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Cholesteatoma of the Renal Pelvis: A Case with Long-Term Followup
0022-534 7/88/1402-0360$02.00/0 Vol. 140, August
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1988 by The Williams & Wilkins Co.
CHOLESTEATOMA OF THE RENAL PELVIS: A CASE WITH LONGTERM FOLLOWUP AARON LUPOVITCH, HENRY DOMZALSKI
AND
RONALD TIPPINS
From the Departments of Patho/,ogy, Surgery and Radio/,ogy, Holy Cross Hospital, Detroit, Michigan
ABSTRACT
We report a case of cholesteatoma of the renal pelvis with a 56-year followup. Quiescence of the disease with remission of symptoms, stabilization of the lesion and absence of further anatomical change was related temporally to treatment with vitamin A. The disease in our patient was idiopathic and no causative or predisposing factors were demonstrated. (J. Urol., 140: 360-361, 1988) years later with an attack of right lower quadrant pain. An IVP showed hydronephrosis and hydroureter. After passage of a compact mass of keratinized squames (fig. 5), similar to that seen in the pyelotomy specimen, a retrograde pyelogram showed a normal ureter and only a minute residue (3 X 5 mm.) on the medial side of the kidney pelvis. The patient was advised to resume taking vitamin A and to return for annual followup.
Keratinizing squamous metaplasia of the renal pelvis is a rare lesion of unknown etiology. 1 The implications of this diagnosis for patient morbidity and mortality are not clearly understood. As a result, recommended therapy has ranged from prompt nephrectomy to periodic followup with minimal concern. Therefore, we report a case of this entity with a well documented course that has now spanned more than 50 years. CASE REPORT
A 70-year-old white man first suffered an attack of right lower quadrant and right flank pain when he was 14 years old (fig. 1). These episodes recurred at 2 to 3-month intervals and they were frequently associated with passage of "material" in the urine followed by relief of pain. When he was 40 years old the patient had been hospitalized elsewhere for surgery, which he declined. During the next 13 years he was seen in our emergency room 7 times for symptoms of renal colic. Relief occurred with narcotic type of medication or the passing of a "stone", "tissue" or "material" per urethram. Qualitative chemical analysis of the passed "stone" on 2 occasions provided negative results for calcium, magnesium, phosphate, ammonium, oxalate and urate ions, and cysteine. When he was 53 years old the patient was seen by a urologist after an episode of renal colic and he was hospitalized. Urinalysis at that time was normal. A retrograde pyelogram showed an irregular, lamellated mass with a corkscrew appearance (fig. 2). Right pyelotomy was performed. When the bulging renal pelvis was opened, a large amount of tissue-like debris in sheets and fragments was extracted directly by forceps and then by irrigating with saline. Microscopically, the resected material consisted of slender strips and sheets of mature, compact, stratified squamous epithelium with keratohyalin granules and prominent keratinization (fig. 3). A retrograde pyelogram 5 months postoperatively showed 3 ridges of tissue over an 8 X 4 mm. area (fig. 4). The patient was started on 50,000 units of vitamin A twice daily, which he took for the next 15 years (age 67 years). During this time 1 additional episode of renal colic occurred when he was 55 years old, which was relieved by passage of tissue-like material. Radiographic examinations were repeated annually, and the ridged area gradually enlarged to 20 X 10 mm. 5 years later and remained this approximate size until the patient was 65 years old. Urinalysis on multiple occasions was normal or demonstrated microscopic hematuria. When the patient was 60 years old a chemistry profile, including serum urea, creatinine, uric acid, calcium, phosphate and alkaline phosphatase, was normal. The patient left the metropolitan area and discontinued taking the vitamin A when he was 68 years old. He returned 2
l
n,.,rn,---so,0000,wl
ER VISIT RENAL COLIC PASSED "STONE"
EVERY 2-3 MONTHS PASSED "MATERIAL"
V,\A URETHRA\
ER VISI:A• NUMERO\OS ~RB:GBT
'/ l ,fl "
RLQ PAUi
15
HOSPITALIZED; REFUSEO SURGERY
l
l
1"11
j
NORMAL URINALYSIS
YELOTOMY
RLQ PAIN
PASSr TISSUE
"1
ER VISIT FOR ER VISIT FOR RENAL COLIC RIGHT FLANK, "PASSED STONE" TESTICULAR PA!N
RIGHT URETERAL ER VISIT COLIC PASSED "STONE" PASSED TISSUE-LIKE KATERIAL!NUR!NE
ER VISIT
NORMAL
UR1NlLYS1S
PASSED TISSUE-LIKE MATERIAL lN URINE
65t
70
PASSED 'TISSUE" RELIEVED RLQ PA!N URINE:
1 100-2DORBC/IIPF
NORMAL URINALYSIS
· Fm. 1. Chronologic record of genitourinary tract disease from time patient was 15 to 70 years old (abscissa). UA, urinalysis. RLQ, right lower quadrant. ER, emergency room.
FIG. 2. IVP demonstrates "corkscrew" defect of cholesteatoma
Accepted for publication November 25, 1987. 360
361
CHOLESTEATOMA OF RENAL PELVIS
FIG. 3. Metaplastic keratinizing squamous epithelium. H & E, reduced from X40.
Fm. 4. IVP demonstrates persisting long-term mucosal defect
DISCUSSION
The disease entity in our patient is sometimes referred to as cholesteatoma of the kidney, leukoplakia of the renal pelvis or keratinizing squamous metaplasia of the renal pelvic mucosa. 1 The last phrase is the most descriptive and characterizes the anatomical changes seen in the renal pelvic epithelium. Multiple radiographic and laboratory studies during 4 decades failed to show evidence of genitourinary tract malformation, infection or calculi. Although others have considered these to be causative or contributory factors, in our patient the lesion
Fm. 5. Compact mass of keratin passed per urethram when patient was 70 years old that is considered typical of urinary "tissue" or "material". H & E, reduced from X400.
was idiopathic with no demonstrable predisposing cause. The course and appropriate therapy for renal pelvic epidermization also are not clearly understood. Some authors regard the aforementioned changes to have malignant potential and, indeed, an infrequent case of squamous cell carcinoma has been associated with epidermal change in the renal pelvic mucosa. However, this occurrence borders on being anecdotal. The course of the disease also is poorly understood, since the evolution of the pathological process was abruptly stopped in the majority of patients by nephrectomy. Notable in our patient was the relatively constant radiographic findings after pyelotomy and limited mucosal resection. The beneficial role of vitamin A therapy in our case is speculative, since the postoperative period free of symptoms may have been only a coincidental parallel to the years of vitamin A therapy. However, we note that the lesion stabilized and remained unchanged for 5 years until the patient discontinued therapy. In other diseases, such as acne and oral leukoplakia, vitamin A analogues have been shown to have significant therapeutic value. 2 REFERENCES 1. Haugen, S. G. and Wasserman, N. F.: Keratinizing desquamative
squamous metaplasia of the upper urinary tract. Urol. Rad., 8: 211, 1986. 2. Hong, W. K., Endicott, J., Itri, L. M., Doos, W., Batsakis, J. G., Bell, R., Fofonoff, S., Byers, R., Atkinson, E. N., Vaughan, C., Toth, B. B., Kramer, A., Dimery, I. W., Skipper, P. and Strong, S.: 13-cis-retinoic acid in the treatment of oral leukoplakia. New Engl. J. Med., 315: 1501, 1986.
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1988 by The Williams & Wilkins Co.
CHOLESTEATOMA OF THE RENAL PELVIS: A CASE WITH LONGTERM FOLLOWUP AARON LUPOVITCH, HENRY DOMZALSKI
AND
RONALD TIPPINS
From the Departments of Patho/,ogy, Surgery and Radio/,ogy, Holy Cross Hospital, Detroit, Michigan
ABSTRACT
We report a case of cholesteatoma of the renal pelvis with a 56-year followup. Quiescence of the disease with remission of symptoms, stabilization of the lesion and absence of further anatomical change was related temporally to treatment with vitamin A. The disease in our patient was idiopathic and no causative or predisposing factors were demonstrated. (J. Urol., 140: 360-361, 1988) years later with an attack of right lower quadrant pain. An IVP showed hydronephrosis and hydroureter. After passage of a compact mass of keratinized squames (fig. 5), similar to that seen in the pyelotomy specimen, a retrograde pyelogram showed a normal ureter and only a minute residue (3 X 5 mm.) on the medial side of the kidney pelvis. The patient was advised to resume taking vitamin A and to return for annual followup.
Keratinizing squamous metaplasia of the renal pelvis is a rare lesion of unknown etiology. 1 The implications of this diagnosis for patient morbidity and mortality are not clearly understood. As a result, recommended therapy has ranged from prompt nephrectomy to periodic followup with minimal concern. Therefore, we report a case of this entity with a well documented course that has now spanned more than 50 years. CASE REPORT
A 70-year-old white man first suffered an attack of right lower quadrant and right flank pain when he was 14 years old (fig. 1). These episodes recurred at 2 to 3-month intervals and they were frequently associated with passage of "material" in the urine followed by relief of pain. When he was 40 years old the patient had been hospitalized elsewhere for surgery, which he declined. During the next 13 years he was seen in our emergency room 7 times for symptoms of renal colic. Relief occurred with narcotic type of medication or the passing of a "stone", "tissue" or "material" per urethram. Qualitative chemical analysis of the passed "stone" on 2 occasions provided negative results for calcium, magnesium, phosphate, ammonium, oxalate and urate ions, and cysteine. When he was 53 years old the patient was seen by a urologist after an episode of renal colic and he was hospitalized. Urinalysis at that time was normal. A retrograde pyelogram showed an irregular, lamellated mass with a corkscrew appearance (fig. 2). Right pyelotomy was performed. When the bulging renal pelvis was opened, a large amount of tissue-like debris in sheets and fragments was extracted directly by forceps and then by irrigating with saline. Microscopically, the resected material consisted of slender strips and sheets of mature, compact, stratified squamous epithelium with keratohyalin granules and prominent keratinization (fig. 3). A retrograde pyelogram 5 months postoperatively showed 3 ridges of tissue over an 8 X 4 mm. area (fig. 4). The patient was started on 50,000 units of vitamin A twice daily, which he took for the next 15 years (age 67 years). During this time 1 additional episode of renal colic occurred when he was 55 years old, which was relieved by passage of tissue-like material. Radiographic examinations were repeated annually, and the ridged area gradually enlarged to 20 X 10 mm. 5 years later and remained this approximate size until the patient was 65 years old. Urinalysis on multiple occasions was normal or demonstrated microscopic hematuria. When the patient was 60 years old a chemistry profile, including serum urea, creatinine, uric acid, calcium, phosphate and alkaline phosphatase, was normal. The patient left the metropolitan area and discontinued taking the vitamin A when he was 68 years old. He returned 2
l
n,.,rn,---so,0000,wl
ER VISIT RENAL COLIC PASSED "STONE"
EVERY 2-3 MONTHS PASSED "MATERIAL"
V,\A URETHRA\
ER VISI:A• NUMERO\OS ~RB:GBT
'/ l ,fl "
RLQ PAUi
15
HOSPITALIZED; REFUSEO SURGERY
l
l
1"11
j
NORMAL URINALYSIS
YELOTOMY
RLQ PAIN
PASSr TISSUE
"1
ER VISIT FOR ER VISIT FOR RENAL COLIC RIGHT FLANK, "PASSED STONE" TESTICULAR PA!N
RIGHT URETERAL ER VISIT COLIC PASSED "STONE" PASSED TISSUE-LIKE KATERIAL!NUR!NE
ER VISIT
NORMAL
UR1NlLYS1S
PASSED TISSUE-LIKE MATERIAL lN URINE
65t
70
PASSED 'TISSUE" RELIEVED RLQ PA!N URINE:
1 100-2DORBC/IIPF
NORMAL URINALYSIS
· Fm. 1. Chronologic record of genitourinary tract disease from time patient was 15 to 70 years old (abscissa). UA, urinalysis. RLQ, right lower quadrant. ER, emergency room.
FIG. 2. IVP demonstrates "corkscrew" defect of cholesteatoma
Accepted for publication November 25, 1987. 360
361
CHOLESTEATOMA OF RENAL PELVIS
FIG. 3. Metaplastic keratinizing squamous epithelium. H & E, reduced from X40.
Fm. 4. IVP demonstrates persisting long-term mucosal defect
DISCUSSION
The disease entity in our patient is sometimes referred to as cholesteatoma of the kidney, leukoplakia of the renal pelvis or keratinizing squamous metaplasia of the renal pelvic mucosa. 1 The last phrase is the most descriptive and characterizes the anatomical changes seen in the renal pelvic epithelium. Multiple radiographic and laboratory studies during 4 decades failed to show evidence of genitourinary tract malformation, infection or calculi. Although others have considered these to be causative or contributory factors, in our patient the lesion
Fm. 5. Compact mass of keratin passed per urethram when patient was 70 years old that is considered typical of urinary "tissue" or "material". H & E, reduced from X400.
was idiopathic with no demonstrable predisposing cause. The course and appropriate therapy for renal pelvic epidermization also are not clearly understood. Some authors regard the aforementioned changes to have malignant potential and, indeed, an infrequent case of squamous cell carcinoma has been associated with epidermal change in the renal pelvic mucosa. However, this occurrence borders on being anecdotal. The course of the disease also is poorly understood, since the evolution of the pathological process was abruptly stopped in the majority of patients by nephrectomy. Notable in our patient was the relatively constant radiographic findings after pyelotomy and limited mucosal resection. The beneficial role of vitamin A therapy in our case is speculative, since the postoperative period free of symptoms may have been only a coincidental parallel to the years of vitamin A therapy. However, we note that the lesion stabilized and remained unchanged for 5 years until the patient discontinued therapy. In other diseases, such as acne and oral leukoplakia, vitamin A analogues have been shown to have significant therapeutic value. 2 REFERENCES 1. Haugen, S. G. and Wasserman, N. F.: Keratinizing desquamative
squamous metaplasia of the upper urinary tract. Urol. Rad., 8: 211, 1986. 2. Hong, W. K., Endicott, J., Itri, L. M., Doos, W., Batsakis, J. G., Bell, R., Fofonoff, S., Byers, R., Atkinson, E. N., Vaughan, C., Toth, B. B., Kramer, A., Dimery, I. W., Skipper, P. and Strong, S.: 13-cis-retinoic acid in the treatment of oral leukoplakia. New Engl. J. Med., 315: 1501, 1986.