done by Stuppler and Kandzari in 1975.’ Since primary ureteral neoplasms are uncommon and benign ureteral tumors are even rarer, it is important to draw attention to this lesion from time to time. Herein we review the cases published since 1950 and add 4 cases of so-called fibroma of the ureter to the literature. Tumors of the ureter can be divided into epithelial and mesodermal neoplasms. The epithelial transitional or squamous tumors are malignant or potentially malignant (even the so-called benign papilloma). Mesodermal tumors are marked by almost benign conditions, which present a great variety of morphologic patterns, such as hemangioma, myxoma, leiomyoma, etc. The benign polyp of the ureter is found most frequently in this group. According to Scott,* 50 per cent of mesodermal tumors are benign polyps of the ureter. At present, diagnosis of this lesion seems easy and the therapy applicable to it appears to be defined clearly. Nevertheless the following cases demonstrate that difficulties in exact preoperative diagnosis and, consequently, in therapy may occur. Case Reports Case 1 A twenty-four-year-old man complaints of total, painless,
UROLOGY
/
OCTOBER
1980
/
presented with macroscopic
VOLUME XVI, NUMBER 4
hematuria. Findings on physical examination were normal. Urinalysis showed erythrocyturia. There was no bacterial infection. Biochemically, there were no abnormalities, and renal function was intact. Intravenous pyelography revealed good function bilaterally and normal collecting systems, but in the proximal third of the right ureter an oblong nonobstructing regular filling defect was demonstrated. This was confirmed by retrograde pyelography. On surgical exploration, a fusiform swelling of the proximal one third of the ureter was encountered. After incision of the ureter a polyp with a small stalk was extracted and sectioned at the base. Microscopic examination of the frozen section and examination of permanent sections after the operation confirmed the diagnosis of fibroepithelial polyp of the ureter. The patient’s postoperative course was uneventful. His intravenous pyelographies have been normal, and he has remained in excellent health for fifteen years after surgery. Case 2 A man, forty-five years of age, was admitted to the hospital in September, 1977. His only symptom was painless macroscopic hematuria three days before admission. Physical examination as well as urinalysis and blood chemistry were unremarkable. On intravenous pyelography the right collecting system and ureter were normal, but on the left side a mild hydronephrosis was noticed, caused by a long
355
FIGURE 1. (A and B) lntravenous urography: irregular filling defect in lower part of ureter with marked dilation of ureteral segment involved.
m irregular filling defect in the lowest one third of the ureter (Fig. 1). Retrograde pyelography and selective sampling of urine from the left ureter failed. The diagnosis was nevertheless determined as a papillary tumor of the ureter, and an en bloc nephroureterectomy was performed with excision of the cuff of the bladder, without incision of the ureter. Exploration of the specimen postrevealed a 12-cm. long operatively, however, ureteral polyp (Fig. 2), which was confirmed microscopically. Convalescence was uneventful. However, retrospectively, a local resection would have been preferable in this case. Case 3 A man, born in 1949, had shown urologic abnormalities since 1962. At that time an intravenous pyelogram had been performed because of complaints of left flank pain. Bilateral hydronephrosis, mild on the right and more pronounced on the left side, caused by stenosis of pelvic-ureteral junction had been found. A conservative approach initially was proposed, but because of recurrent attacks of flank pain and increasing hydronephrosis, a left pyeloplasty was performed in 1970 elsewhere. Afterward the patient was treated for recurrent nephrolithiasis and recurrent prostatitis.
356
FIGURE 2. Macroscopic view of specimen. Long irregular polypoid lesion attached to ureter with small base.
Because of complaints of urinary tract infections, he was first seen by us in August, 1975. Stable hydronephrosis was noted on an excretory urogram. In March, 1978, a new episode of prostatitis and colicky pain prompted us to repeat the intravenous urography which revealed an obvious filling defect in the right upper ureter. This lesion was confirmed by retrograde
UROLOGY
/ OCTOBER1980
/ VOLUMEXVI
NUMBER4
FIGURE 3. Intravenous urography (A) shows small, long filling a!&ec t in lower ureter. Defect is located in middle third of ureter during retrograde pyelography (B).
tion a slight tenderness of the right flank as well as mild pain on palpation of the suprapubic region was noted. Urinalysis, urine cytology, as well as routine blood studies were normal. Intravenous pyelography revealed good function bilaterally and normal collecting systems. In the lower part of the right ureter an oblong filling defect (Fig. 3A) of 8 cm. was suspected and confirmed by retrograde pyelography. It was noted that the filling defect had shifted to a much more proximal location in the ureter suggesting a mobile lesion (Fig. 3B). The regular aspect and mobility of the lesion allowed the preoperative diagnosis of a benign polyp of the ureter. On exploration a slender 1.5-mm. broad and smooth polyp with a small stalk was found and excised. Microscopic examination showed fibrous stroma lined by normal transitional cell epithelium. The patient’s postoperative course was uneventful.
pyelography. At surgery a polypoid lesion was encountered at the ureteropelvic junction. The ureteropelvic and proximal ureter containing the small mass was excised, and a ureteropelvic reanastomosis according to Anderson-Hynes was performed. Microscopic examination revealed a polyp of fibrous stroma lined by a normal transitional cell epithelium. Convalescence was uneventful. Excretory urography six months postoperatively showed good function and a nearly normal right collecting system. Case 4 A forty-seven-year-old woman had complained of vague pain in the right groin with radiation to the right flank for one-half year. There were no previous complaints of urinary tract infection. Two days before admission to the hospital in March, 1978, painless macroscopic hematuria occurred. On physical examina-
UROLOCY
i
OCTOBER
1980
/
VOLUME
XVI
NUMBER
4
357
Comment Fibroepithelial polyps of the ureter are extremely rare. Although several case reports of such polyps had been published earlier, Vest in I9453 gave the first review of these benign lesions of the ureter. This review contains not only mesodermal tumors but also deals particularly with benign epithelial polyps. Brock (1960)* and more recently Stuppler and Kandzari (1975)’ gave reviews of the literature concerning benign fibroepithelial tumors of the ureter. In the earlier literature histologic documentation is rather scarce, and therefore our review was limited to the reports published since 1950 with clear histopathologic description of the lesions. In this review 108 cases of benign fibroepithelial tumors have been collected. With our additional cases, a total of 112 patients can be discussed. It is shown that the age distribution exhibits a predominance for the second, third, and fourth decade. These tumors may also occur in children and to the present time 16 cases of children under the age of fifteen years have been described. The youngest patient was a neonate, the eldest seventy-six years of age. A predominance of men over women (3 to 2 ratio) is clear. The lesion occurs by slight preference in the left ureter and is usually located in the upper part and is almost exclusively a solitary one. Only 1 case of bilateral ureteral polyps has been published. Only 3 cases of multiple polyps in the same ureter have been described. Association of a ureteral carcinoma with a fibrous polyp has been mentioned in only I case. The cause is not known. Infection, chronic irritation, obstruction as well as “hormonal imbalance” are mentioned as possible causes. Arguments for a congenital origin may possibly be deduced from the fact that the lesion frequently occurs in the earlier decades of life without any demonstrable infection. In the literature one frequently finds a coincidence of polyps and obstruction. The association with lithiasis as mentioned by Ikegami et al.” might indicate the origin to be infection and irritation, but all in all the real cause is not known. Two initial symptoms usually lead to the diagnosis of ureteral fibroma: flank pain and macroscopic or microscopic hematuria. The pain often has a colicky character due to (intermittent) partial obstruction and is sometimes associated with symptoms suggesting urinary tract infection. Association of pain and hematuria also
358
occurs. However, pain alone with normal urinalysis is not uncommon, and sometimes there are no symptoms and the lesion is detected accidentally during examination for other urologic diseases. In the literature of 100 patients with initial symptoms, 41 presented with pain and 39 with hematuria, while 14 patients had both symptoms. About the same diversity is noticed in our 4 patients. Without hematuria, urinalysis is mostly normal and therefore a cytologic evaluation is of no value. In complete agreement with the benign aspects of the lesion there are no abnormalities detectable in blood analysis. It is important to demonstrate the benign character of the lesion before considering surgical intervention. An intravenous urography will reveal the lesion but cannot differentiate between a benign or malignant abnormality. Retrograde pyelography is therefore helpful and even necessary. The polyps, usually located in the proximal part of the ureter, are demonstrated by a retrograde pyelography as sharply defined intraluminal lesions. In contrast, malignant (epithelial) proliferations usually cause more irregular and more obstructive intraluminal defects. In the differential diagnosis nonradiopaque ureteral stones or blood clots should always be considered. Angiography does not provide additional information in the case of a benign tumor. Cystoscopy is only of interest when the polyp protrudes through the ureteral orifice. Since most polyps originate proximally, this occurs only rarely. Intussusception of the ureteral wall by invagination due to the polyp are rarely mentioned. Six cases could be traced in the literature. In spite of extensive preoperative investigation, in many cases the diagnosis will not have been ascertained preoperatively. It is, however, important to keep in mind the possibility of this benign lesion, since surgical correction is always necessary and local resection is the treatment of choice. Even if the preoperative diagnosis remains uncertain, there are on exploration definite signs suggesting a benign or malignant ureteral lesion. A transitional cell carcinoma of the ureter in general causes a more or less pronounced periureteral reaction, sometimes with distinct fixation of the ureter in the retroperitoneal space. Round a ureteral polyp, on the other hand, this fibrous reaction always fails and the ureter is always normal and can be mobilized easily. Once the ureter is opened,
UROLOGY
i
OCTOBER
1980
/
VOLUMEXVI
NUMBER4
which should be done in uncertain cases, the two conditions can easily be differentiated. A polyp has a regular smooth, shiny surface, is pendulous and feels rather solid. An epithelial tumor has a more irregular surface, is weak, vulnerable, and bleeds easily. Local resection is the treatment of choice. This however, is often replaced by a nephroureterectomy. Most nephroureterectomies are executed because of an uncertain preoperative diagnosis with the fear of a malignant disease. Reviewing the 111 cases in the literature, which were operated on, 63 per cent were treated by local resections, but still 41 times (37 per cent) a nephroureterectomy was performed. Such was the case in our third patient in whom a benign lesion was not suspected preoperatively. This radical surgical approach is justified only in the rare case in which a fibrous polyp with an associated carcinoma of the ureter has been found. Furthermore, nephrectomy is necessary when severe obstruction has destroyed the kidney completely. In dubious cases examination of frozen sections may confirm the diagnosis and help to avoid an unnecessary nephrectomy. This conservative surgical approach is more desirable since the lesion usually occurs in
UROLOGY
/
OCTOBER
1980
/
VOLUME XVI
NUMBER4
younger patients. The results of this treatment are excellent. No major complications have been described in the literature. On the other hand an existing hydronephrosis postoperatively usually disappears with complete restoration of renal function. Recurrences after local resection have not been described, and we are not familiar with any such cases. Postbus 9101 6500 HB Nijmegen The Netherlands (DR. DEBRUYNE) References* 1. Stuppler SA, and Kandzari SJ: Fibroepithelial polyp of the ureter. A benign ureteral tumor, Urology 5: 553 (1975). 2. Scott WW: Tumours of the ureter, in Campbell MF, and Harrison JH, Eds: Urology, Philadelphia, W.B. Saunders, 1963, vol. 2, p. 999. 3. Vest SA: Conservative surgery in certain benigu tumors of the ureter, J. Ural. 53: 97 (1945). 4. Brock DR: Benign polyp of the ureter, ibid. 83: 572 (1960). 5. Ikegami K, Ogata J, Seta Y, and Oka M: Five cases of ureteral polyps associated with calculi of the upper urinary tract, Acta Urol. Jap. 12: 377 (1966).
*A complete world literature
list of references of cases reported in the is available from the authors upon request.
359
F. M. J. DEBRUYNE, W. A. MOONEN,
POLYP OF URETER
M.D.
M.D.
A. A. DAENEKINDT, K. P. J. DELAERE,
M.D. M.D.
From the Departments of Urology, Radboud University Hospital Nijmegen, and Catharina City Hospital, Eindhoven, The Netherlands
__________~__
________
-__
ABSTRACT - Fibroepithelial polyp of the ureter, a mesodermal benign tumor, is extremely rare. Four cases of our own prompted us to review the world literature on this subject (108 documented cases since 1950). The mode of presentation, etiology, clinical features, and treatment modalities of this lesion are discussed.
The most recent review concerning fibroepithelial benign polyps of the ureter was done by Stuppler and Kandzari in 1975.’ Since primary ureteral neoplasms are uncommon and benign ureteral tumors are even rarer, it is important to draw attention to this lesion from time to time. Herein we review the cases published since 1950 and add 4 cases of so-called fibroma of the ureter to the literature. Tumors of the ureter can be divided into epithelial and mesodermal neoplasms. The epithelial transitional or squamous tumors are malignant or potentially malignant (even the so-called benign papilloma). Mesodermal tumors are marked by almost benign conditions, which present a great variety of morphologic patterns, such as hemangioma, myxoma, leiomyoma, etc. The benign polyp of the ureter is found most frequently in this group. According to Scott,* 50 per cent of mesodermal tumors are benign polyps of the ureter. At present, diagnosis of this lesion seems easy and the therapy applicable to it appears to be defined clearly. Nevertheless the following cases demonstrate that difficulties in exact preoperative diagnosis and, consequently, in therapy may occur. Case Reports Case 1 A twenty-four-year-old man complaints of total, painless,
UROLOGY
/
OCTOBER
1980
/
presented with macroscopic
VOLUME XVI, NUMBER 4
hematuria. Findings on physical examination were normal. Urinalysis showed erythrocyturia. There was no bacterial infection. Biochemically, there were no abnormalities, and renal function was intact. Intravenous pyelography revealed good function bilaterally and normal collecting systems, but in the proximal third of the right ureter an oblong nonobstructing regular filling defect was demonstrated. This was confirmed by retrograde pyelography. On surgical exploration, a fusiform swelling of the proximal one third of the ureter was encountered. After incision of the ureter a polyp with a small stalk was extracted and sectioned at the base. Microscopic examination of the frozen section and examination of permanent sections after the operation confirmed the diagnosis of fibroepithelial polyp of the ureter. The patient’s postoperative course was uneventful. His intravenous pyelographies have been normal, and he has remained in excellent health for fifteen years after surgery. Case 2 A man, forty-five years of age, was admitted to the hospital in September, 1977. His only symptom was painless macroscopic hematuria three days before admission. Physical examination as well as urinalysis and blood chemistry were unremarkable. On intravenous pyelography the right collecting system and ureter were normal, but on the left side a mild hydronephrosis was noticed, caused by a long
355
FIGURE 1. (A and B) lntravenous urography: irregular filling defect in lower part of ureter with marked dilation of ureteral segment involved.
m irregular filling defect in the lowest one third of the ureter (Fig. 1). Retrograde pyelography and selective sampling of urine from the left ureter failed. The diagnosis was nevertheless determined as a papillary tumor of the ureter, and an en bloc nephroureterectomy was performed with excision of the cuff of the bladder, without incision of the ureter. Exploration of the specimen postrevealed a 12-cm. long operatively, however, ureteral polyp (Fig. 2), which was confirmed microscopically. Convalescence was uneventful. However, retrospectively, a local resection would have been preferable in this case. Case 3 A man, born in 1949, had shown urologic abnormalities since 1962. At that time an intravenous pyelogram had been performed because of complaints of left flank pain. Bilateral hydronephrosis, mild on the right and more pronounced on the left side, caused by stenosis of pelvic-ureteral junction had been found. A conservative approach initially was proposed, but because of recurrent attacks of flank pain and increasing hydronephrosis, a left pyeloplasty was performed in 1970 elsewhere. Afterward the patient was treated for recurrent nephrolithiasis and recurrent prostatitis.
356
FIGURE 2. Macroscopic view of specimen. Long irregular polypoid lesion attached to ureter with small base.
Because of complaints of urinary tract infections, he was first seen by us in August, 1975. Stable hydronephrosis was noted on an excretory urogram. In March, 1978, a new episode of prostatitis and colicky pain prompted us to repeat the intravenous urography which revealed an obvious filling defect in the right upper ureter. This lesion was confirmed by retrograde
UROLOGY
/ OCTOBER1980
/ VOLUMEXVI
NUMBER4
FIGURE 3. Intravenous urography (A) shows small, long filling a!&ec t in lower ureter. Defect is located in middle third of ureter during retrograde pyelography (B).
tion a slight tenderness of the right flank as well as mild pain on palpation of the suprapubic region was noted. Urinalysis, urine cytology, as well as routine blood studies were normal. Intravenous pyelography revealed good function bilaterally and normal collecting systems. In the lower part of the right ureter an oblong filling defect (Fig. 3A) of 8 cm. was suspected and confirmed by retrograde pyelography. It was noted that the filling defect had shifted to a much more proximal location in the ureter suggesting a mobile lesion (Fig. 3B). The regular aspect and mobility of the lesion allowed the preoperative diagnosis of a benign polyp of the ureter. On exploration a slender 1.5-mm. broad and smooth polyp with a small stalk was found and excised. Microscopic examination showed fibrous stroma lined by normal transitional cell epithelium. The patient’s postoperative course was uneventful.
pyelography. At surgery a polypoid lesion was encountered at the ureteropelvic junction. The ureteropelvic and proximal ureter containing the small mass was excised, and a ureteropelvic reanastomosis according to Anderson-Hynes was performed. Microscopic examination revealed a polyp of fibrous stroma lined by a normal transitional cell epithelium. Convalescence was uneventful. Excretory urography six months postoperatively showed good function and a nearly normal right collecting system. Case 4 A forty-seven-year-old woman had complained of vague pain in the right groin with radiation to the right flank for one-half year. There were no previous complaints of urinary tract infection. Two days before admission to the hospital in March, 1978, painless macroscopic hematuria occurred. On physical examina-
UROLOCY
i
OCTOBER
1980
/
VOLUME
XVI
NUMBER
4
357
Comment Fibroepithelial polyps of the ureter are extremely rare. Although several case reports of such polyps had been published earlier, Vest in I9453 gave the first review of these benign lesions of the ureter. This review contains not only mesodermal tumors but also deals particularly with benign epithelial polyps. Brock (1960)* and more recently Stuppler and Kandzari (1975)’ gave reviews of the literature concerning benign fibroepithelial tumors of the ureter. In the earlier literature histologic documentation is rather scarce, and therefore our review was limited to the reports published since 1950 with clear histopathologic description of the lesions. In this review 108 cases of benign fibroepithelial tumors have been collected. With our additional cases, a total of 112 patients can be discussed. It is shown that the age distribution exhibits a predominance for the second, third, and fourth decade. These tumors may also occur in children and to the present time 16 cases of children under the age of fifteen years have been described. The youngest patient was a neonate, the eldest seventy-six years of age. A predominance of men over women (3 to 2 ratio) is clear. The lesion occurs by slight preference in the left ureter and is usually located in the upper part and is almost exclusively a solitary one. Only 1 case of bilateral ureteral polyps has been published. Only 3 cases of multiple polyps in the same ureter have been described. Association of a ureteral carcinoma with a fibrous polyp has been mentioned in only I case. The cause is not known. Infection, chronic irritation, obstruction as well as “hormonal imbalance” are mentioned as possible causes. Arguments for a congenital origin may possibly be deduced from the fact that the lesion frequently occurs in the earlier decades of life without any demonstrable infection. In the literature one frequently finds a coincidence of polyps and obstruction. The association with lithiasis as mentioned by Ikegami et al.” might indicate the origin to be infection and irritation, but all in all the real cause is not known. Two initial symptoms usually lead to the diagnosis of ureteral fibroma: flank pain and macroscopic or microscopic hematuria. The pain often has a colicky character due to (intermittent) partial obstruction and is sometimes associated with symptoms suggesting urinary tract infection. Association of pain and hematuria also
358
occurs. However, pain alone with normal urinalysis is not uncommon, and sometimes there are no symptoms and the lesion is detected accidentally during examination for other urologic diseases. In the literature of 100 patients with initial symptoms, 41 presented with pain and 39 with hematuria, while 14 patients had both symptoms. About the same diversity is noticed in our 4 patients. Without hematuria, urinalysis is mostly normal and therefore a cytologic evaluation is of no value. In complete agreement with the benign aspects of the lesion there are no abnormalities detectable in blood analysis. It is important to demonstrate the benign character of the lesion before considering surgical intervention. An intravenous urography will reveal the lesion but cannot differentiate between a benign or malignant abnormality. Retrograde pyelography is therefore helpful and even necessary. The polyps, usually located in the proximal part of the ureter, are demonstrated by a retrograde pyelography as sharply defined intraluminal lesions. In contrast, malignant (epithelial) proliferations usually cause more irregular and more obstructive intraluminal defects. In the differential diagnosis nonradiopaque ureteral stones or blood clots should always be considered. Angiography does not provide additional information in the case of a benign tumor. Cystoscopy is only of interest when the polyp protrudes through the ureteral orifice. Since most polyps originate proximally, this occurs only rarely. Intussusception of the ureteral wall by invagination due to the polyp are rarely mentioned. Six cases could be traced in the literature. In spite of extensive preoperative investigation, in many cases the diagnosis will not have been ascertained preoperatively. It is, however, important to keep in mind the possibility of this benign lesion, since surgical correction is always necessary and local resection is the treatment of choice. Even if the preoperative diagnosis remains uncertain, there are on exploration definite signs suggesting a benign or malignant ureteral lesion. A transitional cell carcinoma of the ureter in general causes a more or less pronounced periureteral reaction, sometimes with distinct fixation of the ureter in the retroperitoneal space. Round a ureteral polyp, on the other hand, this fibrous reaction always fails and the ureter is always normal and can be mobilized easily. Once the ureter is opened,
UROLOGY
i
OCTOBER
1980
/
VOLUMEXVI
NUMBER4
which should be done in uncertain cases, the two conditions can easily be differentiated. A polyp has a regular smooth, shiny surface, is pendulous and feels rather solid. An epithelial tumor has a more irregular surface, is weak, vulnerable, and bleeds easily. Local resection is the treatment of choice. This however, is often replaced by a nephroureterectomy. Most nephroureterectomies are executed because of an uncertain preoperative diagnosis with the fear of a malignant disease. Reviewing the 111 cases in the literature, which were operated on, 63 per cent were treated by local resections, but still 41 times (37 per cent) a nephroureterectomy was performed. Such was the case in our third patient in whom a benign lesion was not suspected preoperatively. This radical surgical approach is justified only in the rare case in which a fibrous polyp with an associated carcinoma of the ureter has been found. Furthermore, nephrectomy is necessary when severe obstruction has destroyed the kidney completely. In dubious cases examination of frozen sections may confirm the diagnosis and help to avoid an unnecessary nephrectomy. This conservative surgical approach is more desirable since the lesion usually occurs in
UROLOGY
/
OCTOBER
1980
/
VOLUME XVI
NUMBER4
younger patients. The results of this treatment are excellent. No major complications have been described in the literature. On the other hand an existing hydronephrosis postoperatively usually disappears with complete restoration of renal function. Recurrences after local resection have not been described, and we are not familiar with any such cases. Postbus 9101 6500 HB Nijmegen The Netherlands (DR. DEBRUYNE) References* 1. Stuppler SA, and Kandzari SJ: Fibroepithelial polyp of the ureter. A benign ureteral tumor, Urology 5: 553 (1975). 2. Scott WW: Tumours of the ureter, in Campbell MF, and Harrison JH, Eds: Urology, Philadelphia, W.B. Saunders, 1963, vol. 2, p. 999. 3. Vest SA: Conservative surgery in certain benigu tumors of the ureter, J. Ural. 53: 97 (1945). 4. Brock DR: Benign polyp of the ureter, ibid. 83: 572 (1960). 5. Ikegami K, Ogata J, Seta Y, and Oka M: Five cases of ureteral polyps associated with calculi of the upper urinary tract, Acta Urol. Jap. 12: 377 (1966).
*A complete world literature
list of references of cases reported in the is available from the authors upon request.
359