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Renal cholesteatoma
RENAL CHOLESTEATOMA RENE P MOLINA,
M.D.
DAVID A. DULABON, RUSSELL
B. ROTH,
M.D. M.D.
From the Saint Vincent Health Center, Department Urology, Erie, Pennsylvania
of
ABSTRACT-Renal cholesteatoma is being reported more frequently in the literature. A case with a twenty-year documented history is presented, together with observations on differential diagnosis.
Cholesteatoma of the renal pelvis has been described by Harbison’ as a “rare condition in which tumour-like masses of keratin form in the calyceal system as the result of inflammation and squamous metaplasia.” As of 1977, only 28 cases had been reported in the world literature, but this number has increased markedly, so that in a 1982 review article of the subject Hertle and Androulakaki@ brought the total to 80 cases. These authors, in coping with the fact that the disease has been designated by some as leukoplakia and by others as cholesteatoma, have suggested the inclusive term “keratinizing desquamative squamous metaplasia.” We will not review here the accumulated data on this condition except to stress that it may occur in men, women, and children, has rather rarely been diagnosed preoperatively, and has apparently never been found to be associated with frank malignant changes. The case which we report herein would appear to be of special interest because of the documented changes over a twenty-year period, and because of the contribution to diagnosis made by computerized axial tomography (CAT) scanning, together with its limitations. Case Report First admission This forty-seven-year-old male farmer was hospitalized on April 1, 1963, for investigation
152
of episodic right flank pain and hematuria. He had been aware of “blood clots” in the urine on occasion for many years; otherwise his medical history was essentially negative. General physical examination was normal. Laboratory results were as follows: blood pressure 120/74 mm Hg; urine showed 30-40 white blood cells and many red blood cells/HPF; culture sterile; acidfast studies, negative; serology, nonreactive. Radiographic studies including retrograde pyelography showed right hydronephrosis secondary to upper third ureteral narrowing. Whitish material with some crystalline appearance was lavaged from the pelvis, and cytologic examination reported metaplastic cellular atypia, but no evidence of malignant change. The patient was subsequently examined on April 15 and June 19, 1963. He reported complete relief of symptoms. Recheck urograms showed no change in the degree of hydronephrosis or alteration in the urinary findings. Follow-up was recommended, but the patient did not return for twenty years. Second admission On July 19, 1983, the patient was seen with gross painless hematuria. Urograms showed no visualization of the right kidney and substantial left hydronephrosis due to obstruction in the lower third of the left ureter. Retrograde studies showed an angulated narrowed right ureter in
UROLOGY
/ FEBRUARY
1988
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VOLUME
XXXI, NUMBER 2
FIGURE 1. (A) Retrograde right ureteropyelogram showing “goblet sign”in renal pelvis. (B) Retrograde left ureteropyelogram showing ureteral stenosis at pelvic brim. (C) Follow-up excretory urogram showing satisfactory return of left kidney and ureter to normal.
FIGURE 2. CAT scan interpreted as showing extreme right hydronephrosis and moderate left hydronephrosis.
FIGURE 3. Right kidney keratinous material.
the upper third and a “goblet sign” at the right ureteropelvic junction suggesting tissue filling of the right renal pelvis (Fig. 1A). On the left there was an elongated lower ureteral stricture (Fig. 1B). Blood urea nitrogen was 25 mg/lOO mL and creatinine 1.9 mg/lOO mL. On July 21, 1983, a left percutaneous nephrostomy tube was introduced. A CAT scan
was reported as showing moderate left hydronephrosis and a completely obstructed hydronephrotic right kidney with a thin shell of cortex (Fig. 2). The patient was discharged on nephrostomy drainage and was readmitted on August 19, 1983. His creatinine had decreased to 1.5 mg/ 100 mL and the urine was clear. A right
UROLOGY
/ FEBRUARY
1988
I
VOLUME
XxX1. NUMBER 2
with
pelvis
filled
with
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nephrectomy was done, and the strictured area of the left ureter was excised followed by endto-end ureteral anastomosis. Recovery was uneventful. The pathologist’s report described the right kidney as consisting of a very thin shell of extensively scarred renal parenchyma filled with a semisolid mass of keratinous material together with extensive pelvic and ureteral squamous metaplasia (Fig. 3). The excised segment of the left ureter simply showed nonspecific fibrosis and scarring. Although our pathologists did not use the term, they agree that we have been dealing with an advanced example of renal cholesteatoma. Even retrospectively our radiologists claim inability on the CAT scan to distinguish between a fluid-filled hydronephrotic kidney and one filled with desquamated keratin. Our unwillingness to accept a preoperative diagnosis of
154
hydronephrosis hinged on the interpretation of the retrograde ureteropyelogram suggesting that the greatly distended renal pelvis was full of tumor-like material-with papillary carcinoma of the renal pelvis or cholesteatoma being the principal choices. The twenty-year history and the benign metaplastic cytology report favored cholesteatoma. Follow-up urograms show a strikingly normal left kidney (Fig. 1C). Urinalysis is normal, and the patient is asymptomatic. Erie, Pennsylvania 16502-2772 (DR. ROTH) References 1. Harbison P: Cholesteatoma of the kidney, Br J Urol40: 485 (1968). 2. Hertle L, and Androulakakis P: Keratinizing desquamative squamous metaplasia of the upper urinary tract: leukoplakiacholesteatoma, J Urol 127: 631 (1982).
UROLOGY
I
FEBRUARY
1988
!
VOLUME
XXXI, NUMBER
2
M.D.
DAVID A. DULABON, RUSSELL
B. ROTH,
M.D. M.D.
From the Saint Vincent Health Center, Department Urology, Erie, Pennsylvania
of
ABSTRACT-Renal cholesteatoma is being reported more frequently in the literature. A case with a twenty-year documented history is presented, together with observations on differential diagnosis.
Cholesteatoma of the renal pelvis has been described by Harbison’ as a “rare condition in which tumour-like masses of keratin form in the calyceal system as the result of inflammation and squamous metaplasia.” As of 1977, only 28 cases had been reported in the world literature, but this number has increased markedly, so that in a 1982 review article of the subject Hertle and Androulakaki@ brought the total to 80 cases. These authors, in coping with the fact that the disease has been designated by some as leukoplakia and by others as cholesteatoma, have suggested the inclusive term “keratinizing desquamative squamous metaplasia.” We will not review here the accumulated data on this condition except to stress that it may occur in men, women, and children, has rather rarely been diagnosed preoperatively, and has apparently never been found to be associated with frank malignant changes. The case which we report herein would appear to be of special interest because of the documented changes over a twenty-year period, and because of the contribution to diagnosis made by computerized axial tomography (CAT) scanning, together with its limitations. Case Report First admission This forty-seven-year-old male farmer was hospitalized on April 1, 1963, for investigation
152
of episodic right flank pain and hematuria. He had been aware of “blood clots” in the urine on occasion for many years; otherwise his medical history was essentially negative. General physical examination was normal. Laboratory results were as follows: blood pressure 120/74 mm Hg; urine showed 30-40 white blood cells and many red blood cells/HPF; culture sterile; acidfast studies, negative; serology, nonreactive. Radiographic studies including retrograde pyelography showed right hydronephrosis secondary to upper third ureteral narrowing. Whitish material with some crystalline appearance was lavaged from the pelvis, and cytologic examination reported metaplastic cellular atypia, but no evidence of malignant change. The patient was subsequently examined on April 15 and June 19, 1963. He reported complete relief of symptoms. Recheck urograms showed no change in the degree of hydronephrosis or alteration in the urinary findings. Follow-up was recommended, but the patient did not return for twenty years. Second admission On July 19, 1983, the patient was seen with gross painless hematuria. Urograms showed no visualization of the right kidney and substantial left hydronephrosis due to obstruction in the lower third of the left ureter. Retrograde studies showed an angulated narrowed right ureter in
UROLOGY
/ FEBRUARY
1988
!
VOLUME
XXXI, NUMBER 2
FIGURE 1. (A) Retrograde right ureteropyelogram showing “goblet sign”in renal pelvis. (B) Retrograde left ureteropyelogram showing ureteral stenosis at pelvic brim. (C) Follow-up excretory urogram showing satisfactory return of left kidney and ureter to normal.
FIGURE 2. CAT scan interpreted as showing extreme right hydronephrosis and moderate left hydronephrosis.
FIGURE 3. Right kidney keratinous material.
the upper third and a “goblet sign” at the right ureteropelvic junction suggesting tissue filling of the right renal pelvis (Fig. 1A). On the left there was an elongated lower ureteral stricture (Fig. 1B). Blood urea nitrogen was 25 mg/lOO mL and creatinine 1.9 mg/lOO mL. On July 21, 1983, a left percutaneous nephrostomy tube was introduced. A CAT scan
was reported as showing moderate left hydronephrosis and a completely obstructed hydronephrotic right kidney with a thin shell of cortex (Fig. 2). The patient was discharged on nephrostomy drainage and was readmitted on August 19, 1983. His creatinine had decreased to 1.5 mg/ 100 mL and the urine was clear. A right
UROLOGY
/ FEBRUARY
1988
I
VOLUME
XxX1. NUMBER 2
with
pelvis
filled
with
153
nephrectomy was done, and the strictured area of the left ureter was excised followed by endto-end ureteral anastomosis. Recovery was uneventful. The pathologist’s report described the right kidney as consisting of a very thin shell of extensively scarred renal parenchyma filled with a semisolid mass of keratinous material together with extensive pelvic and ureteral squamous metaplasia (Fig. 3). The excised segment of the left ureter simply showed nonspecific fibrosis and scarring. Although our pathologists did not use the term, they agree that we have been dealing with an advanced example of renal cholesteatoma. Even retrospectively our radiologists claim inability on the CAT scan to distinguish between a fluid-filled hydronephrotic kidney and one filled with desquamated keratin. Our unwillingness to accept a preoperative diagnosis of
154
hydronephrosis hinged on the interpretation of the retrograde ureteropyelogram suggesting that the greatly distended renal pelvis was full of tumor-like material-with papillary carcinoma of the renal pelvis or cholesteatoma being the principal choices. The twenty-year history and the benign metaplastic cytology report favored cholesteatoma. Follow-up urograms show a strikingly normal left kidney (Fig. 1C). Urinalysis is normal, and the patient is asymptomatic. Erie, Pennsylvania 16502-2772 (DR. ROTH) References 1. Harbison P: Cholesteatoma of the kidney, Br J Urol40: 485 (1968). 2. Hertle L, and Androulakakis P: Keratinizing desquamative squamous metaplasia of the upper urinary tract: leukoplakiacholesteatoma, J Urol 127: 631 (1982).
UROLOGY
I
FEBRUARY
1988
!
VOLUME
XXXI, NUMBER
2