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Unilateral Hydroureteronephrosis in Asymptomatic Woman
0022-5347 /82/1283-0562$02.00/0 Vol. 128, September Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1982 by The Williams & Wilkins Co.
Clinicopathologi cal Conference
P. TRUETT RAY,* ERICH K. LANG,t JOHN R. STRIPLING, III,:j: SUSAN SCIONEAUX,§ JERRY W. SULLIVANJI AND RODNEY A. APPELL,, ** From the Departments of Urology, Radiology and Pathology, Louisiana State University Medical Center, New Orleans, Louisiana PRESENTATION OF A CASEtt
Dr. P. Truett Ray. A 32-year-old black woman was referred by her family physician to an outlying hospital for evaluation of dysmenorrhea and inter-menstrual pelvic pain 8 months in duration. The patient was evaluated by the gynecologic service at that hospital and the diagnosis was uterine leiomyomas. The treatment plan consisted of a transabdominal hysterectomy. A preoperative excretory urogram (IVP) revealed left hydronephrosis. It was believed by the gynecology service that the hydronephrosis was secondary to the leiomyomas and an operation was performed without urological investigation. The surgical report indicated that the left ureter was not identified and that portions of the leiomyomas were left in place on each pelvic sidewall because of difficulty in dissection. The pathology report from that institution indicated benign, intramural leiomyomas and chronic endocervicitis. A followup IVP 6 weeks postoperatively demonstrated no significant change in the degree of left hydronephrosis and the patient was transferred to our hospital for urological evaluation. She denied a history of urinary tract infection, calculous disorder, hematuria or voiding dysfunction. She was nulliparous. On physical examination the patient was afebrile and normotensive, and an abdominal examination revealed no abnormality other than a healed, lower midline scar. Laboratory evaluations, which included a complete blood count, blood urea nitrogen, serum creatinine, serum uric acid, serum calcium, intermediate strength purified protein derivative, urinalysis and urine culture, and urinary cytology were within normal limits. Cystourethroscopy, which revealed no abnormality. However, a left bulb-tip retrograde pyelogram demonstrated a severely narrowed 2 cm. segment of the distal left ureter, with a negative filling defect noted on a drainage film. Attempts to pass various sizes of ureteral catheters beyond the obstruction failed and, likewise, a brush biopsy catheter could not be passed. RADIOGRAPHIC INTERPRETATION
Dr. Erich K. Lang. The IVP demonstrated a hydronephrotic appearance of the left pyelocaliceal structure with delayed drainage (fig. 1). The precise site of obstruction was not identified on these films. The retrograde ureterogram demonstrated a severely narrowed segment of the distal left ureter, where * Chief Resident, Department of Urology.
t Professor and Chairman, Department of Radiology.
t Clinical Instructor, Department of Urology.
§ Chief Resident, Department of Pathology.
2 cm. of the intramural portion of the ureter appear to be involved (fig. 2). A drainage film after the ureterogram demonstrated a shelf-like defect in the distal portion of the left ureter (fig. 3). This defect simulated the "apple core" deformity characteristically associated with infiltrative transitional cell carcinoma. Differentiation against a transitional cell carcinoma of the ureter or a transitional cell carcinoma of the bladder extending into the ureter is not possible from these films. With these characteristics suggestive of a lesion intrinsic to the ureter one must consider calculous disease or blood clot in addition to tumor. Of course, hematuria would most likely have been observed if this were a clot. I believe that the radiographic findings would be most characteristically associated with an infiltrative transitional cell carcinoma. CLINICAL DIAGNOSIS
Dr. Rodney A. Appell. Essentially, we are presented with a filling defect of the ureter of unknown etiology. While I agree with Doctor Lang that the most obvious diagnosis from the film would be that of an infiltrative tumor there are some points against this. In cases of ureteral tumors the ureter usually is dilated immediately below the growth, in contradistinction to that found in calculous obstruction, in which case the ureter is collapsed below the stone. 1 This so-called "goblet sign" is not present in these films, in which case the ureter is of normal caliber below the defect. Additionally, transitional cell lesions of the ureter are rare in pre-menopausal women and the most common cause of metastatic implants to the ureter are from carcinomas of the uterine cervix, which already has been removed in this patient and was benign. The normal urinalysis appears to eliminate the possibility of blood clots, sloughed papilla and nonopaque calculous disease. The negative purified protein derivative would tend to eliminate the possibility of a tuberculous stricture, especially in light of the concurrent filling defect and lack of apparent renal involvement. Ureteritis cystica usually demonstrates alternate areas of dilatation and constriction, and involves a much larger segment of the ureter. Less common disease processes that must be included to complete the differential diagnosis are such entities as malacoplakia and endometriosis. Again, the lack of clinical findings, the normal urinalysis and the lack of bladder involvement tend to eliminate these possibilities. I would lean toward the diagnosis of transitional cell carcinoma of the ureter. Doctor Ray. The patient was informed of the diagnostic possibilities and scheduled for an operation. DISCUSSION OF SURGERY
II Assistant Professor, Department of Urology.
Doctor Ray. The patient underwent exploration through the lower midline abdominal incision. The left ureter was identified as it crossed the left iliac vessels. Distal to this point the ureter was displaced laterally and densely adherent to a cystic mass, which measured approximately 5 X 6 cm. This mass proved to be a chocolate cyst involving most of the left ovary. Left
1 Assistant Professor, Department of Urology.
** Requests for reprints: Department of Urology, Louisiana State University Medical Center, 1542 Tulane Ave., New Orleans, Louisiana 70112. tt Presented at Urological Death and Complications Conference, Louisiana State University Medical Center, New Orleans, Louisiana, April 1981. 562
1Jl'>IJLAfI'EilAL flYDitOTJRE~fERONE:PHROSIS IN iiSYf./fP'I'0IvfA01'lC -vifOlV[A.N
Fm. 3. Drainage film of retrograde ureterogram demonstrates shelflike defect (arrow) in distal portion of left ureter. Defect simulates "apple core" deformity, characteristically associated with infiltrative transitional cell carcinoma.
Fw. 1. IVP, 25-minute roentgenogram, demonstrates hydronephrotic appearance of left pyelocaliceal structures, with delayed drainage from left side. Precise site of obstruction is not identifiable nor does obstruction appear to be complete on basis of residual function in left kidney.
cm. above the left ureterovesical junction. The bladder was anteriorly and the left ureteral orifice was dissected out a cuff of bladder, and by a combined intravesical and extravesical the left ureter was freed and then transected 5 cm. the uppermost extent of the induratioR A simple left ureteroneocystostomy was without establishment of a submucosal tunnel. The postoperative course was uneventful and a followup IVP just before the patient was ,.c.:,...-»,r~Pn from the hospital was normal, Did you obtain a frozen section of the ureter at the time the operation? Dr. John R. Stripling. The presence of the chocolate ovarian cyst at laparotomy alerted us to the possibility of an inflammatory lesion of the genital tract involving the ureter, such as endometriosis. The extensive fibrosis and perim·eteral inflanunatory reaction to confinn this and vve thought that a conservative was in order. 'Ne also thought that if we were wrong and this was caused infiltrative transitional cell carcinoma of the ureter then the survival rate would not be =,~~.~,,A~ nephroureterectomy. In other a frozen section at the time of the operation would not have altered the ultimately n1>ctC\rTI°!<'d Doctor I would like to ask Doctor Sullivan if segrnental resection the ureter is an"-,('"'''""'"'" in tha management of transitional cell carcin-:ima of the ureter? Dr. w. Sullivan. UC1',l,,Ho.uc,,c µc,JC.<7'.H.LHC
Fm. 2. Retrograde ureterogram demonstrates severely narrowed segment of distal left ureter. Intramural portion of ureter for 2 cm. segment appears to be involved. Compensatory dilatation of pelvic portion of ureter above this strictured segment is noted.
salpingo-oophorectomy and ureterolysis were performed. The ureter was then traced down to an area of marked induration and periureteral inflammatory reaction, which was located 2
carcinoma tends to be the ro0nn,rf,,c,rl incidence contralateral ureteral carcinoma <2 per cent. 2 If the ureteral cancer occurs in the distal ureter and a distal ur,etE,rect<)my with a cuff of bladder is performed there are few ""''~'°"""'" cases of recurrent ~.reteral cancer. 3 If a segm.ental ureterectomy is performed and the lesion is grade I, likewise, the recurrence proximally and distally is infrequent, and if the lesion is grade II or greater then the rate of recurrence is frequent. 4 However, it must be realized that even with nephroureterectomy in grade III or IV lesions the survival rate is not improved over simple segmental resection. 5 Regardless of whether segmental ureteral resection or radical nephroureterectomy is performed the patient must be followed closely, since the incidence of subsequent bladder cancer is high.
564
CLINICOPATHOLOGICAL CONFERENCE DISCUSSION OF PATHOLOGY
Dr. Susan Scioneaux. The distal 5 cm. of the left ureter had been removed. The ureter was dilated and thickened by hemorrhagic, brown-gray, pliable soft tissue and measured up to 1.5 cm. in largest diameter. Microscopic evaluation of the proximal portion revealed a patent lumen, lined by well preserved transitional epithelium and surrounded by an intact muscular wall. There were acute and chronic inflammatory cells scattered focally throughout the ureter al tissue. The distal portion of the ureter was distorted and the wall was thickened. Lying between the smooth muscle bundles of the ureter were numerous glandular structures lined by tall columnar epithelial cells with a tendency to pseudostratification (fig. 4, A). A well developed endometrial stroma surrounded these glands and consisted of plump, basophilic, elongated cells that were distinguished easily from surrounding muscular and connective tissue. Evidence of old and recent hemorrhage was present within the glands, the surrounding stroma and the connective tissue. Some glands were dilated and contained debris and hemosiderin-laden macrophages (fig. 4, B). There was an extensive fibrotic reaction, collections of lymphocytes and macrophages were within the ureteral wall, and the original lumen was difficult to demonstrate. Polypoid endometrial tissue filled the ureteral lumen. The histologic features in this case are compatible with intrinsic ureteral involvement with endometriosis. Ureteral endometriosis is a rare disorder and the lesion, as in this case, nearly always occurs below the pelvic brim and only rarely is a cause of ureteral obstruction. In the extrinsic type there are endometrial glands and stroma in the adventitia and surround-
FIG. 4. A, intrinsic endometriosis of ureter. Low power photomicrograph demonstrates that well developed endometrial glands and surrounding stroma are interspersed between smooth muscle bundles of ureteral wall. H & E, reduced from X25. B, dilated endometrial glands are cystiform and contain hemosiderin-laden macrophages and debris. H & E, reduced from X 100.
ing connective tissue of the ureters, with ureteral compression resulting from pressure caused by an endometriotic site in the parietal perineum, ovary or broad ligament. In the intrinsic variety the endometrial glands and stroma are found within the ureteral wall, and this probably represents a more severe disorder with invasive elements of a pre-existing extrinsic form. Kerr reported only 9 of 43 cases of ureteral endometriosis as intrinsic. 6 The ovary and fallopian tube examined in conjunction with this case revealed cystic follicles and chronic salpingitis but they demonstrated no involvement with endometriosis. CLINICAL DISCUSSION
Doctor Appell. Although endometriosis is a common disease, reported in 15 per cent of pre-menopausal women,7 urinary tract involvement occurs in <5 per cent of the patients with the bladder as the most commonly involved organ. 6 Stanley and associates have suggested that ureteral and bladder involvement arises from pelvic foci, with implantation owing to passage by lymphatics or the blood stream or, perhaps, to Sampson's well known theory ofretrograde menstruation. 8• ~ Abdel-Shahid and associates reported histologic evidence that supports the dissemination of endometrium via venous channels. 10 Since the blood supply of the ureters is variable enough avenues are open for ureteral involvement at any level, as exemplified by Rosemberg and Jacobs, who demonstrated ureteral involvement above the pelvic brim. 11 The clinical picture of ureteral endometriosis is nonspecific. Hematuria occurs in 25 per cent of the patients and is cyclical because of sloughing characteristically most pronounced during menstruation. Ochsner and Markland noted that 14 of 18 patients complained of cyclic flank pain and stressed the relation with menstruation. 12 Kerr, on the other hand, thought that the clinical picture of ureteral endometriosis could include flank pain when obstruction existed but this usually was not cyclical. 6 Although involvement of the ureters by endometriosis usually occurs in the presence of widespread pelvic disease it also may occur without evidence of endometriosis elsewhere. 7 Our patient was asymptomatic, had no hematuria and was without evidence of endometriosis elsewhere in the pelvis on ultimate surgical exploration. Additionally, radiologic findings are nonspecific and, for this reason, it is almost impossible to diagnose ureteral endometriosis preoperatively. The cyclical change in the mass can cause presentation, as in this case, with nearly complete obstruction of the ureter and a shelf-like filling defect highly reminiscent of an infiltrative transitional cell carcinoma. On the other hand, the radiograph may show a mere irregularity of the ureteral lumen when the stage of endocrine function causes regression in the size of the lesion. Our case had the radiographic characteristic appearance of an intraluminal tumor producing a characteristic tumor shelf and differentiation against a transitional cell carcinoma of the ureter was not possible. In this case the IVP was of value in demonstrating the severity of the obstruction and its approximate location. The retrograde ureterogram was helpful in evaluating the precise location, extent, configuration and degree of stenosis. Doctor Lang. Variability in the degree of obstruction on the IVP is dependent on the endocrine phase, in which the examination has been done. Only documentation of a substantial change in the appearance of the lesion within a 4-week period would strongly support the diagnosis of an intramural endometrioma. Doctor Appell. The aims of treatment in ureteral endometriosis are preservation ofrenal function and basic disease control. Operations of this type are notoriously difficult because of extensive fibrosis and periureteral inflammatory reaction, and the area of involvement should dictate the extent of operation involved. Ureteral obstruction owing to endometriosis should be treated and ought to be accomplished conservatively by lysis of periureteral adhesions, resection of endometriotic implants
UN";J_,f-l')~ER.AL }IYDROlJRT'.-l'ERO:t-~EPHROS!S
2..nd ur2terBl resection v1ith either re&nasto:i.~rosis or urete:roneocystostomy. c;,·,~n-~n•An vvho are opposed to segn).eJC:lt:H resection of the ureter for transitional cell carcinoma should certainly consider frozen pathologic sections during the operation to prevent an unnecessary total nephroureterectomy in the patient whose lesion is a benign endometrioma, which occurred in a case reported by Brock. 13 REFERENCES
5.
6. 7.
8.
9.
1. Bergman, H., Friedenberg, R. M. and Sayegh, V.: New roentgeno-
logic signs of carcinoma of the ureter. Amer. J. Roentgen., 86: 707, 1961. 2. Bloom, N. A., Vidone, R. A. and Lytton, B.: Prii.-nary carcinoma of the ureter: a report of 102 new cases. J. Urol., Hl3: 590, 1970. 3. Strong, D. W., Pearse, H. D., Tank, E. S., Jr. and Hodges, C. V.: The ureteral stump after nephroureterectomy. J. Urol., 115: 654, 1976. 4. Murphy, D. M., Zincke, H. and Furlow, W. L.: Management of high grade transitional cell cancer of the upper urinary tract. J. Urol., 125: 25, 1981.
10.
11. 12. 13.
N. it.1.L, l\Tocks, B. I:{> Daley, f_L Blizer P_ H. and Pct-.:-khurst, · Prognostic factors b carcinoma of the urete:r. J. Urol., 125: 632, 1981. Kerr, W. S., Jr.: Endometriosis involving the urinary tract. Clin. Obst. Gynec., !}: 331, 1966. Fagan, C. J.: Endometriosis: clinical and roentgenographic manifestations. Rad. Clin. N. Amer., 12: 109, 1974. Stanley, KE., Utz, D. C. and Dockerty, M. B.: Clinically significant endometriosis of the urinary tract. Surg., Gynec. & Obst., 120: 491, 1965. Sampson, J. A.: The life history of ovarian hematomas (hemorrhagic cysts) of the endometrial (Miillerian) type. Amer. J. Obst. Gynec., 4: 451, 1922. Abdel-Shahid, R. B., Beresford, J.M. and Curry, R.H.: Endometriosis of the ureter with vascular involvement. Obst. Gynec., 43: 113, 1974. Rosemberg, S. K and Jacobs. H.: Endometriosis of the upper ureter. J. Urol., 121: 512, 1979. Ochsner, T. and Markland, C.: Endometriosis obstructing the ureter. J. Urol., 98: 462, 1967. Brock, D.R.: Ureteral obstruction from endometriosis. J. Urol., 83: 100, 1960. 1
THE JOURNAL OF UROLOGY
Copyright© 1982 by The Williams & Wilkins Co.
Clinicopathologi cal Conference
P. TRUETT RAY,* ERICH K. LANG,t JOHN R. STRIPLING, III,:j: SUSAN SCIONEAUX,§ JERRY W. SULLIVANJI AND RODNEY A. APPELL,, ** From the Departments of Urology, Radiology and Pathology, Louisiana State University Medical Center, New Orleans, Louisiana PRESENTATION OF A CASEtt
Dr. P. Truett Ray. A 32-year-old black woman was referred by her family physician to an outlying hospital for evaluation of dysmenorrhea and inter-menstrual pelvic pain 8 months in duration. The patient was evaluated by the gynecologic service at that hospital and the diagnosis was uterine leiomyomas. The treatment plan consisted of a transabdominal hysterectomy. A preoperative excretory urogram (IVP) revealed left hydronephrosis. It was believed by the gynecology service that the hydronephrosis was secondary to the leiomyomas and an operation was performed without urological investigation. The surgical report indicated that the left ureter was not identified and that portions of the leiomyomas were left in place on each pelvic sidewall because of difficulty in dissection. The pathology report from that institution indicated benign, intramural leiomyomas and chronic endocervicitis. A followup IVP 6 weeks postoperatively demonstrated no significant change in the degree of left hydronephrosis and the patient was transferred to our hospital for urological evaluation. She denied a history of urinary tract infection, calculous disorder, hematuria or voiding dysfunction. She was nulliparous. On physical examination the patient was afebrile and normotensive, and an abdominal examination revealed no abnormality other than a healed, lower midline scar. Laboratory evaluations, which included a complete blood count, blood urea nitrogen, serum creatinine, serum uric acid, serum calcium, intermediate strength purified protein derivative, urinalysis and urine culture, and urinary cytology were within normal limits. Cystourethroscopy, which revealed no abnormality. However, a left bulb-tip retrograde pyelogram demonstrated a severely narrowed 2 cm. segment of the distal left ureter, with a negative filling defect noted on a drainage film. Attempts to pass various sizes of ureteral catheters beyond the obstruction failed and, likewise, a brush biopsy catheter could not be passed. RADIOGRAPHIC INTERPRETATION
Dr. Erich K. Lang. The IVP demonstrated a hydronephrotic appearance of the left pyelocaliceal structure with delayed drainage (fig. 1). The precise site of obstruction was not identified on these films. The retrograde ureterogram demonstrated a severely narrowed segment of the distal left ureter, where * Chief Resident, Department of Urology.
t Professor and Chairman, Department of Radiology.
t Clinical Instructor, Department of Urology.
§ Chief Resident, Department of Pathology.
2 cm. of the intramural portion of the ureter appear to be involved (fig. 2). A drainage film after the ureterogram demonstrated a shelf-like defect in the distal portion of the left ureter (fig. 3). This defect simulated the "apple core" deformity characteristically associated with infiltrative transitional cell carcinoma. Differentiation against a transitional cell carcinoma of the ureter or a transitional cell carcinoma of the bladder extending into the ureter is not possible from these films. With these characteristics suggestive of a lesion intrinsic to the ureter one must consider calculous disease or blood clot in addition to tumor. Of course, hematuria would most likely have been observed if this were a clot. I believe that the radiographic findings would be most characteristically associated with an infiltrative transitional cell carcinoma. CLINICAL DIAGNOSIS
Dr. Rodney A. Appell. Essentially, we are presented with a filling defect of the ureter of unknown etiology. While I agree with Doctor Lang that the most obvious diagnosis from the film would be that of an infiltrative tumor there are some points against this. In cases of ureteral tumors the ureter usually is dilated immediately below the growth, in contradistinction to that found in calculous obstruction, in which case the ureter is collapsed below the stone. 1 This so-called "goblet sign" is not present in these films, in which case the ureter is of normal caliber below the defect. Additionally, transitional cell lesions of the ureter are rare in pre-menopausal women and the most common cause of metastatic implants to the ureter are from carcinomas of the uterine cervix, which already has been removed in this patient and was benign. The normal urinalysis appears to eliminate the possibility of blood clots, sloughed papilla and nonopaque calculous disease. The negative purified protein derivative would tend to eliminate the possibility of a tuberculous stricture, especially in light of the concurrent filling defect and lack of apparent renal involvement. Ureteritis cystica usually demonstrates alternate areas of dilatation and constriction, and involves a much larger segment of the ureter. Less common disease processes that must be included to complete the differential diagnosis are such entities as malacoplakia and endometriosis. Again, the lack of clinical findings, the normal urinalysis and the lack of bladder involvement tend to eliminate these possibilities. I would lean toward the diagnosis of transitional cell carcinoma of the ureter. Doctor Ray. The patient was informed of the diagnostic possibilities and scheduled for an operation. DISCUSSION OF SURGERY
II Assistant Professor, Department of Urology.
Doctor Ray. The patient underwent exploration through the lower midline abdominal incision. The left ureter was identified as it crossed the left iliac vessels. Distal to this point the ureter was displaced laterally and densely adherent to a cystic mass, which measured approximately 5 X 6 cm. This mass proved to be a chocolate cyst involving most of the left ovary. Left
1 Assistant Professor, Department of Urology.
** Requests for reprints: Department of Urology, Louisiana State University Medical Center, 1542 Tulane Ave., New Orleans, Louisiana 70112. tt Presented at Urological Death and Complications Conference, Louisiana State University Medical Center, New Orleans, Louisiana, April 1981. 562
1Jl'>IJLAfI'EilAL flYDitOTJRE~fERONE:PHROSIS IN iiSYf./fP'I'0IvfA01'lC -vifOlV[A.N
Fm. 3. Drainage film of retrograde ureterogram demonstrates shelflike defect (arrow) in distal portion of left ureter. Defect simulates "apple core" deformity, characteristically associated with infiltrative transitional cell carcinoma.
Fw. 1. IVP, 25-minute roentgenogram, demonstrates hydronephrotic appearance of left pyelocaliceal structures, with delayed drainage from left side. Precise site of obstruction is not identifiable nor does obstruction appear to be complete on basis of residual function in left kidney.
cm. above the left ureterovesical junction. The bladder was anteriorly and the left ureteral orifice was dissected out a cuff of bladder, and by a combined intravesical and extravesical the left ureter was freed and then transected 5 cm. the uppermost extent of the induratioR A simple left ureteroneocystostomy was without establishment of a submucosal tunnel. The postoperative course was uneventful and a followup IVP just before the patient was ,.c.:,...-»,r~Pn from the hospital was normal, Did you obtain a frozen section of the ureter at the time the operation? Dr. John R. Stripling. The presence of the chocolate ovarian cyst at laparotomy alerted us to the possibility of an inflammatory lesion of the genital tract involving the ureter, such as endometriosis. The extensive fibrosis and perim·eteral inflanunatory reaction to confinn this and vve thought that a conservative was in order. 'Ne also thought that if we were wrong and this was caused infiltrative transitional cell carcinoma of the ureter then the survival rate would not be =,~~.~,,A~ nephroureterectomy. In other a frozen section at the time of the operation would not have altered the ultimately n1>ctC\rTI°!<'d Doctor I would like to ask Doctor Sullivan if segrnental resection the ureter is an"-,('"'''""'"'" in tha management of transitional cell carcin-:ima of the ureter? Dr. w. Sullivan. UC1',l,,Ho.uc,,c µc,JC.<7'.H.LHC
Fm. 2. Retrograde ureterogram demonstrates severely narrowed segment of distal left ureter. Intramural portion of ureter for 2 cm. segment appears to be involved. Compensatory dilatation of pelvic portion of ureter above this strictured segment is noted.
salpingo-oophorectomy and ureterolysis were performed. The ureter was then traced down to an area of marked induration and periureteral inflammatory reaction, which was located 2
carcinoma tends to be the ro0nn,rf,,c,rl incidence contralateral ureteral carcinoma <2 per cent. 2 If the ureteral cancer occurs in the distal ureter and a distal ur,etE,rect<)my with a cuff of bladder is performed there are few ""''~'°"""'" cases of recurrent ~.reteral cancer. 3 If a segm.ental ureterectomy is performed and the lesion is grade I, likewise, the recurrence proximally and distally is infrequent, and if the lesion is grade II or greater then the rate of recurrence is frequent. 4 However, it must be realized that even with nephroureterectomy in grade III or IV lesions the survival rate is not improved over simple segmental resection. 5 Regardless of whether segmental ureteral resection or radical nephroureterectomy is performed the patient must be followed closely, since the incidence of subsequent bladder cancer is high.
564
CLINICOPATHOLOGICAL CONFERENCE DISCUSSION OF PATHOLOGY
Dr. Susan Scioneaux. The distal 5 cm. of the left ureter had been removed. The ureter was dilated and thickened by hemorrhagic, brown-gray, pliable soft tissue and measured up to 1.5 cm. in largest diameter. Microscopic evaluation of the proximal portion revealed a patent lumen, lined by well preserved transitional epithelium and surrounded by an intact muscular wall. There were acute and chronic inflammatory cells scattered focally throughout the ureter al tissue. The distal portion of the ureter was distorted and the wall was thickened. Lying between the smooth muscle bundles of the ureter were numerous glandular structures lined by tall columnar epithelial cells with a tendency to pseudostratification (fig. 4, A). A well developed endometrial stroma surrounded these glands and consisted of plump, basophilic, elongated cells that were distinguished easily from surrounding muscular and connective tissue. Evidence of old and recent hemorrhage was present within the glands, the surrounding stroma and the connective tissue. Some glands were dilated and contained debris and hemosiderin-laden macrophages (fig. 4, B). There was an extensive fibrotic reaction, collections of lymphocytes and macrophages were within the ureteral wall, and the original lumen was difficult to demonstrate. Polypoid endometrial tissue filled the ureteral lumen. The histologic features in this case are compatible with intrinsic ureteral involvement with endometriosis. Ureteral endometriosis is a rare disorder and the lesion, as in this case, nearly always occurs below the pelvic brim and only rarely is a cause of ureteral obstruction. In the extrinsic type there are endometrial glands and stroma in the adventitia and surround-
FIG. 4. A, intrinsic endometriosis of ureter. Low power photomicrograph demonstrates that well developed endometrial glands and surrounding stroma are interspersed between smooth muscle bundles of ureteral wall. H & E, reduced from X25. B, dilated endometrial glands are cystiform and contain hemosiderin-laden macrophages and debris. H & E, reduced from X 100.
ing connective tissue of the ureters, with ureteral compression resulting from pressure caused by an endometriotic site in the parietal perineum, ovary or broad ligament. In the intrinsic variety the endometrial glands and stroma are found within the ureteral wall, and this probably represents a more severe disorder with invasive elements of a pre-existing extrinsic form. Kerr reported only 9 of 43 cases of ureteral endometriosis as intrinsic. 6 The ovary and fallopian tube examined in conjunction with this case revealed cystic follicles and chronic salpingitis but they demonstrated no involvement with endometriosis. CLINICAL DISCUSSION
Doctor Appell. Although endometriosis is a common disease, reported in 15 per cent of pre-menopausal women,7 urinary tract involvement occurs in <5 per cent of the patients with the bladder as the most commonly involved organ. 6 Stanley and associates have suggested that ureteral and bladder involvement arises from pelvic foci, with implantation owing to passage by lymphatics or the blood stream or, perhaps, to Sampson's well known theory ofretrograde menstruation. 8• ~ Abdel-Shahid and associates reported histologic evidence that supports the dissemination of endometrium via venous channels. 10 Since the blood supply of the ureters is variable enough avenues are open for ureteral involvement at any level, as exemplified by Rosemberg and Jacobs, who demonstrated ureteral involvement above the pelvic brim. 11 The clinical picture of ureteral endometriosis is nonspecific. Hematuria occurs in 25 per cent of the patients and is cyclical because of sloughing characteristically most pronounced during menstruation. Ochsner and Markland noted that 14 of 18 patients complained of cyclic flank pain and stressed the relation with menstruation. 12 Kerr, on the other hand, thought that the clinical picture of ureteral endometriosis could include flank pain when obstruction existed but this usually was not cyclical. 6 Although involvement of the ureters by endometriosis usually occurs in the presence of widespread pelvic disease it also may occur without evidence of endometriosis elsewhere. 7 Our patient was asymptomatic, had no hematuria and was without evidence of endometriosis elsewhere in the pelvis on ultimate surgical exploration. Additionally, radiologic findings are nonspecific and, for this reason, it is almost impossible to diagnose ureteral endometriosis preoperatively. The cyclical change in the mass can cause presentation, as in this case, with nearly complete obstruction of the ureter and a shelf-like filling defect highly reminiscent of an infiltrative transitional cell carcinoma. On the other hand, the radiograph may show a mere irregularity of the ureteral lumen when the stage of endocrine function causes regression in the size of the lesion. Our case had the radiographic characteristic appearance of an intraluminal tumor producing a characteristic tumor shelf and differentiation against a transitional cell carcinoma of the ureter was not possible. In this case the IVP was of value in demonstrating the severity of the obstruction and its approximate location. The retrograde ureterogram was helpful in evaluating the precise location, extent, configuration and degree of stenosis. Doctor Lang. Variability in the degree of obstruction on the IVP is dependent on the endocrine phase, in which the examination has been done. Only documentation of a substantial change in the appearance of the lesion within a 4-week period would strongly support the diagnosis of an intramural endometrioma. Doctor Appell. The aims of treatment in ureteral endometriosis are preservation ofrenal function and basic disease control. Operations of this type are notoriously difficult because of extensive fibrosis and periureteral inflammatory reaction, and the area of involvement should dictate the extent of operation involved. Ureteral obstruction owing to endometriosis should be treated and ought to be accomplished conservatively by lysis of periureteral adhesions, resection of endometriotic implants
UN";J_,f-l')~ER.AL }IYDROlJRT'.-l'ERO:t-~EPHROS!S
2..nd ur2terBl resection v1ith either re&nasto:i.~rosis or urete:roneocystostomy. c;,·,~n-~n•An vvho are opposed to segn).eJC:lt:H resection of the ureter for transitional cell carcinoma should certainly consider frozen pathologic sections during the operation to prevent an unnecessary total nephroureterectomy in the patient whose lesion is a benign endometrioma, which occurred in a case reported by Brock. 13 REFERENCES
5.
6. 7.
8.
9.
1. Bergman, H., Friedenberg, R. M. and Sayegh, V.: New roentgeno-
logic signs of carcinoma of the ureter. Amer. J. Roentgen., 86: 707, 1961. 2. Bloom, N. A., Vidone, R. A. and Lytton, B.: Prii.-nary carcinoma of the ureter: a report of 102 new cases. J. Urol., Hl3: 590, 1970. 3. Strong, D. W., Pearse, H. D., Tank, E. S., Jr. and Hodges, C. V.: The ureteral stump after nephroureterectomy. J. Urol., 115: 654, 1976. 4. Murphy, D. M., Zincke, H. and Furlow, W. L.: Management of high grade transitional cell cancer of the upper urinary tract. J. Urol., 125: 25, 1981.
10.
11. 12. 13.
N. it.1.L, l\Tocks, B. I:{> Daley, f_L Blizer P_ H. and Pct-.:-khurst, · Prognostic factors b carcinoma of the urete:r. J. Urol., 125: 632, 1981. Kerr, W. S., Jr.: Endometriosis involving the urinary tract. Clin. Obst. Gynec., !}: 331, 1966. Fagan, C. J.: Endometriosis: clinical and roentgenographic manifestations. Rad. Clin. N. Amer., 12: 109, 1974. Stanley, KE., Utz, D. C. and Dockerty, M. B.: Clinically significant endometriosis of the urinary tract. Surg., Gynec. & Obst., 120: 491, 1965. Sampson, J. A.: The life history of ovarian hematomas (hemorrhagic cysts) of the endometrial (Miillerian) type. Amer. J. Obst. Gynec., 4: 451, 1922. Abdel-Shahid, R. B., Beresford, J.M. and Curry, R.H.: Endometriosis of the ureter with vascular involvement. Obst. Gynec., 43: 113, 1974. Rosemberg, S. K and Jacobs. H.: Endometriosis of the upper ureter. J. Urol., 121: 512, 1979. Ochsner, T. and Markland, C.: Endometriosis obstructing the ureter. J. Urol., 98: 462, 1967. Brock, D.R.: Ureteral obstruction from endometriosis. J. Urol., 83: 100, 1960. 1