- Email: [email protected]
Two Malignant Tumors in a Polycystic Kidney
Vol. 102, Aug. Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1969 by The Williams & Wilkins Co.
TWO MALIGNANT TUMORS IN A POLYCYSTIC KIDNEY RALPH M. HOWARD
AND
J. D. YOUNG, JR.
From the Division of Urology, Department of Surgery, University of Maryland School of 1vledicine, Baltimore, Maryland
men on the left side. The right kidney was thought palpable just below the right costal margin. There were no other positive findings. An excretory urogram (IVP) indicated multiple, smooth filling defects involving both collecting systems (fig. 1). The right kidney measured 16 cm. and the left 23 cm. in length. A large mass could be seen arising from the inferior pole of the left kidney (fig. 2, A). A retrograde femoral arteriogram showed an area of increased. vascularity with tumor staining in the upper and
Carcinoma in a polycystic kidney has been reported on at least 17 occasions. 1- 11 The palpable masses and/or hematuria of polycystic disease might mask a neoplasm. Coincidental discovery by aortography of 2 renal cell tumors in the left polycystic kidney of a young woman was thought of sufficient interest to report. CASE REPORT
A 32-year-old Negress was admitted to the University of Maryland Hospital for evaluation of a left abdominal mass. Although she had been aware of the mass for about 9 months, it had become painful just prior to admission. She had had intermittent gross hematuria for 8 years but denied any other urinary symptoms. One sibling had polycystic kidneys. Physical examination revealed a well-developed, young woman with blood pressure of 130/70 mm. Hg. An irregular, non-tender mass was palpated in the left flank and upper abdoAccepted for publication September 5, 1968. 'Walters, W. and Braasch, W. F.: Surgical aspects of polycystic kidneys. Surg., Gynec. & Obst., 58: 649, 1934. 2 Oppenheimer, G. D.: Polycystic disease of the kidney. Ann. Surg., 100: 1136, 1934. 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 Bobbitt, R. M.: Secondary pathological changes in polycystic kidney disease. J. Urol., 50: 134, 1943. 5 Lowsley, 0. S. and Curtis, M. S.: Surgical aspects of cystic kidney disease. J.A.M.A., 127: 1112, 1945. 6 Hayward, W. G.: Hypernephroma in a polycystic kidney. J. Urol., 56: 190, 1946. 7 Rall, J. E. and Ode!, H. M.: Congenital polycystic disease of kidney: review of literature and data on 207 cases. Amer. J. Med. Sci., 218: 399, 1949. 8 Baurys, W. and Morton, W.: Papillary carcinoma in a polycystic kidney. Urol. & Cutan. Rev., 54: 662, 1950. 9 Lewis, E. L. and Kimbrough, J. C.: Bilateral hypernephroma associated with polycystic disease. Urol. & Cutan. Rev., 56: 79, 1952. 10 Johnson, W. F.: Carcinoma in a polycystic kidney. J. Urol., 69: 10, 1953. 11 Brannan, W., Miller, W. and Crisler, M.: Coexistence of renal neoplasms and renal cysts. South. Med. J., 55: 749, 1962.
Fm. 1. IVP reveals multiple smooth filling defects in both kidneys. lower poles of the left kidney (fig. 2, B). Multiple smooth defects were again seen in both kidneys on the arteriogram (figs. 2, B and 3). Creatinine clearance was 124 ml. per minute. All other laboratory data were within normal limits. Exploration of the left kidney was performed on September 6, 1967. The mass was biopsied and a frozen section showed renal cell carcinoma. A nephrectomy was performed.. Gross and microscopic examination of the specimen showed renal cell carcinoma in the upper and lower poles of a polycystic kidney (fig. 4).
162
TWO MALIGNANT TUMORS IN POLYCYSTIC KIDNEY
163
FIG. 2. A, IVP of left kidney shows large mass arising from inferior pole. B, retrograde femoral arteriogram shows area of increased vascularity with tumor staining in upper and lower poles of left kidney.
Convalescence 1vas uneventful. The postoperative creatinine clearance ,vas 58 ml. per minute. DISCUSSION
The defects in both kidneys observed on the pyclogram appeared typical of polycystic disease. The urologists felt that no further studies were indicated. An arteriogram was requested by the surgeon under whose care the patient was admitted because of a tense mass in the lower pole of the kidney. This particular mass was found at exploration to be a large cyst. However, the arteriogram revealed 2 areas suggesting neoplasm. Since it has not been our practice to perform arteriograms on patients with polycystic disease, the question arises as to how often this should be done. Although other individual cases are reported, a preoperative diagnosis is definitely stated in only one of these (table 1). 3 The types of neoplasm associated with polycystic disease are listed in table 2. Butt and Wright reported on 237 patients with kidney tumors from 1938 to 1963. 12 Three of FIG. 3. Right nephrogram shows radiolncent defects.
12 Butt, W. P. and Wright, F. W.: Renal tumours masquerading as polycystic disease. Brit. J. Radio!., 37: 952, 1964.
164
HOWARD AND YOUNG
Frn. 4. A, left nephrectomy specimen shows cysts but tumors are difficult to identify. B, left kidney bivalved shows renal cell carcinoma in upper and lower poles. TABLE
1. Malignant tumors in polycystic kidneys Total reported-17 Diagnosed preoperative1y-l Diagnosed at operation-6 Diagnosed at autopsy-2 Time of diagnosis not men tioned-8
TABLE
2. Histological diagnosis of titmors polycystic kidneys
in
Adenocarcinoma-10 Intracystic papillomas-2 Angiomyosarcoma-1 Fibrosarcoma-1 Malignant papillary cystadenomas-1 Pathology report not mentioned-2
these cases were initially diagnosed as polycystic kidneys. It is doubtful that arteriography is a justifiable procedure in all patients with polycystic kidneys. On the other hand, associated tumor may be more common than suspected, as suggested by the chance diagnosis in our patient. A much larger left kidney was a feature of the findings in this patient. Perhaps such asymmetry might be an indication for investigation beyond pyelography. A difference in kidney size is mentioned in only 3 of the 17 reported cases. According to Rall and Odel, the difference in size might also be due to unilateral polycystic disease in 12 per cent of
cases. 7 Other indications suggesting further exploration, including the use of arteriography, might be calcification in the region of the kidneys on a scout film, amputation of a calyx, giant calyces or erosion as evidence of subepithelial infiltration. Our patient has normal function in her remaining right kidney. Her life expectancy with this one polycystic kidney should be better than with renal cell carcinoma at the age of 32 years. The hematuria in this patient might arouse suspicion of something other than polycystic disease. It is our impression that hematuria from polycystic disease alone usually occurs in more advanced stages of the disease when some degree of hypertension and/or renal failure has developed. Although this patient had episodes of hematuria for 8 years, she had no evidence of renal failure or hypertension. Two other patients in the literature presented with painless hematuria and normal renal function. 3 • 6 Presenting signs and symptoms were not mentioned in 11 cases. SUMMARY AND CONCLUSIONS
A patient with 2 areas of renal cell carcinoma in a left polycystic kidney is presented. Selected patients with pyelographic changes indicating polycystic kidneys should be further evaluated for renal carcinoma,
THE JOURNAL OF UROLOGY
Copyright © 1969 by The Williams & Wilkins Co.
TWO MALIGNANT TUMORS IN A POLYCYSTIC KIDNEY RALPH M. HOWARD
AND
J. D. YOUNG, JR.
From the Division of Urology, Department of Surgery, University of Maryland School of 1vledicine, Baltimore, Maryland
men on the left side. The right kidney was thought palpable just below the right costal margin. There were no other positive findings. An excretory urogram (IVP) indicated multiple, smooth filling defects involving both collecting systems (fig. 1). The right kidney measured 16 cm. and the left 23 cm. in length. A large mass could be seen arising from the inferior pole of the left kidney (fig. 2, A). A retrograde femoral arteriogram showed an area of increased. vascularity with tumor staining in the upper and
Carcinoma in a polycystic kidney has been reported on at least 17 occasions. 1- 11 The palpable masses and/or hematuria of polycystic disease might mask a neoplasm. Coincidental discovery by aortography of 2 renal cell tumors in the left polycystic kidney of a young woman was thought of sufficient interest to report. CASE REPORT
A 32-year-old Negress was admitted to the University of Maryland Hospital for evaluation of a left abdominal mass. Although she had been aware of the mass for about 9 months, it had become painful just prior to admission. She had had intermittent gross hematuria for 8 years but denied any other urinary symptoms. One sibling had polycystic kidneys. Physical examination revealed a well-developed, young woman with blood pressure of 130/70 mm. Hg. An irregular, non-tender mass was palpated in the left flank and upper abdoAccepted for publication September 5, 1968. 'Walters, W. and Braasch, W. F.: Surgical aspects of polycystic kidneys. Surg., Gynec. & Obst., 58: 649, 1934. 2 Oppenheimer, G. D.: Polycystic disease of the kidney. Ann. Surg., 100: 1136, 1934. 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 Bobbitt, R. M.: Secondary pathological changes in polycystic kidney disease. J. Urol., 50: 134, 1943. 5 Lowsley, 0. S. and Curtis, M. S.: Surgical aspects of cystic kidney disease. J.A.M.A., 127: 1112, 1945. 6 Hayward, W. G.: Hypernephroma in a polycystic kidney. J. Urol., 56: 190, 1946. 7 Rall, J. E. and Ode!, H. M.: Congenital polycystic disease of kidney: review of literature and data on 207 cases. Amer. J. Med. Sci., 218: 399, 1949. 8 Baurys, W. and Morton, W.: Papillary carcinoma in a polycystic kidney. Urol. & Cutan. Rev., 54: 662, 1950. 9 Lewis, E. L. and Kimbrough, J. C.: Bilateral hypernephroma associated with polycystic disease. Urol. & Cutan. Rev., 56: 79, 1952. 10 Johnson, W. F.: Carcinoma in a polycystic kidney. J. Urol., 69: 10, 1953. 11 Brannan, W., Miller, W. and Crisler, M.: Coexistence of renal neoplasms and renal cysts. South. Med. J., 55: 749, 1962.
Fm. 1. IVP reveals multiple smooth filling defects in both kidneys. lower poles of the left kidney (fig. 2, B). Multiple smooth defects were again seen in both kidneys on the arteriogram (figs. 2, B and 3). Creatinine clearance was 124 ml. per minute. All other laboratory data were within normal limits. Exploration of the left kidney was performed on September 6, 1967. The mass was biopsied and a frozen section showed renal cell carcinoma. A nephrectomy was performed.. Gross and microscopic examination of the specimen showed renal cell carcinoma in the upper and lower poles of a polycystic kidney (fig. 4).
162
TWO MALIGNANT TUMORS IN POLYCYSTIC KIDNEY
163
FIG. 2. A, IVP of left kidney shows large mass arising from inferior pole. B, retrograde femoral arteriogram shows area of increased vascularity with tumor staining in upper and lower poles of left kidney.
Convalescence 1vas uneventful. The postoperative creatinine clearance ,vas 58 ml. per minute. DISCUSSION
The defects in both kidneys observed on the pyclogram appeared typical of polycystic disease. The urologists felt that no further studies were indicated. An arteriogram was requested by the surgeon under whose care the patient was admitted because of a tense mass in the lower pole of the kidney. This particular mass was found at exploration to be a large cyst. However, the arteriogram revealed 2 areas suggesting neoplasm. Since it has not been our practice to perform arteriograms on patients with polycystic disease, the question arises as to how often this should be done. Although other individual cases are reported, a preoperative diagnosis is definitely stated in only one of these (table 1). 3 The types of neoplasm associated with polycystic disease are listed in table 2. Butt and Wright reported on 237 patients with kidney tumors from 1938 to 1963. 12 Three of FIG. 3. Right nephrogram shows radiolncent defects.
12 Butt, W. P. and Wright, F. W.: Renal tumours masquerading as polycystic disease. Brit. J. Radio!., 37: 952, 1964.
164
HOWARD AND YOUNG
Frn. 4. A, left nephrectomy specimen shows cysts but tumors are difficult to identify. B, left kidney bivalved shows renal cell carcinoma in upper and lower poles. TABLE
1. Malignant tumors in polycystic kidneys Total reported-17 Diagnosed preoperative1y-l Diagnosed at operation-6 Diagnosed at autopsy-2 Time of diagnosis not men tioned-8
TABLE
2. Histological diagnosis of titmors polycystic kidneys
in
Adenocarcinoma-10 Intracystic papillomas-2 Angiomyosarcoma-1 Fibrosarcoma-1 Malignant papillary cystadenomas-1 Pathology report not mentioned-2
these cases were initially diagnosed as polycystic kidneys. It is doubtful that arteriography is a justifiable procedure in all patients with polycystic kidneys. On the other hand, associated tumor may be more common than suspected, as suggested by the chance diagnosis in our patient. A much larger left kidney was a feature of the findings in this patient. Perhaps such asymmetry might be an indication for investigation beyond pyelography. A difference in kidney size is mentioned in only 3 of the 17 reported cases. According to Rall and Odel, the difference in size might also be due to unilateral polycystic disease in 12 per cent of
cases. 7 Other indications suggesting further exploration, including the use of arteriography, might be calcification in the region of the kidneys on a scout film, amputation of a calyx, giant calyces or erosion as evidence of subepithelial infiltration. Our patient has normal function in her remaining right kidney. Her life expectancy with this one polycystic kidney should be better than with renal cell carcinoma at the age of 32 years. The hematuria in this patient might arouse suspicion of something other than polycystic disease. It is our impression that hematuria from polycystic disease alone usually occurs in more advanced stages of the disease when some degree of hypertension and/or renal failure has developed. Although this patient had episodes of hematuria for 8 years, she had no evidence of renal failure or hypertension. Two other patients in the literature presented with painless hematuria and normal renal function. 3 • 6 Presenting signs and symptoms were not mentioned in 11 cases. SUMMARY AND CONCLUSIONS
A patient with 2 areas of renal cell carcinoma in a left polycystic kidney is presented. Selected patients with pyelographic changes indicating polycystic kidneys should be further evaluated for renal carcinoma,