Vol. 118, October Printed in U .SA.
THE JOURNAL OF UROLOGY
Copyright © 1977 by The Williams & Wilkins Co.
PELVIC LIPOMATOSIS: 5-YEAR FOLLOWUP DAVID B. CRANE*
AND
M. J. V. SMITH
From the Divisions of Urology, Medical College of Virginia, Virginia Commonwealth U niuersity, Richmond, Virginia and Washington University School of Medicine and John Cochran Veterans Administration Hospital, St. Louis, Missouri
ABSTRACT
Followup of 5 cases presented in 1971 and report of 8 additional cases confirm the initial conclusions that pelvic lipomatosis is a benign but potentially progressive process leading to ureteral obstruction. Young black subjects are affected most commonly. More than 40 per cent of the patients have required permanent urinary diversion. Pelvic lipomatosis has been a subject of increasing interest during the last several years, mainly in the urologic and radiologic literature. More than 65 cases have been reported but long-term followup has been sparse. A followup of the 5 cases initially reported by Lucey and Smith 1 and our successive experience are presented (table 1). ORIGINAL CASE REPORT FOLLOWUPS
Case 1. J.C., MCV 3000770, a 30-year-old black man, died of uremia in 1962. This patient had undergone diversion 6 years previously for ureteral obstruction. The disease had been progressive since the initial evaluation in 1950. No new data were available. Case 2. R. W., MCV 4009403, a 25-year-old black man, was seen initially in 1963. The patient was reported erroneously to have died of uremia but, in fact, was seen again in 1975 with medically uncontrollable hypertension (180/130). Small kidneys bilaterally and marked left pyelocaliectasis were noted on excretory urography (IVP). A cystourethrogram revealed a pear-shaped bladder and a renal arteriogram showed end artery disease with severe nephrosclerosis. Creatinine clearance was 25 to 30 cc per minute. An ileal conduit urinary diversion was done in March 1975. A year later the blood pressure was 148/100 and the patient still required methyldopa and hydralazine. There was no further upper tract deterioration on IVP. Case 3. L.B., MCV 5068008, a 35-year-old black man, was diagnosed in 1969 and has continued to do well with only mild dilatation of the ureters. However, blood pressure on the most recent followup in July 1976 was 180/110 on no medications. A followup IVP has shown no change in the upper tracts. Case 4. G. I., MCV 5269859, a 51-year-old black man, was diagnosed initially in 1968 and remained stable through 1976. The initial hypertension of 260/160 has been controlled with thiazides and diet, and is currently 130/110. Case 5. F. T., MCV 5274235, a 35-year-old thin black man, was seen initially in March 1969 with a blood pressure of 230/ 110. He underwent left nephroureterectomy, right ureteroneocystostomy with ureteral intraperitonealization, right nephrostomy and suprapubic cystostomy. The distal ureter never opened and the patient was converted to an ileal conduit several months later. The 7-year followup shows no change in the right upper tract. Blood pressure in June 1976 was 130/84 on no medications.
left kidney and severe right hydroureteronephrosis. A cystogram and barium enema were consistent with pelvic lipomatosis. A left nephroureterectomy and a right cutaneous ureterostomy were done. In February the patient had a right ureteroneocystostomy with distal ureteral tailoring. Perivesical and periureteral biopsy showed normal adult fat, chronic inflammation of the ureteral mucosa, a thickened wall secondary to fibrosis and hypertrophy of the ureteral musculature. He was treated with prolonged tetracycline but the distal ureter failed to open and the patient has remained with the cutaneous ureterostomy. The right kidney had remained stable through May 1976. Case 7. W. C., MCV 5340327, an obese 65-year-old black man, was evaluated in 1971 for traumatic avulsion of the diaphragm. Ancillary pelvic lipomatosis was noted. By September 1975 mild hypertension had developed. An IVP showed a pelvic lucency, progressive hydroureteronephrosis (the right greater than the left) and a pear-shaped bladder. A cystogram and barium enema were consistent with pelvic lipomatosis. Cystoscopy was attempted but was unsuccessful because the bladder was elevated. The patient also was maintained on tetracycline. The upper tracts were unchanged through May 1976 but the patient is now under treatment with sulfamethoxazole and trimethoprim for chronic urinary tract infections. Case 8. L. P., MCV 5093111, a 47-year-old black man, was seen initially in August 1975 with irritable bladder symptoms and pain radiating into the rectum and right leg. The bladder was elevated by a pelvic lucency but there was no evidence of upper tract obstruction. Bladder biopsy showed cystitis glandularis. A pelvic arteriogram showed diffuse vascularity
NEW CASE REPORTS
Case 6. T. B., MCV 5142258, a 30-year-old black man, was seen initially in January 1974 with non-visualization of the Accepted for publication December 30, 1976. Read at annual meeting of Mid-Atlantic Section, American Urological Association, Dorado Beach, Puerto Rico, September 26-0ctober 1, 1976. *Requests for reprints: Veterans Administration Hospital, St. Louis, Missouri 63125. 547
TABLE
Case-Pt. -Age-Race No.
(yrs.)
1-JC-30-B 2-RW-25-B 3-LB-35-B 4-GI-51-B 5-FT-35-B
6-TB-30-B 7-WC-65-B 8-LP-47-B 9-GL-47-B 10-CW-30-B 11-LB-53-B 12-WM-52-B 13-AL-58-B
1. Current status
Followup (yrs.)
Status as of 1976
Original cases 12 Died of uremia, 1962 13 Ileal conduit, 1975 7 Stable, blood pressure 180/110 8 Stable, blood pressure 130/110 (meds) 7 Lt. nephroureterectomy, ilea! conduit, 1969 Subsequent cases 2.5 Lt. nephroureterectomy, rt. cutaneous ureterostomy, 1974 5 Moderate hydroureteronephrosis (rt. greater than It.), progressive since 1971 0.83 Stable, blood pressure 130/100 Stable 4 0 Lost to followup 0 Recently diagnosed 0.67 Ilea! conduit, 1976 2 Bilat. cutaneous ureterostomy, 1974
548
CRANE AND SMITH Age not
listed Total Black
rn
D Not Listed D
White
35 17 9
10
61
19
20-29
2 3 2 7
30-39
37 20 II 68
40-49
50-59
60-69 70-79
AGE AT INITIAL DIAGNOSIS
FIG. 1 Epidemiologic data
around the bladder. Exploratory laparotomy for continued pain in December 1975 showed normal fat around the bladder. A 6-month followup pyelogram has shown no change in the pelvic lucency or in the upper tracts. Case 9. G. L., VAH 223205933, a 47-year-old black man, was seen initially in January 1972 for hypertension (190/130). An IVP showed normal upper tracts with a grossly elevated bladder. Cystoscopy showed bullous edema. Laparotomy and biopsy returned normal fat. The patient has remained stable through November 1975 with acellular urine, no upper tract changes and hypertension controlled medically. Case 10. C. W., MCV 3006697, a 30-year-old black man, was seen initially in December 1972 for left flank pain and total gross painless hematuria. He had labile hypertension ranging as high as 180/110. An IVP, cystogram and cystoscopy were compatible with pelvic lipomatosis. This patient has been lost to followup. Case 11. L. B., MCV 5067460, a 53-year-old black man on hydrochlorothiazide for hypertension, was seen recently for a 3 by 4 cm. left upper quadrant abdominal wall lipoma. An IVP and barium enema are consistent with pelvic lipomatosis. Case 12. W. M., VAH 427301485, a 52-year-old black man, was seen initially in November 1975 for renal failure. He had a blood urea nitrogen (BUN) of 120, a creatinine of 6 and blood pressure of 158/92. An IVP showed bilateral severe hydroureteronephrosis. A barium enema and a cystogram were compatible with pelvic lipomatosis. Cystoscopy showed an elevated bladder neck and a biopsy indicated cystitis glandularis. Heal conduit urinary diversion was performed in June 1976. Biopsied perivesical fat was benign. Blood pressure is now 120/82 on hydrochlorothiazide and BUN has decreased to 85 as of August 1976. Case 13. A. L., VAR 719123964,* a 58-year-old black man, was seen initially in October 1974 with a blood pressure of 148/ 98. An IVP showed bilateral hydronephrosis secondary to pelvic lipomatosis, leading to a diversion by bilateral cutaneous ureterostomy in November 1974. The upper tracts have shown no further deterioration through August 1976 and blood pressure is 130/70 without medication. DISCUSSION
Pelvic lipomatosis has been well reviewed by many investigators. It is an uncommon disease that is being seen with increasing frequency. When race is reported, the incidence is almost double in black subjects (fig. l). 1- 32 Only 3 women have been reported to have pelvic lipomatosis, 3 • 4 all of whom were white. Patients ranged in age at initial diagnosis from 9 to 80 years (fig. 1). No specific cause has been determined. Speculations as to etiology have included chronic infection, obesity, 5 • 6 sclerosing agents' and hormonal alterations. Only 2 of our 13 patients were considered overweight or obese.
* Previously reported by Radinsky and associates. 2
Presenting symptoms are variable. Irritable bladder and vesical outlet obstructive symptoms predominate. Suprapubic pain, mild gastrointestinal complaints and hypertensive evaluation also have led to the diagnosis. Only 3 of our 13 cases were initially normotensive without medication. Physical examination usually is unremarkable except possibly for an elevated prostate on rectal examination. Occasionally, a suprapubic mass may be noted. Residual urine is minimal. Diagnosis usually can be suspected from an IVP when the findings of a pelvic lucency, medial ureteral deviation and an elevated bladder without prostatic indentation occur. A low kilovoltage plain film of the kidneys, ureters and bladder will enhance the pelvic fat lucency. 8 A prominent obturator muscle shadow also may be apparent. 4 A voiding cystourethrogram will show an elongated posterior urethra with anterior displacement and elevation of the bladder. When the cystogram is performed concomitantly with a barium enema·the anterior displacement and lower pelvic mass are seen prominently. The barium enema will show the characteristic sigmoid effacement secondary to the extrinsic mass. While the majority of the reported cases have been explored for pathologic confirmation the triad of elongated bladder neck, sigmoid effacement and medially deviated distal ureters is considered diagnostic. 8 Cystoscopy has shown a high incidence of cystitic changes: chronic inflammation, cystitis glandularis and cystitis cystica (table 2). Cystoscopy often has been difficult because of the elongated bladder neck. In our series bladder biopsies were obtained in 8 patients; 7 of which were positive (table 2). Cystoscopy was reported in 21 cases and 14 had biopsies, all but 1 of which were positive (table 2). 5• 9-!6 A differential diagnosis should include all the various presentations of retroperitoneal fibrosis and masses. Classical retroperitoneal fibrosis has a different location, usually causing medial ureteral deviation above the true pelvic brim. Metastatic carcinoma more commonly presents with a different history and an asymmetric, lobular, extrinsic compression of the ureters or bladder. History again will differentiate retroperitoneal hemorrhage or postoperative adhesions. Retrovesical liposarcoma may present a similar roentgenographic picture.'3" Nussbaum reported a case of carcinoma of the prostate that presented a marked similarity to pelvic lipomatosis. 34 Surgically explored cases reveal the lower pelvic organs to be encased in normal-appearing unencapsulated fat (fig. 2). Variable amounts of fibrous tissue also have been noted, supporting chronic inflammation as a possible etiology. Even though the bladder and sigmoid colon are encased in fat they maintain their distensibility. Bladder capacity has not been compromised and lower gastrointestinal symptoms are surprisingly rare. Particular attention should be paid to the pathologic diagnosis of the fat surrounding the pelvic organs to differentiate hypertrophied adipose tissue from a lipoma. A lipoma, a true neoplasm, will arise from a single focus and expand centrifugally;19 it also will be encapsulated. Adipose cell size but not number commonly increases in adult obesity. 35 Electron miTABLE
Bullous edema Chronic inflammation Cystitis glandularis Cystitis cystica Cystitis follicularis Negative
2. Cystoscopic results Current Series
Literature Review
0 2 4 1 0 1
1 4 4 (1)* 4 (l)t (l)t 1
8
14
* One case was cystitis cystica initially and then cystitis glandularis on repeat biopsy. 14 t One case showed all 3 forms of cystitis. 12
549
PELVIC LIPOMATOSIS
Fm. 2 A, lower pelvic organs. B, bladder surrounded by hypertrophied fat rro~
THE JOURNAL OF UROLOGY
Copyright © 1977 by The Williams & Wilkins Co.
PELVIC LIPOMATOSIS: 5-YEAR FOLLOWUP DAVID B. CRANE*
AND
M. J. V. SMITH
From the Divisions of Urology, Medical College of Virginia, Virginia Commonwealth U niuersity, Richmond, Virginia and Washington University School of Medicine and John Cochran Veterans Administration Hospital, St. Louis, Missouri
ABSTRACT
Followup of 5 cases presented in 1971 and report of 8 additional cases confirm the initial conclusions that pelvic lipomatosis is a benign but potentially progressive process leading to ureteral obstruction. Young black subjects are affected most commonly. More than 40 per cent of the patients have required permanent urinary diversion. Pelvic lipomatosis has been a subject of increasing interest during the last several years, mainly in the urologic and radiologic literature. More than 65 cases have been reported but long-term followup has been sparse. A followup of the 5 cases initially reported by Lucey and Smith 1 and our successive experience are presented (table 1). ORIGINAL CASE REPORT FOLLOWUPS
Case 1. J.C., MCV 3000770, a 30-year-old black man, died of uremia in 1962. This patient had undergone diversion 6 years previously for ureteral obstruction. The disease had been progressive since the initial evaluation in 1950. No new data were available. Case 2. R. W., MCV 4009403, a 25-year-old black man, was seen initially in 1963. The patient was reported erroneously to have died of uremia but, in fact, was seen again in 1975 with medically uncontrollable hypertension (180/130). Small kidneys bilaterally and marked left pyelocaliectasis were noted on excretory urography (IVP). A cystourethrogram revealed a pear-shaped bladder and a renal arteriogram showed end artery disease with severe nephrosclerosis. Creatinine clearance was 25 to 30 cc per minute. An ileal conduit urinary diversion was done in March 1975. A year later the blood pressure was 148/100 and the patient still required methyldopa and hydralazine. There was no further upper tract deterioration on IVP. Case 3. L.B., MCV 5068008, a 35-year-old black man, was diagnosed in 1969 and has continued to do well with only mild dilatation of the ureters. However, blood pressure on the most recent followup in July 1976 was 180/110 on no medications. A followup IVP has shown no change in the upper tracts. Case 4. G. I., MCV 5269859, a 51-year-old black man, was diagnosed initially in 1968 and remained stable through 1976. The initial hypertension of 260/160 has been controlled with thiazides and diet, and is currently 130/110. Case 5. F. T., MCV 5274235, a 35-year-old thin black man, was seen initially in March 1969 with a blood pressure of 230/ 110. He underwent left nephroureterectomy, right ureteroneocystostomy with ureteral intraperitonealization, right nephrostomy and suprapubic cystostomy. The distal ureter never opened and the patient was converted to an ileal conduit several months later. The 7-year followup shows no change in the right upper tract. Blood pressure in June 1976 was 130/84 on no medications.
left kidney and severe right hydroureteronephrosis. A cystogram and barium enema were consistent with pelvic lipomatosis. A left nephroureterectomy and a right cutaneous ureterostomy were done. In February the patient had a right ureteroneocystostomy with distal ureteral tailoring. Perivesical and periureteral biopsy showed normal adult fat, chronic inflammation of the ureteral mucosa, a thickened wall secondary to fibrosis and hypertrophy of the ureteral musculature. He was treated with prolonged tetracycline but the distal ureter failed to open and the patient has remained with the cutaneous ureterostomy. The right kidney had remained stable through May 1976. Case 7. W. C., MCV 5340327, an obese 65-year-old black man, was evaluated in 1971 for traumatic avulsion of the diaphragm. Ancillary pelvic lipomatosis was noted. By September 1975 mild hypertension had developed. An IVP showed a pelvic lucency, progressive hydroureteronephrosis (the right greater than the left) and a pear-shaped bladder. A cystogram and barium enema were consistent with pelvic lipomatosis. Cystoscopy was attempted but was unsuccessful because the bladder was elevated. The patient also was maintained on tetracycline. The upper tracts were unchanged through May 1976 but the patient is now under treatment with sulfamethoxazole and trimethoprim for chronic urinary tract infections. Case 8. L. P., MCV 5093111, a 47-year-old black man, was seen initially in August 1975 with irritable bladder symptoms and pain radiating into the rectum and right leg. The bladder was elevated by a pelvic lucency but there was no evidence of upper tract obstruction. Bladder biopsy showed cystitis glandularis. A pelvic arteriogram showed diffuse vascularity
NEW CASE REPORTS
Case 6. T. B., MCV 5142258, a 30-year-old black man, was seen initially in January 1974 with non-visualization of the Accepted for publication December 30, 1976. Read at annual meeting of Mid-Atlantic Section, American Urological Association, Dorado Beach, Puerto Rico, September 26-0ctober 1, 1976. *Requests for reprints: Veterans Administration Hospital, St. Louis, Missouri 63125. 547
TABLE
Case-Pt. -Age-Race No.
(yrs.)
1-JC-30-B 2-RW-25-B 3-LB-35-B 4-GI-51-B 5-FT-35-B
6-TB-30-B 7-WC-65-B 8-LP-47-B 9-GL-47-B 10-CW-30-B 11-LB-53-B 12-WM-52-B 13-AL-58-B
1. Current status
Followup (yrs.)
Status as of 1976
Original cases 12 Died of uremia, 1962 13 Ileal conduit, 1975 7 Stable, blood pressure 180/110 8 Stable, blood pressure 130/110 (meds) 7 Lt. nephroureterectomy, ilea! conduit, 1969 Subsequent cases 2.5 Lt. nephroureterectomy, rt. cutaneous ureterostomy, 1974 5 Moderate hydroureteronephrosis (rt. greater than It.), progressive since 1971 0.83 Stable, blood pressure 130/100 Stable 4 0 Lost to followup 0 Recently diagnosed 0.67 Ilea! conduit, 1976 2 Bilat. cutaneous ureterostomy, 1974
548
CRANE AND SMITH Age not
listed Total Black
rn
D Not Listed D
White
35 17 9
10
61
19
20-29
2 3 2 7
30-39
37 20 II 68
40-49
50-59
60-69 70-79
AGE AT INITIAL DIAGNOSIS
FIG. 1 Epidemiologic data
around the bladder. Exploratory laparotomy for continued pain in December 1975 showed normal fat around the bladder. A 6-month followup pyelogram has shown no change in the pelvic lucency or in the upper tracts. Case 9. G. L., VAH 223205933, a 47-year-old black man, was seen initially in January 1972 for hypertension (190/130). An IVP showed normal upper tracts with a grossly elevated bladder. Cystoscopy showed bullous edema. Laparotomy and biopsy returned normal fat. The patient has remained stable through November 1975 with acellular urine, no upper tract changes and hypertension controlled medically. Case 10. C. W., MCV 3006697, a 30-year-old black man, was seen initially in December 1972 for left flank pain and total gross painless hematuria. He had labile hypertension ranging as high as 180/110. An IVP, cystogram and cystoscopy were compatible with pelvic lipomatosis. This patient has been lost to followup. Case 11. L. B., MCV 5067460, a 53-year-old black man on hydrochlorothiazide for hypertension, was seen recently for a 3 by 4 cm. left upper quadrant abdominal wall lipoma. An IVP and barium enema are consistent with pelvic lipomatosis. Case 12. W. M., VAH 427301485, a 52-year-old black man, was seen initially in November 1975 for renal failure. He had a blood urea nitrogen (BUN) of 120, a creatinine of 6 and blood pressure of 158/92. An IVP showed bilateral severe hydroureteronephrosis. A barium enema and a cystogram were compatible with pelvic lipomatosis. Cystoscopy showed an elevated bladder neck and a biopsy indicated cystitis glandularis. Heal conduit urinary diversion was performed in June 1976. Biopsied perivesical fat was benign. Blood pressure is now 120/82 on hydrochlorothiazide and BUN has decreased to 85 as of August 1976. Case 13. A. L., VAR 719123964,* a 58-year-old black man, was seen initially in October 1974 with a blood pressure of 148/ 98. An IVP showed bilateral hydronephrosis secondary to pelvic lipomatosis, leading to a diversion by bilateral cutaneous ureterostomy in November 1974. The upper tracts have shown no further deterioration through August 1976 and blood pressure is 130/70 without medication. DISCUSSION
Pelvic lipomatosis has been well reviewed by many investigators. It is an uncommon disease that is being seen with increasing frequency. When race is reported, the incidence is almost double in black subjects (fig. l). 1- 32 Only 3 women have been reported to have pelvic lipomatosis, 3 • 4 all of whom were white. Patients ranged in age at initial diagnosis from 9 to 80 years (fig. 1). No specific cause has been determined. Speculations as to etiology have included chronic infection, obesity, 5 • 6 sclerosing agents' and hormonal alterations. Only 2 of our 13 patients were considered overweight or obese.
* Previously reported by Radinsky and associates. 2
Presenting symptoms are variable. Irritable bladder and vesical outlet obstructive symptoms predominate. Suprapubic pain, mild gastrointestinal complaints and hypertensive evaluation also have led to the diagnosis. Only 3 of our 13 cases were initially normotensive without medication. Physical examination usually is unremarkable except possibly for an elevated prostate on rectal examination. Occasionally, a suprapubic mass may be noted. Residual urine is minimal. Diagnosis usually can be suspected from an IVP when the findings of a pelvic lucency, medial ureteral deviation and an elevated bladder without prostatic indentation occur. A low kilovoltage plain film of the kidneys, ureters and bladder will enhance the pelvic fat lucency. 8 A prominent obturator muscle shadow also may be apparent. 4 A voiding cystourethrogram will show an elongated posterior urethra with anterior displacement and elevation of the bladder. When the cystogram is performed concomitantly with a barium enema·the anterior displacement and lower pelvic mass are seen prominently. The barium enema will show the characteristic sigmoid effacement secondary to the extrinsic mass. While the majority of the reported cases have been explored for pathologic confirmation the triad of elongated bladder neck, sigmoid effacement and medially deviated distal ureters is considered diagnostic. 8 Cystoscopy has shown a high incidence of cystitic changes: chronic inflammation, cystitis glandularis and cystitis cystica (table 2). Cystoscopy often has been difficult because of the elongated bladder neck. In our series bladder biopsies were obtained in 8 patients; 7 of which were positive (table 2). Cystoscopy was reported in 21 cases and 14 had biopsies, all but 1 of which were positive (table 2). 5• 9-!6 A differential diagnosis should include all the various presentations of retroperitoneal fibrosis and masses. Classical retroperitoneal fibrosis has a different location, usually causing medial ureteral deviation above the true pelvic brim. Metastatic carcinoma more commonly presents with a different history and an asymmetric, lobular, extrinsic compression of the ureters or bladder. History again will differentiate retroperitoneal hemorrhage or postoperative adhesions. Retrovesical liposarcoma may present a similar roentgenographic picture.'3" Nussbaum reported a case of carcinoma of the prostate that presented a marked similarity to pelvic lipomatosis. 34 Surgically explored cases reveal the lower pelvic organs to be encased in normal-appearing unencapsulated fat (fig. 2). Variable amounts of fibrous tissue also have been noted, supporting chronic inflammation as a possible etiology. Even though the bladder and sigmoid colon are encased in fat they maintain their distensibility. Bladder capacity has not been compromised and lower gastrointestinal symptoms are surprisingly rare. Particular attention should be paid to the pathologic diagnosis of the fat surrounding the pelvic organs to differentiate hypertrophied adipose tissue from a lipoma. A lipoma, a true neoplasm, will arise from a single focus and expand centrifugally;19 it also will be encapsulated. Adipose cell size but not number commonly increases in adult obesity. 35 Electron miTABLE
Bullous edema Chronic inflammation Cystitis glandularis Cystitis cystica Cystitis follicularis Negative
2. Cystoscopic results Current Series
Literature Review
0 2 4 1 0 1
1 4 4 (1)* 4 (l)t (l)t 1
8
14
* One case was cystitis cystica initially and then cystitis glandularis on repeat biopsy. 14 t One case showed all 3 forms of cystitis. 12
549
PELVIC LIPOMATOSIS
Fm. 2 A, lower pelvic organs. B, bladder surrounded by hypertrophied fat rro~
