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Renal Carcinoma and Polycystic Disease
Vol. 107, April Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1972 hy The Williams & Wilkins Co.
RENAL CARCINOMA AND POLYCYSTIC DISEASE WARREN L. McFARLAND, SIDNEY WALLACE
AND
DOUGLAS E. JOHNSON
From the Departments of Radiology and Surgery, Section of Urology, The University of Texas M. D. Anderson Hospital and Tumor Institute, Houston, Texas
The development of renal carcinoma in association with polycystic disease of the kidney is a rare finding which presents special diagnostic and therapeutic considerations. Only 15 cases have been reported in the literature1- 15 and in most instances the malignancy was not suspected until surgical exploration or necropsy. In the case reported herein the diagnosis was established preoperatively by selective renal angiography. CASE REPORT
A 45-year-old white woman with known polycystic renal disease was referred to this hospital for evaluation of a pathologic fracture of the right midclavicle. A biopsy of the destructive bone lesion had been interpreted as metastatic adenocarcinoma with no histologic indication of a primary site of origin. Complete radiographic surveys, including excretory urography (IVP), failed to demonstrate either a primary malignancy or other secondary Accepted for publication May 7, 1971. 1 Walters, W. and Braasch, W. F.: Surgical aspects of polycystic kidneys. Surg., Gynec. & Obst., 58: 649, 1934. 2 Tomoff, W.: Hypernephroma dex. mit policystischer Degeneration. Ztschr. f. Urol., 31: 67, 1937. 3 Melicow, M. M. and Gile, H. H.: An hypernephroma in a polycystic kidney: review of literature and report of a case. J. Urol., 43: 767, 1940. 4 Hayward, W. G.: Hypernephroma in a polycystic kidney. J. Urol., 56: 190, 1946. 5 Lewis, E. L. and Kimbrough, J.C.: Bilateral hypernephroma associated with polycystic disease. Urol. & Cutan. Rev., 56: 79, 1952. 6 Borski, A. A. and Kimbrough, J. C.: Bilateral carcinoma in polycystic renal disease-an unique case. J. Urol., 71: 677, 1954. 7 Howard, R. M. and Young, J. D., Jr.: Two malignant tumors in a polycystic kidney. J. Urol., 102: 162, 1969. 8 Bobbitt, R. M.: Secondary pathological changes in polycystic kidney disease. J. Urol., 50: 131, 1943. 9 Clemmesen, J.: Familiaen t malign t hypernephrom i en slaegt med hereditaer cystenyre. Nord. Med., 14: 1472, 1942. 10 Brannan, W., Miller, W. and Crisler, M.: Coexistence of renal neoplasms and renal cysts. Southern Med. J., 55: 749, 1962. 11 Pnigvert, A.: Polykystose renale et cancer bilateral. J. Urol. Med. Chir., 64: 30, 1958. 12 Tallarigo, A.: Su un rar caso di associazione tra rene policistico e tumor ipernefroide. Riv. anat. pat. e. one., 3: 510, 1950. 13 Van der Vuurst de Vries, J. H. J.: Un cas de tumeur clans un gros rein polykystique. J. d'urol., 46: 130, 1938. 14 Banrys, W. and Morton, W.: Papillary carcinoma in a polycystic kidney. Urol. & Cutan. Rev., 54: 662, 1950. 15 Johnson, W. F.: Carcinoma in a polycystic kidney. J. Urol., 69: 10, 1953.
deposits. External irradiation was delivered to the clavicle to promote healing and reduce local pain before the patient was referred for additional evaluation. Significant physical findings included blood pressure of 140/80 mm. Hg. There was an area of increased pigmentation of the skin overlying the previously irradiated right clavicular metastasis. An irregular non-tender mass, presumed to be the kidney, was found in the left flank and upper abdomen. The right kidney was not palpable. The blood urea nitrogen and creatinine were normal. The serum creatinine clearance time was slightly depressed at 71 ml. per minute. Urinalysis showed occasional red blood cells. A 131I hippuran renogram was interpreted as abnormal with bilateral reduction in the height of the curves. The kidneys were poorly delineated when studied by renal scintiscan. IVP revealed the kidneys to be enlarged, with the right kidney 20 cm. and the left kidney 22.5 cm. in length. The pelviocaliceal systems were attenuated and distorted and the upper pole of the left kidney was rounded (fig. 1, A). An aortogram and bilateral selective renal arteriograms were performed (fig. 1, B). A large, well-circumscribed mass was demonstrated in the upper pole of the left kidney with an absence of contrast material in the richly vascular tumor. The attenuation and distortion of the intrarenal arteries of the left lower pole and the right kidney together with the multiple well-defined lucencies of the nephrogram were characteristic of polycystic disease. In view of the patient's age and general medical condition and the fact that the single metastatic focus was controlled by radiation therapy, it was believed that her life expectancy with a single fairly well-functioning polycystic kidney would be greater than with 2 kidneys, one of which had uncontrolled renal carcinoma. Therefore, a transabdominal, transperitoneal left nephrectomy was performed. Gross and microscopic examination of the specimen revealed a renal carcinoma in the upper pole of a polycystic kidney (fig. 2). The neoplasm was 9.5 cm. in greatest diameter and exhibited central necrosis. Convalescence was uneventful, and subsequent renal function studies were unchanged in comparison to preoperative studies. The patient has been maintained on medroxyprogesterone acetate, 400 mg. intramuscularly at weekly intervals, and has not demonstrated other evidence of metastatic disease since the operation on February 2, 1970. 530
RENAL CARCINOMA AND POLYCYSTIC DISEASE
/531
FIG. 1. A, both kidneys are enlarged. Attenuation of collecting system on right side is more obvious than on left. Upper pole of left kidney is rounded. B, aortogram shows bilateral changes of polycystic disease and massive tumor in upper pole of left kidney. ·
Fw. 2. t'lurgical specimen. Kidney has been transected with renal carcinoma occupying a portion of upper pole. Polycystie disease is seen throughout. DISCUSSION
The paucity of case reports describing the coexi0tence of a neoplasm and polycystic disease reflects in part the difficulties in detecting neoplasms in a kidney already enlarged and a collecting system already distorted. Asymmetry in size and
shape, a palpable mass and hematuria can be asso ciated with polycystic disease, neoplasm or a combination of the 2. The presence of any of these findings indicates the need for further investigation. Nephrotomography is of value when there is adequate renal function in the polycystic kidneys.
532
MCFARLAND, WALLACE AND JOHNSON
Renal arteriography is the more definitive procedure. Howard and Young first demonstrated a malignant neoplasm in a polycystic kidney by renal arteriography. 7 They described 2 renal carcinomas arising in the same polycystic kidney. The discrepancy in the size of the kidneys in their patient stimulated the arteriographic investigation. The most common neoplasm found in association with polycystic disease is renal carcinoma-15 cases have been reported. Other histologic entities reported in combination with polycystic disease are intracystic papilloma, 1 case;16 fibrosarcoma, 1 case and angiomyosarcoma, 1 case.17 The unpredictable behavior of renal carcinoma prompted surgical removal of the neoplasm in our 16 Wells, C.: Polycystic and "unilateral" polycystic kidney. A review of the literature and two cases, one with intra-cystic papilloma. Brit. J. Urol., 8: 22,
1936.
17 Lowsley, 0. S. and Curtis, M. S.: Surgical aspects of cystic kidney disease. J.A.M.A., 127: 1112, 1945.
case, despite the presence of metastatic disease. Surgical management reduces many of the late local symptoms such as pain, fever, hemorrhage or an enlarging mass. In addition, reduction of the tumor cell population may allow better endocrine or cytotoxic control of the remaining tumor. Previous experience with medroxyprogesterone acetate at this institution dictated its initial use in this case. 18 SUMMARY
The sixteenth case of renal carcinoma occurring in association with polycystic disease of the kidney is presented. The diagnosis was established preoperatively by selective renal angiography. Further investigation using nephrotomography and arteriography is warranted whenever a discrepancy in renal size is demonstrated in a patient with polycystic renal disease. 18 Samuels, M. L., Sullivan, P. and Howe, C. D.: Medroxyprogesterone acetate in the treatment of renal cell carcinoma (hypernephroma). Cancer, 22:
525, 1968.
THE JOURNAL OF UROLOGY
Copyright © 1972 hy The Williams & Wilkins Co.
RENAL CARCINOMA AND POLYCYSTIC DISEASE WARREN L. McFARLAND, SIDNEY WALLACE
AND
DOUGLAS E. JOHNSON
From the Departments of Radiology and Surgery, Section of Urology, The University of Texas M. D. Anderson Hospital and Tumor Institute, Houston, Texas
The development of renal carcinoma in association with polycystic disease of the kidney is a rare finding which presents special diagnostic and therapeutic considerations. Only 15 cases have been reported in the literature1- 15 and in most instances the malignancy was not suspected until surgical exploration or necropsy. In the case reported herein the diagnosis was established preoperatively by selective renal angiography. CASE REPORT
A 45-year-old white woman with known polycystic renal disease was referred to this hospital for evaluation of a pathologic fracture of the right midclavicle. A biopsy of the destructive bone lesion had been interpreted as metastatic adenocarcinoma with no histologic indication of a primary site of origin. Complete radiographic surveys, including excretory urography (IVP), failed to demonstrate either a primary malignancy or other secondary Accepted for publication May 7, 1971. 1 Walters, W. and Braasch, W. F.: Surgical aspects of polycystic kidneys. Surg., Gynec. & Obst., 58: 649, 1934. 2 Tomoff, W.: Hypernephroma dex. mit policystischer Degeneration. Ztschr. f. Urol., 31: 67, 1937. 3 Melicow, M. M. and Gile, H. H.: An hypernephroma in a polycystic kidney: review of literature and report of a case. J. Urol., 43: 767, 1940. 4 Hayward, W. G.: Hypernephroma in a polycystic kidney. J. Urol., 56: 190, 1946. 5 Lewis, E. L. and Kimbrough, J.C.: Bilateral hypernephroma associated with polycystic disease. Urol. & Cutan. Rev., 56: 79, 1952. 6 Borski, A. A. and Kimbrough, J. C.: Bilateral carcinoma in polycystic renal disease-an unique case. J. Urol., 71: 677, 1954. 7 Howard, R. M. and Young, J. D., Jr.: Two malignant tumors in a polycystic kidney. J. Urol., 102: 162, 1969. 8 Bobbitt, R. M.: Secondary pathological changes in polycystic kidney disease. J. Urol., 50: 131, 1943. 9 Clemmesen, J.: Familiaen t malign t hypernephrom i en slaegt med hereditaer cystenyre. Nord. Med., 14: 1472, 1942. 10 Brannan, W., Miller, W. and Crisler, M.: Coexistence of renal neoplasms and renal cysts. Southern Med. J., 55: 749, 1962. 11 Pnigvert, A.: Polykystose renale et cancer bilateral. J. Urol. Med. Chir., 64: 30, 1958. 12 Tallarigo, A.: Su un rar caso di associazione tra rene policistico e tumor ipernefroide. Riv. anat. pat. e. one., 3: 510, 1950. 13 Van der Vuurst de Vries, J. H. J.: Un cas de tumeur clans un gros rein polykystique. J. d'urol., 46: 130, 1938. 14 Banrys, W. and Morton, W.: Papillary carcinoma in a polycystic kidney. Urol. & Cutan. Rev., 54: 662, 1950. 15 Johnson, W. F.: Carcinoma in a polycystic kidney. J. Urol., 69: 10, 1953.
deposits. External irradiation was delivered to the clavicle to promote healing and reduce local pain before the patient was referred for additional evaluation. Significant physical findings included blood pressure of 140/80 mm. Hg. There was an area of increased pigmentation of the skin overlying the previously irradiated right clavicular metastasis. An irregular non-tender mass, presumed to be the kidney, was found in the left flank and upper abdomen. The right kidney was not palpable. The blood urea nitrogen and creatinine were normal. The serum creatinine clearance time was slightly depressed at 71 ml. per minute. Urinalysis showed occasional red blood cells. A 131I hippuran renogram was interpreted as abnormal with bilateral reduction in the height of the curves. The kidneys were poorly delineated when studied by renal scintiscan. IVP revealed the kidneys to be enlarged, with the right kidney 20 cm. and the left kidney 22.5 cm. in length. The pelviocaliceal systems were attenuated and distorted and the upper pole of the left kidney was rounded (fig. 1, A). An aortogram and bilateral selective renal arteriograms were performed (fig. 1, B). A large, well-circumscribed mass was demonstrated in the upper pole of the left kidney with an absence of contrast material in the richly vascular tumor. The attenuation and distortion of the intrarenal arteries of the left lower pole and the right kidney together with the multiple well-defined lucencies of the nephrogram were characteristic of polycystic disease. In view of the patient's age and general medical condition and the fact that the single metastatic focus was controlled by radiation therapy, it was believed that her life expectancy with a single fairly well-functioning polycystic kidney would be greater than with 2 kidneys, one of which had uncontrolled renal carcinoma. Therefore, a transabdominal, transperitoneal left nephrectomy was performed. Gross and microscopic examination of the specimen revealed a renal carcinoma in the upper pole of a polycystic kidney (fig. 2). The neoplasm was 9.5 cm. in greatest diameter and exhibited central necrosis. Convalescence was uneventful, and subsequent renal function studies were unchanged in comparison to preoperative studies. The patient has been maintained on medroxyprogesterone acetate, 400 mg. intramuscularly at weekly intervals, and has not demonstrated other evidence of metastatic disease since the operation on February 2, 1970. 530
RENAL CARCINOMA AND POLYCYSTIC DISEASE
/531
FIG. 1. A, both kidneys are enlarged. Attenuation of collecting system on right side is more obvious than on left. Upper pole of left kidney is rounded. B, aortogram shows bilateral changes of polycystic disease and massive tumor in upper pole of left kidney. ·
Fw. 2. t'lurgical specimen. Kidney has been transected with renal carcinoma occupying a portion of upper pole. Polycystie disease is seen throughout. DISCUSSION
The paucity of case reports describing the coexi0tence of a neoplasm and polycystic disease reflects in part the difficulties in detecting neoplasms in a kidney already enlarged and a collecting system already distorted. Asymmetry in size and
shape, a palpable mass and hematuria can be asso ciated with polycystic disease, neoplasm or a combination of the 2. The presence of any of these findings indicates the need for further investigation. Nephrotomography is of value when there is adequate renal function in the polycystic kidneys.
532
MCFARLAND, WALLACE AND JOHNSON
Renal arteriography is the more definitive procedure. Howard and Young first demonstrated a malignant neoplasm in a polycystic kidney by renal arteriography. 7 They described 2 renal carcinomas arising in the same polycystic kidney. The discrepancy in the size of the kidneys in their patient stimulated the arteriographic investigation. The most common neoplasm found in association with polycystic disease is renal carcinoma-15 cases have been reported. Other histologic entities reported in combination with polycystic disease are intracystic papilloma, 1 case;16 fibrosarcoma, 1 case and angiomyosarcoma, 1 case.17 The unpredictable behavior of renal carcinoma prompted surgical removal of the neoplasm in our 16 Wells, C.: Polycystic and "unilateral" polycystic kidney. A review of the literature and two cases, one with intra-cystic papilloma. Brit. J. Urol., 8: 22,
1936.
17 Lowsley, 0. S. and Curtis, M. S.: Surgical aspects of cystic kidney disease. J.A.M.A., 127: 1112, 1945.
case, despite the presence of metastatic disease. Surgical management reduces many of the late local symptoms such as pain, fever, hemorrhage or an enlarging mass. In addition, reduction of the tumor cell population may allow better endocrine or cytotoxic control of the remaining tumor. Previous experience with medroxyprogesterone acetate at this institution dictated its initial use in this case. 18 SUMMARY
The sixteenth case of renal carcinoma occurring in association with polycystic disease of the kidney is presented. The diagnosis was established preoperatively by selective renal angiography. Further investigation using nephrotomography and arteriography is warranted whenever a discrepancy in renal size is demonstrated in a patient with polycystic renal disease. 18 Samuels, M. L., Sullivan, P. and Howe, C. D.: Medroxyprogesterone acetate in the treatment of renal cell carcinoma (hypernephroma). Cancer, 22:
525, 1968.