ADENOCARCINOMA
IN MULTILOCULAR
RENAL CYST RONALD PAUL
W. SADLOWSKI,
SMEY,
JAMES JEROME
M.D.
M.D.
WILLIAMS, TAXY,
M.D. M.D.
From the Departments of Urology and Pathology, McGaw Medical Center of Northwestern University, and Lakeside Veterans Administration Hospital, Chicago, Illinois
with an associated ABSTRACT - The jifih case of a patient with a multilocular cystic nephroma adenocarcinoma is reported. The histology of the tumor and biochemical analysis of the cyst fluid are presented and the literature summarized.
Benign multilocular cystic nephroma is an unusual renal lesion with about 70 cases reported in the literature.’ Only 4 of these cases have been associated with renal cell carcinoma.2-4 This report describes the fifth case of a multirenal cell locular cyst with an associated carcinoma. Case Report A forty-three-year-old male bus driver was hematuria, discovered examination one year
asymptomatic black evaluated for microon routine physical previously. His past
medical history and physical examination were unremarkable, and he was normotensive. Urinalysis showed lo-15 red blood cells and no white blood cells per high-power field. An intravenous pyelogram revealed a mass in the lower pole of the right kidney. At cystoscopy the bladder was normal, and there was clear efflux of urine from both ureteral orifices. Ultrasonography detected a nonhomogeneous mass in the lower pole of the right kidney containing multiple echoes. Arteriography demonstrated an 8-cm. relatively avascular mass in the lower pole of the right kidney (Fig. 1A). In the center of the mass abnormal vessels highlighted by an
FIGURE 1. {A) Right selective arteriogram shows poor vascularization of lower pole lesion. (B) Selective arteriogram after epinephrine injection shows neovascular pattern in lesion.
512
UROLOGY
/ NOVEMBER
1979 / VOLUME
XIV. NUMBER 5
FIGURE 2. (A) Gross specimen shows typical multilocular cystic appearance of lesion with well-defined capsule and normal adjacent parenchyma. (B) Histopathology shows typical clear cell renal carcinoma infiltrating septa and replacing epithelium of one of cyst walls.
epinephrine study were identified which were small and tortuous with some vascular puddling (Fig. 1B). Since a presumptive diagnosis of a malignant renal neoplasm was made, a right radical nephrectomy was performed through an eleventh rib incision. Grossly, the specimen consisted of a multiloculated cystic tumor compressing the surface of the lower pole adjacent renal parenchyma (Fig. 2A). Aspirated cyst fluid had a deep yellow color and was slightly turbid. Laboratory values from the cyst fluid are listed in Table I. Microscopically, the tumor consisted of numerous fibrous septa lined by a single, occasionally stratified, layer of cuboidal cells with clear cytoplasm and a slight nuclear pleomorphism, indicative of renal cell carcinoma (Fig. 2B). The patient is alive without evidence of recurrence or metastases twenty months later. Comment Since the first description of benign multilocular cystic nephroma by Edmunds in 1892,5 about 70 cases have been reported.’ The majority of these cases have been found in children less than four years of age. The adult cases have ranged from eighteen to seventyone years of age, with a peak incidence in the sixth decade. In contrast to the even sex distribution in children, twice as many adult cases have occurred in women as in men. Although absent in the present case, hypertension may be present. Neither bilaterality, familial occurrence, nor an association with cysts of the liver have been described. Iv6 The generally accepted
UROLOGY
/ NOVEMBER1979
/ VOLUMEXIV,
NUMBER5
morphologic features require that this lesion be solitary and unilateral with an epithelium-lined multilocular cyst, no communication between locules of the cyst or between cyst and collecting system, and no differentiated renal tissue in the septa. 7 Although Emmett and Witten state that arteriography is useful in differentiating a malignant tumor from a multilocular cyst, recent reports contradict this statement.+1° The tumorappearing vessels seen in reported cases of benign multilocular cysts have reflected the increased vascularity in the septa between the cysts. Several features of interest found from the analysis of the cyst fluid in our case are shown in Table I. The total protein was markedly elevated, accounting for the turbidity of the cyst fluid. The total lactic dehydrogenase (LDH) was likewise elevated; although contrary to previous reports, the LDH V/I ratio was not elevated. The pathogenesis of multilocular renal cyst remains controversial. From a clinical viewpoint, some anecdotal information is available to support a neoplastic theory. A forty-five-year-
TABLE
I. Cyst fluid analysis
Color Total protein Albumin Cholesterol Alkaline phosphatase LDH LDH V/I ratio Cytology
Deep yellow, turbid 11.5 Gm./lOO ml. 6.4 Gm./lOO ml. 180 mg./lOO ml. 16 P./ml. (n=30-85) 341 P./ml. (n=90-200) 0.346 Class 1 (no malignant cells seen)
513
old woman, with a previously normal intravenous pyelogram, presented five years later with a palpable renal mass which, following removal proved to be a multilocular cyst.2 Another case described is that of a fifty-year-old woman presenting with an asymptomatic abdominal mass who was found to have a 5-cm. benign multilocular cyst in the removed kidney. Her IVP twenty years previously had been normal. I1 The rarity of the lesion, vagaries in terminology, and the common characteristics of multilocular cysts and/or cystic nephroma have complicated attempts to understand its basic nature. The increasing recognition of areas of Wilms tumor in pediatric cases12-15 has suggested that the documentation of malignancy may be a function of histologic sampling. These patients, frequently treated only by nephrectomy, have enjoyed disease-free survival up to seven and one-half years.r4J5 Cases of multilocular cyst and adenocarcinoma are grossly similar to those with areas of Wilms tumor. The 4 previous cases with associated adenocarcinoma include a forty-three-year-old woman with a left lower pole mass which proved to be a multilocular cyst containing cysts lined in several areas by cells typical of renal carcinoma of the clear cell variety.3 Another case was a twenty-year-old man with a multilocular cyst in the upper pole of the right kidney containing focal mural renal cell carcinoma.4 Uson and Melicow2 state that 2 of their 10 cases of multilocular cysts contained clear cell carcinoma, although no further information is supplied. It seems clear that the extensive histologic sampling by which these lesions should be studied will continue to document the basic neoplastic nature of the lesion. The metastatic
514
potential and natural history of adenocarcinoma associated with multilocular cyst remain to be defined. Veterans Administration Hospital 13000 North 30th Street Tampa, Florida 33612 (DR. SADLOWSKI) References 1. Baldauf MC, and Schulz DM: Multilocular cyst of the kidney: report of three cases and review of the literature, Am. J. Clin. Pathol. 65: 93 (1976). 2. Uson AC, and Melicow MM: Multilocular cysts of kidney with intrapelvic hemiation of a “daughter” cyst: report of four cases, J. Ural. 89: 341 (1963). 3. Pfister RC, and Galli SJ: Multicystic renal mass in a 43 year old woman - case records of the Massachusetts General Hospital, N. Engl. J. Med. 292: 415 (1975). 4. Posso M, Safadi D, and Orrin JV: Unilateral polycystic or multicystic kidney associated with focal mural renal cell carcinoma, J. Ural. 109: 559 (1973). 5. Edmunds W: Cystic adenoma of the kidney, Trans. Pathol. 30~. Iond. 43: 89 (1892). 6. Filmer RB, and Taxy JB: In Kelalis P and King L, Eds: Clinical Pediatric Urology, Philadelphia, W. B. Saunders Co., 1976, vol. 2, p. 695. 7. Boggs L, and Kimmelstiel P: Benign multilocular cystic nephroma: report of two cases of so-called multilocular cyst of the kidney, J. Urol. 76: 530 (1956). 8. Rafe CC, and Witten DM: Congenital renal cystic disease, in Emmett JM, and Witten DM, Eds: Clinical Urography, 3rd ed., Philadelphia, W. B. Saunders Co., 1971, vol. 2, p. 1007. 9. Javadpour N, Dellon AL, and Kumpe DA: Multilocular cystic disease in adults: imitator of renal cell carcinoma, Urology 1: 596 (1973). 10. Johnson DE, Ayala AC, Medellin H, and Wilbur J: Multilocular renal cystic disease in children, J. Urol. 169: 101 (1973). 11. Smith L: Multilocular renal cyst with intrapelvic hemiation of a daughter locule, Br. J. Ural. 44: 309 (1972). 12. Christ ML: Polycystic Nephroblastoma, J. Urol. 98: 570 (1968). 13. Uson AC, Del Rosario C, and Melicow MM: Wilms tumor in association with cystic renal disease: report of two cases, ibid. 83: 262 (1960). 14. Joshi W, Banerjee AK, Yadav K, and Pathak IC: Cystic partially differentiated nephroblastoma, Cancer 49: 789 (1977). 15. Gallo GE, and Pencbansky L: Cystic nephroma, ibid. 39: 1322 (1977).
UROLOGY
/
NOVEMBER
1979
/
VOLUME
XIV, NUMBER
5
IN MULTILOCULAR
RENAL CYST RONALD PAUL
W. SADLOWSKI,
SMEY,
JAMES JEROME
M.D.
M.D.
WILLIAMS, TAXY,
M.D. M.D.
From the Departments of Urology and Pathology, McGaw Medical Center of Northwestern University, and Lakeside Veterans Administration Hospital, Chicago, Illinois
with an associated ABSTRACT - The jifih case of a patient with a multilocular cystic nephroma adenocarcinoma is reported. The histology of the tumor and biochemical analysis of the cyst fluid are presented and the literature summarized.
Benign multilocular cystic nephroma is an unusual renal lesion with about 70 cases reported in the literature.’ Only 4 of these cases have been associated with renal cell carcinoma.2-4 This report describes the fifth case of a multirenal cell locular cyst with an associated carcinoma. Case Report A forty-three-year-old male bus driver was hematuria, discovered examination one year
asymptomatic black evaluated for microon routine physical previously. His past
medical history and physical examination were unremarkable, and he was normotensive. Urinalysis showed lo-15 red blood cells and no white blood cells per high-power field. An intravenous pyelogram revealed a mass in the lower pole of the right kidney. At cystoscopy the bladder was normal, and there was clear efflux of urine from both ureteral orifices. Ultrasonography detected a nonhomogeneous mass in the lower pole of the right kidney containing multiple echoes. Arteriography demonstrated an 8-cm. relatively avascular mass in the lower pole of the right kidney (Fig. 1A). In the center of the mass abnormal vessels highlighted by an
FIGURE 1. {A) Right selective arteriogram shows poor vascularization of lower pole lesion. (B) Selective arteriogram after epinephrine injection shows neovascular pattern in lesion.
512
UROLOGY
/ NOVEMBER
1979 / VOLUME
XIV. NUMBER 5
FIGURE 2. (A) Gross specimen shows typical multilocular cystic appearance of lesion with well-defined capsule and normal adjacent parenchyma. (B) Histopathology shows typical clear cell renal carcinoma infiltrating septa and replacing epithelium of one of cyst walls.
epinephrine study were identified which were small and tortuous with some vascular puddling (Fig. 1B). Since a presumptive diagnosis of a malignant renal neoplasm was made, a right radical nephrectomy was performed through an eleventh rib incision. Grossly, the specimen consisted of a multiloculated cystic tumor compressing the surface of the lower pole adjacent renal parenchyma (Fig. 2A). Aspirated cyst fluid had a deep yellow color and was slightly turbid. Laboratory values from the cyst fluid are listed in Table I. Microscopically, the tumor consisted of numerous fibrous septa lined by a single, occasionally stratified, layer of cuboidal cells with clear cytoplasm and a slight nuclear pleomorphism, indicative of renal cell carcinoma (Fig. 2B). The patient is alive without evidence of recurrence or metastases twenty months later. Comment Since the first description of benign multilocular cystic nephroma by Edmunds in 1892,5 about 70 cases have been reported.’ The majority of these cases have been found in children less than four years of age. The adult cases have ranged from eighteen to seventyone years of age, with a peak incidence in the sixth decade. In contrast to the even sex distribution in children, twice as many adult cases have occurred in women as in men. Although absent in the present case, hypertension may be present. Neither bilaterality, familial occurrence, nor an association with cysts of the liver have been described. Iv6 The generally accepted
UROLOGY
/ NOVEMBER1979
/ VOLUMEXIV,
NUMBER5
morphologic features require that this lesion be solitary and unilateral with an epithelium-lined multilocular cyst, no communication between locules of the cyst or between cyst and collecting system, and no differentiated renal tissue in the septa. 7 Although Emmett and Witten state that arteriography is useful in differentiating a malignant tumor from a multilocular cyst, recent reports contradict this statement.+1° The tumorappearing vessels seen in reported cases of benign multilocular cysts have reflected the increased vascularity in the septa between the cysts. Several features of interest found from the analysis of the cyst fluid in our case are shown in Table I. The total protein was markedly elevated, accounting for the turbidity of the cyst fluid. The total lactic dehydrogenase (LDH) was likewise elevated; although contrary to previous reports, the LDH V/I ratio was not elevated. The pathogenesis of multilocular renal cyst remains controversial. From a clinical viewpoint, some anecdotal information is available to support a neoplastic theory. A forty-five-year-
TABLE
I. Cyst fluid analysis
Color Total protein Albumin Cholesterol Alkaline phosphatase LDH LDH V/I ratio Cytology
Deep yellow, turbid 11.5 Gm./lOO ml. 6.4 Gm./lOO ml. 180 mg./lOO ml. 16 P./ml. (n=30-85) 341 P./ml. (n=90-200) 0.346 Class 1 (no malignant cells seen)
513
old woman, with a previously normal intravenous pyelogram, presented five years later with a palpable renal mass which, following removal proved to be a multilocular cyst.2 Another case described is that of a fifty-year-old woman presenting with an asymptomatic abdominal mass who was found to have a 5-cm. benign multilocular cyst in the removed kidney. Her IVP twenty years previously had been normal. I1 The rarity of the lesion, vagaries in terminology, and the common characteristics of multilocular cysts and/or cystic nephroma have complicated attempts to understand its basic nature. The increasing recognition of areas of Wilms tumor in pediatric cases12-15 has suggested that the documentation of malignancy may be a function of histologic sampling. These patients, frequently treated only by nephrectomy, have enjoyed disease-free survival up to seven and one-half years.r4J5 Cases of multilocular cyst and adenocarcinoma are grossly similar to those with areas of Wilms tumor. The 4 previous cases with associated adenocarcinoma include a forty-three-year-old woman with a left lower pole mass which proved to be a multilocular cyst containing cysts lined in several areas by cells typical of renal carcinoma of the clear cell variety.3 Another case was a twenty-year-old man with a multilocular cyst in the upper pole of the right kidney containing focal mural renal cell carcinoma.4 Uson and Melicow2 state that 2 of their 10 cases of multilocular cysts contained clear cell carcinoma, although no further information is supplied. It seems clear that the extensive histologic sampling by which these lesions should be studied will continue to document the basic neoplastic nature of the lesion. The metastatic
514
potential and natural history of adenocarcinoma associated with multilocular cyst remain to be defined. Veterans Administration Hospital 13000 North 30th Street Tampa, Florida 33612 (DR. SADLOWSKI) References 1. Baldauf MC, and Schulz DM: Multilocular cyst of the kidney: report of three cases and review of the literature, Am. J. Clin. Pathol. 65: 93 (1976). 2. Uson AC, and Melicow MM: Multilocular cysts of kidney with intrapelvic hemiation of a “daughter” cyst: report of four cases, J. Ural. 89: 341 (1963). 3. Pfister RC, and Galli SJ: Multicystic renal mass in a 43 year old woman - case records of the Massachusetts General Hospital, N. Engl. J. Med. 292: 415 (1975). 4. Posso M, Safadi D, and Orrin JV: Unilateral polycystic or multicystic kidney associated with focal mural renal cell carcinoma, J. Ural. 109: 559 (1973). 5. Edmunds W: Cystic adenoma of the kidney, Trans. Pathol. 30~. Iond. 43: 89 (1892). 6. Filmer RB, and Taxy JB: In Kelalis P and King L, Eds: Clinical Pediatric Urology, Philadelphia, W. B. Saunders Co., 1976, vol. 2, p. 695. 7. Boggs L, and Kimmelstiel P: Benign multilocular cystic nephroma: report of two cases of so-called multilocular cyst of the kidney, J. Urol. 76: 530 (1956). 8. Rafe CC, and Witten DM: Congenital renal cystic disease, in Emmett JM, and Witten DM, Eds: Clinical Urography, 3rd ed., Philadelphia, W. B. Saunders Co., 1971, vol. 2, p. 1007. 9. Javadpour N, Dellon AL, and Kumpe DA: Multilocular cystic disease in adults: imitator of renal cell carcinoma, Urology 1: 596 (1973). 10. Johnson DE, Ayala AC, Medellin H, and Wilbur J: Multilocular renal cystic disease in children, J. Urol. 169: 101 (1973). 11. Smith L: Multilocular renal cyst with intrapelvic hemiation of a daughter locule, Br. J. Ural. 44: 309 (1972). 12. Christ ML: Polycystic Nephroblastoma, J. Urol. 98: 570 (1968). 13. Uson AC, Del Rosario C, and Melicow MM: Wilms tumor in association with cystic renal disease: report of two cases, ibid. 83: 262 (1960). 14. Joshi W, Banerjee AK, Yadav K, and Pathak IC: Cystic partially differentiated nephroblastoma, Cancer 49: 789 (1977). 15. Gallo GE, and Pencbansky L: Cystic nephroma, ibid. 39: 1322 (1977).
UROLOGY
/
NOVEMBER
1979
/
VOLUME
XIV, NUMBER
5
