Scand J Urol Nephrol26:421-423, 1992
GIANT CYSTIC NEPHROMA IN AN ADULT Case Report
Brett Delahunt, Peter J. Hatfield, John N. Nacey and Linda J. Holloway From the Departments of’ Pathology and Surgliry, Wellington School Qf‘Medicine. CJniccrsityof Otago, and the Department of Renal Medicinr, Wdington Hospital. Wellington, Neut Zealand
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/04/15 For personal use only.
(Submitted August 14, 1991. Accepted for publication October 4, 1991)
Cystic nephroma (CN) is an unusual renal lesion that is being reported with increasing frequency. Earlier workers had suggested that CN was either ii hamartoma (2), or of developmental origin and it has been included in Potter’s classification of cystic renal diseases as a variety of polycystic kidney (1 1). More recently it has been suggested that CN is a primary neoplasm being derived from renal blastema and is therefore related to Wilms’ tumor (8). This report outlines a case of CN which appears to be unique for the prolonged clinical course and the size of the tumor at operation. The significance of the presence of simple cortical cysts i n the contralateral kidney is discussed. CASE REPORT In 1976. a 33-year-old Caucasian female. with no family history of renal disease. was investigated in the United Kingdom for abdominal swelling first noticed by her husband. Abdominal X-rays and intravenous urography revealed scattered cysts in both kidneys and a diagnosis of multiple cortical renal cysts was made. During a pregnancy in 1980 the left kidney was noted to increase markedly in size. Fluid was drained from the kidney with little noticeable reduction in renal size. The pregnancy proceeded normally and the patient remained normotensive with normal renal function. She migrated to New Zealand in 1981 and was admitted to hospital with subacute bowel obstruction in 11982. During that admission renal ultrasound and C T scan (Fig. I ) showed apparent extensive multicystic disease of the left kidney. The right kidney contained one large lower pole cyst and two smaller cysts in the upper pole. The patient was followed as an outpatient and a gradual increase in the size of the left kidney was observed. Aspiration of the lower pole o f t h e left kidney was undertaken in 1987 when 1.7 litres of clear fluid was drained with difficulty. An isotope renogram in 1987 showed marked re-
duction in perfusion and significant impairment of function of the left kidney. At that time the patient refused nephrectomy. In March 1989 the patient complained of intermittent abdominal pain and it was noted that the left kidney had increased further in size although there was no impairment in overall renal function with a serum creatinine of 0.10 mmolll. She was admitted in December for elective left nephrectomy. The left kidney measured 3 4 ~ 2 2 x 2 4cm and weighed 7 950 g. The majority of the kidney had been replaced by a large encapsulated multicystic mass (Fig. 2). The cysts contained pale yellow serous fluid. No communication between adjacent cysts was identified and no solid areas were seen. Apparently normal renal tissue measuring up to 0.4 cm in thickness was present over part of the investing capsule. Microscopic examination of tumor showed the cyst walls to consist of cellular fibrous tissue lined by cuboidal epithelium (Fig. 3). No mature renal tubules or primitive nephroblastic elements were identified. Apart from focal pressure atrophy, the remnant renal tissue appeared normal. The left renal tumor was diagnosed as a cystic nephroma and the discrete cysts in the right kidney were identified as simple cortical cysts. Postoperatively the patient made an uneventful recovery and one year later retains normal renal function.
DISCUSSION There is increasing evidence to suggest that CN is a benign primary renal neoplasm (8). The tumor has a biphasic age distribution being most frequently found in childhood and middle age (10). It has been postulated that childhood CN is a differentiated form of Wilms’ tumor (7, 8). Adult CN differs from its childhood counterpart by having a more cellular stroma (9). There is also an absence of primitive nephroblastic elements within the septae as seen in that form of infantile CN that has been recently S c a d J Urol NephrolZ6
422
B. Delahunt et al.
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/04/15 For personal use only.
i
6
Fig. 1. C T scan showing transverse section at L2. A large multilocular cystic mass has displaced the normal renal parenchyma on the left. There is a small cyst within the mid portion of the right kidney which has been displaced and rotated by the left-sided mass.
reclassified as cystic partially differentiated nephroblastoma (8). In general CN is treated by simple nephrectomy and to date recurrence or metastases have not been reported (5). The benign nature of this tumor is further illustrated by the present case which appears to have the longest preoperative course on record. Aoyagi reported a patient with CN who was followed for nine years before nephrectomy was undertaken, during which time the patient remained asymptomatic (1). In
Fig. 2. Transected left kidney containing numerous locules of variable size. No necrosis or haemorrhage is seen. Scand J Urol Nephrol26
.
-
Fig. 3. Photomicrograph of locule wall showing cuboidal lining epithelium. Haematoxylin and eosin stain. Magnification ~ 2 0 0 .
our case progressive tumor enlargement was associated with symptoms necessitating nephrectomy. Adult CN frequently presents as an abdominal mass and may have associated hypertension or hematuria (4). Presentation during pregnancy has been previously described in two patients (13, 14). In one of these, as in our case, the tumor showed accelerated growth as the pregnancy progressed suggesting either a hormonal or hemodynamic influence on tumor cell proliferation. The occurrence of multiple simple renal cysts in the contralateral kidney of a patient with CN is unusual. In his original diagnostic criteria Powell stated that a CN should be multilocular, solitary and unilateral, the cysts should not communicate with the renal pelvis and the loculi should not communicate with one another, the loculi should have a definite epithelial lining, there should be no renal elements within the cyst wall and any residual renal tissue should be normal ( 1 3). These criteria were expanded by Aterman who proposed that the remaining kidney should be normal (3), although more recently it has been suggested that bilateral or multiple tumors should not be excluded if the other features were consistent with a diagnosis of CN (8). Utilizing the criteria of Aterman, the presence of simple cortical cysts in the contralateral kidney should preclude a diagnosis of CN in our case. The co-existence of sim-
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/04/15 For personal use only.
Cystic nephroma ple cysts and CN has been previously reported in the case of a 27-month-old female who had a typical CN of the right kidney and a solitary simple cyst on the contralateral kidney ( 6 ) .Simple cysts are a common finding in adult kidneys being present in some series in up to 50% of patients over the age of 50 years (12). The incidence of simple cysts in the population would therefore suggest that their coincidental occurrence with CN is likely and that their presence should not alone be an excluding factor in the diagnosis of CN. REFERENCES
6. 7.
8.
9.
10.
1 . Aoyagi T, Kakudo K, Satoh S, Hata J, Shiramizu
2. 3. 4.
5.
M, Tamaoki N. Multilocular cystic nephroma in an adult: immunohistochemical study. J Urol 1987; 138: 397-399. Arey JB. Cystic lesions of the kidney in infants and children. J Pediatr 1959; 54: 429-445. Aterman K, Boustani P, Gillis DA. Solitary multilocular cyst of the kidney. J Pediatr Surg 1973: 8: 505-S 16. Baldauf MC, Schulz DM. Multilocular cyst of the kidney. Am J Clin Pathol 1976; 65: 93-102. Bennington JL, Beckwith JB. Tumors of the kidney, renal pelvis and ureter. Armed Forces Institute of Pathology Atlas of Tumor Pathology Sec-
1 1.
12. 13. 14.
423
ond Series, Fascicle 12. Washington DC: AFIP, 1975. Gonzalez-Crussi F, Kidd JM, Hernandez RJ. Cystic nephroma: morphologic spectrum and implications. Urology 1982; 20: 88-93. Joshi VV. Cystic partially differentiated nephroblastoma: an entity in the spectrum of infantile renal neoplasia. Perspect Pediatr Pathol 1979; 5: 2 17-235. Joshi VV, Beckwith JB. Multilocular cyst of the kidney (cystic nephroma) and cystic, partially differentiated nephroblastoma. Cancer 1989; 64: 466-479. Mostofi FK, Sesterhenn IA, Sobin LH. Histological typing of kidney tumours. International Histological Classification of Tumours, No. 25. Geneva: World Health Organization, 1981. Nairn ER, Tudway DC, O’Brien JM, Newman J. Multilocular cystic nephroma. Br J Urol 1990; 65: 543-544. Osathanondh V, Potter EL. Pathogenesis of polycystic kidneys. Type 2 due to inhibition of ampullary activity. Arch Pathol 1964; 77: 474-484. Porter KA. The kidneys. In: Symmers WStC, ed. Systemic pathology. 2nd ed. Edinburgh: Churchill-Livingstone, 1978: 1376-1 503. Powell T, Shackman R, Johnson HD. Multilocular cysts of the kidney. Br J Urol 1951; 23: 93-1 02. Wynn-Williams D, Morgan AD. Lymphangioma of the kidney. Br J Surg 1949; 37: 346-349.
Scand J Urol Nephrol26
GIANT CYSTIC NEPHROMA IN AN ADULT Case Report
Brett Delahunt, Peter J. Hatfield, John N. Nacey and Linda J. Holloway From the Departments of’ Pathology and Surgliry, Wellington School Qf‘Medicine. CJniccrsityof Otago, and the Department of Renal Medicinr, Wdington Hospital. Wellington, Neut Zealand
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/04/15 For personal use only.
(Submitted August 14, 1991. Accepted for publication October 4, 1991)
Cystic nephroma (CN) is an unusual renal lesion that is being reported with increasing frequency. Earlier workers had suggested that CN was either ii hamartoma (2), or of developmental origin and it has been included in Potter’s classification of cystic renal diseases as a variety of polycystic kidney (1 1). More recently it has been suggested that CN is a primary neoplasm being derived from renal blastema and is therefore related to Wilms’ tumor (8). This report outlines a case of CN which appears to be unique for the prolonged clinical course and the size of the tumor at operation. The significance of the presence of simple cortical cysts i n the contralateral kidney is discussed. CASE REPORT In 1976. a 33-year-old Caucasian female. with no family history of renal disease. was investigated in the United Kingdom for abdominal swelling first noticed by her husband. Abdominal X-rays and intravenous urography revealed scattered cysts in both kidneys and a diagnosis of multiple cortical renal cysts was made. During a pregnancy in 1980 the left kidney was noted to increase markedly in size. Fluid was drained from the kidney with little noticeable reduction in renal size. The pregnancy proceeded normally and the patient remained normotensive with normal renal function. She migrated to New Zealand in 1981 and was admitted to hospital with subacute bowel obstruction in 11982. During that admission renal ultrasound and C T scan (Fig. I ) showed apparent extensive multicystic disease of the left kidney. The right kidney contained one large lower pole cyst and two smaller cysts in the upper pole. The patient was followed as an outpatient and a gradual increase in the size of the left kidney was observed. Aspiration of the lower pole o f t h e left kidney was undertaken in 1987 when 1.7 litres of clear fluid was drained with difficulty. An isotope renogram in 1987 showed marked re-
duction in perfusion and significant impairment of function of the left kidney. At that time the patient refused nephrectomy. In March 1989 the patient complained of intermittent abdominal pain and it was noted that the left kidney had increased further in size although there was no impairment in overall renal function with a serum creatinine of 0.10 mmolll. She was admitted in December for elective left nephrectomy. The left kidney measured 3 4 ~ 2 2 x 2 4cm and weighed 7 950 g. The majority of the kidney had been replaced by a large encapsulated multicystic mass (Fig. 2). The cysts contained pale yellow serous fluid. No communication between adjacent cysts was identified and no solid areas were seen. Apparently normal renal tissue measuring up to 0.4 cm in thickness was present over part of the investing capsule. Microscopic examination of tumor showed the cyst walls to consist of cellular fibrous tissue lined by cuboidal epithelium (Fig. 3). No mature renal tubules or primitive nephroblastic elements were identified. Apart from focal pressure atrophy, the remnant renal tissue appeared normal. The left renal tumor was diagnosed as a cystic nephroma and the discrete cysts in the right kidney were identified as simple cortical cysts. Postoperatively the patient made an uneventful recovery and one year later retains normal renal function.
DISCUSSION There is increasing evidence to suggest that CN is a benign primary renal neoplasm (8). The tumor has a biphasic age distribution being most frequently found in childhood and middle age (10). It has been postulated that childhood CN is a differentiated form of Wilms’ tumor (7, 8). Adult CN differs from its childhood counterpart by having a more cellular stroma (9). There is also an absence of primitive nephroblastic elements within the septae as seen in that form of infantile CN that has been recently S c a d J Urol NephrolZ6
422
B. Delahunt et al.
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/04/15 For personal use only.
i
6
Fig. 1. C T scan showing transverse section at L2. A large multilocular cystic mass has displaced the normal renal parenchyma on the left. There is a small cyst within the mid portion of the right kidney which has been displaced and rotated by the left-sided mass.
reclassified as cystic partially differentiated nephroblastoma (8). In general CN is treated by simple nephrectomy and to date recurrence or metastases have not been reported (5). The benign nature of this tumor is further illustrated by the present case which appears to have the longest preoperative course on record. Aoyagi reported a patient with CN who was followed for nine years before nephrectomy was undertaken, during which time the patient remained asymptomatic (1). In
Fig. 2. Transected left kidney containing numerous locules of variable size. No necrosis or haemorrhage is seen. Scand J Urol Nephrol26
.
-
Fig. 3. Photomicrograph of locule wall showing cuboidal lining epithelium. Haematoxylin and eosin stain. Magnification ~ 2 0 0 .
our case progressive tumor enlargement was associated with symptoms necessitating nephrectomy. Adult CN frequently presents as an abdominal mass and may have associated hypertension or hematuria (4). Presentation during pregnancy has been previously described in two patients (13, 14). In one of these, as in our case, the tumor showed accelerated growth as the pregnancy progressed suggesting either a hormonal or hemodynamic influence on tumor cell proliferation. The occurrence of multiple simple renal cysts in the contralateral kidney of a patient with CN is unusual. In his original diagnostic criteria Powell stated that a CN should be multilocular, solitary and unilateral, the cysts should not communicate with the renal pelvis and the loculi should not communicate with one another, the loculi should have a definite epithelial lining, there should be no renal elements within the cyst wall and any residual renal tissue should be normal ( 1 3). These criteria were expanded by Aterman who proposed that the remaining kidney should be normal (3), although more recently it has been suggested that bilateral or multiple tumors should not be excluded if the other features were consistent with a diagnosis of CN (8). Utilizing the criteria of Aterman, the presence of simple cortical cysts in the contralateral kidney should preclude a diagnosis of CN in our case. The co-existence of sim-
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Sydney on 01/04/15 For personal use only.
Cystic nephroma ple cysts and CN has been previously reported in the case of a 27-month-old female who had a typical CN of the right kidney and a solitary simple cyst on the contralateral kidney ( 6 ) .Simple cysts are a common finding in adult kidneys being present in some series in up to 50% of patients over the age of 50 years (12). The incidence of simple cysts in the population would therefore suggest that their coincidental occurrence with CN is likely and that their presence should not alone be an excluding factor in the diagnosis of CN. REFERENCES
6. 7.
8.
9.
10.
1 . Aoyagi T, Kakudo K, Satoh S, Hata J, Shiramizu
2. 3. 4.
5.
M, Tamaoki N. Multilocular cystic nephroma in an adult: immunohistochemical study. J Urol 1987; 138: 397-399. Arey JB. Cystic lesions of the kidney in infants and children. J Pediatr 1959; 54: 429-445. Aterman K, Boustani P, Gillis DA. Solitary multilocular cyst of the kidney. J Pediatr Surg 1973: 8: 505-S 16. Baldauf MC, Schulz DM. Multilocular cyst of the kidney. Am J Clin Pathol 1976; 65: 93-102. Bennington JL, Beckwith JB. Tumors of the kidney, renal pelvis and ureter. Armed Forces Institute of Pathology Atlas of Tumor Pathology Sec-
1 1.
12. 13. 14.
423
ond Series, Fascicle 12. Washington DC: AFIP, 1975. Gonzalez-Crussi F, Kidd JM, Hernandez RJ. Cystic nephroma: morphologic spectrum and implications. Urology 1982; 20: 88-93. Joshi VV. Cystic partially differentiated nephroblastoma: an entity in the spectrum of infantile renal neoplasia. Perspect Pediatr Pathol 1979; 5: 2 17-235. Joshi VV, Beckwith JB. Multilocular cyst of the kidney (cystic nephroma) and cystic, partially differentiated nephroblastoma. Cancer 1989; 64: 466-479. Mostofi FK, Sesterhenn IA, Sobin LH. Histological typing of kidney tumours. International Histological Classification of Tumours, No. 25. Geneva: World Health Organization, 1981. Nairn ER, Tudway DC, O’Brien JM, Newman J. Multilocular cystic nephroma. Br J Urol 1990; 65: 543-544. Osathanondh V, Potter EL. Pathogenesis of polycystic kidneys. Type 2 due to inhibition of ampullary activity. Arch Pathol 1964; 77: 474-484. Porter KA. The kidneys. In: Symmers WStC, ed. Systemic pathology. 2nd ed. Edinburgh: Churchill-Livingstone, 1978: 1376-1 503. Powell T, Shackman R, Johnson HD. Multilocular cysts of the kidney. Br J Urol 1951; 23: 93-1 02. Wynn-Williams D, Morgan AD. Lymphangioma of the kidney. Br J Surg 1949; 37: 346-349.
Scand J Urol Nephrol26
