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Quiz Page Answers October 2005
AJKD
QUIZ PAGE ANSWERS OCTOBER 2005
A 51-year-old African-American man’s symptoms at presentation were 3 days of nausea and vomiting and a 25-lb weight loss during 3 months. He had a 3-year history of chronic kidney disease and hypertension. On admission, blood urea nitrogen level was 83 mg/dL (29.6 mmol/L), and serum creatinine level was 10.6 mg/dL (937 mol/L). Ultrasound revealed that the right kidney (9.3 ⫻ 2.5 cm) had several cysts, including a complex one (3.3 ⫻ 3.5 cm) with thick septations and prominent vascularity. In the left kidney (11.4 ⫻ 5.7 cm), there was a simple cyst (4.3 ⫻ 2.6 cm). Hemodialysis therapy was started, and a computed tomographic (CT) scan was obtained.
Figure 44A. CT scan of the abdomen obtained on admission, showing bilateral complex cysts with thickened septae.
Figure 44C. Light microscopy from kidney tissue obtained at the time of bilateral nephrectomy.
Figure 44B. Light microscopy from kidney tissue obtained at the time of bilateral nephrectomy.
What is your diagnosis? The diagnosis is acquired cystic kidney disease (ACKD) with associated bilateral renal cell carcinoma (RCC; Figs 44A to C). The right kidney contained both papillary and clear cell cancer (Fig 44B); the left kidney had a pure clear cell cancer (Fig 44C). Unique to this case was the presence of concurrent bilateral cancers and morphologically distinct tumors in a patient only recently started on hemodialysis therapy.
AJKD
QUIZ PAGE ANSWERS (continued)
RCC in the setting of cystic kidney disease generally develops on a background ACKD. Dunnill et al1 first described this condition in 1977 in a series of autopsies. It was studied further by Ishiwaka et al,2 who used CT scans to characterize its natural history. In more than 20 years of follow-up, these investigators have shown a male preponderance, a progressive increase in kidney volume, and an association with RCC.2 Other investigators, by using chemical analysis of cystic fluid and ultrastructural analysis, have implicated the proximal tubular cell as the cell of origin of both ACKD and ACKD-associated RCC.2,3 Although a toxic effect of uremia on ACKD-associated RCC has been postulated,4 the pathogenesis remains undetermined.5 Close to 8% of persons starting maintenance dialysis therapy (hemodialysis or peritoneal dialysis) have been shown to have ACKD.6 This incidence repeatedly has been reported to approach 90% during 5 to 10 years.2,6,7 The importance of understanding ACKD is underscored by the high rate of neoplastic transformation. Basille et al8 reported that in the presence of ACKD, there is a 12- to 18-times greater incidence of renal tumors compared with the general population. Although most of these tumors are benign and include papillary adenomas, oncocytomas, angiomyolipomas, and epithelial hyperplasia, 18% have been reported to be malignant.9,10 ACKD-associated RCCs usually are unilateral, multifocal, less than 3 cm in diameter, and of low malignant potential.11 Nevertheless, up to 27% have been found to be metastatic,12 with even a fatal sarcomatoid variant having been reported.13 The present case is the fourth report of bilateral ACKD-associated RCC and the only known case with morphologically distinct tumors.7,14,15 In this patient, the cystic lesions were noted to be complex on initial ultrasound, and on subsequent CT scan, were assigned a Bosniak rank of category III. Although there is little controversy in the management of this patient, it was agreed that surgical exploration and likely bilateral nephrectomies were warranted. For lower grade lesions, current suggested clinical practice is to reexamine by means of serial CT scans, at first after 3 to 6 months, then annually for at least 5 years.16 There presently is no consensus on which dialysis patients should be screened for ACKD, but it has been suggested that young patients who have been on dialysis therapy for years and are transplant candidates may benefit from such screening.
Final diagnosis: ACKD with associated bilateral RCC. Case provided by Mohammad Vaseemuddin, MD, and Mark A. Kraus, MD, PhD, John H. Stroger Jr Hospital of Cook County, Chicago, IL. If you have an interesting case you would like to submit for consideration, please go to http://ajkd.edmgr. com to do so. REFERENCES 1. Dunnill MD, Millard PR, Oliver D: Acquired cystic kidney disease of the kidneys: A hazard of long term intermittent maintenance hemodialysis. J Clin Pathol 30:868877, 1977 2. Ishiwaka I, Saito Y, Asaka M: Twenty year follow-up of acquired renal cystic disease. Clin Nephrol 59:153-159, 2003 3. Truong LD, Krishnan B, Cao JTH, Barrios R, Suki WN: Renal neoplasm in acquired cystic kidney disease. Am J Kidney Dis 26:1-12, 1995 4. Pecqueux JC, Schwarz A, Dieckmann KP, Offermann G: Cancer incidence in patients on chronic dialysis and in renal transplant recipients. Urol Int 45:290-292, 1990 5. Peces R, Martinez-Ara J, Miguel JL, et al: Renal cell carcinoma co-existent with other renal disease: Clinicopathologic features in pre dialysis patients and those receiving
dialysis or renal transplantation. Nephrol Dial Transplant 19:2789-2796, 2004 6. Matson MA, Cohen EP: Acquired cystic kidney disease: Occurrence, prevalence, and renal cancers. Medicine (Baltimore) 69:217-226, 1990 7. Sant GR, Ucci AA: Acquired renal cystic disease and adenocarcinoma following renal transplantation: A current urologic perspective. Urol Int 60:108-112, 1998 8. Basille JJ, Mc Cullogh DL, Harrison LH, Dyer RB: End stage renal disease associated with acquired cystic disease and neoplasia. J Urol 140:938-943, 1998 9. Satoh T, Nagata M, Nukui F, et al: A case of renal oncocytoma associated with acquired cystic disease of the kidney. Hinyokika Kiyo 44:493-496, 1998 10. Bretan PN, Busch MP, Hricak H, Williams RD: Chronic renal failure: A significant risk factor in the development of acquired renal cysts and renal cell carcinoma. Case reports and review of the literature. Cancer 57:1871-1879, 1986 11. Sasagawa I, Nakada T, Kubota Y, Suzuki Y, Ishigooka M, Terasawa Y: Renal cell carcinoma in dialysis patients. Urol Int 53:79-81, 1994 12. Ishiwaka I: Renal cell carcinoma in chronic hemodialysis patients: A 1990 questionnaire study in Japan. Kidney Int Suppl 41:2167-2169, 1993
AJKD
QUIZ PAGE ANSWERS
13. Suvarna SK, Ahuja M, Brown CB: Sarcomatoid renal cell carcinoma arising in hemodialysis-associated acquired kidney disease presenting with disseminated bone marrow infiltration. Am J Kidney Dis 24:581-585, 1994 14. Gotoh A, Gohji K, Mizuno Y: A case of acquired cystic disease of bilateral kidneys with renal cell carcinoma following long term hemodialysis. Nippon Hinyokika Gakkai Zasshi 82:1986-1989, 1991
(continued)
15. Rioux-Leclercq NC, Epstein JI: Renal cell carcinoma with intratumoral calcium oxalate crystal deposition in patients with acquired cystic disease of the kidney. Arch Pathol Lab Med 127:E89-E92, 2003 16. Israel GM, Bosniak MA: Follow up CT of moderately complex cystic lesions of the kidney (Bosniak category II F). AJR Am J Roentgenol 181:627-633, 2003
QUIZ PAGE ANSWERS OCTOBER 2005
A 51-year-old African-American man’s symptoms at presentation were 3 days of nausea and vomiting and a 25-lb weight loss during 3 months. He had a 3-year history of chronic kidney disease and hypertension. On admission, blood urea nitrogen level was 83 mg/dL (29.6 mmol/L), and serum creatinine level was 10.6 mg/dL (937 mol/L). Ultrasound revealed that the right kidney (9.3 ⫻ 2.5 cm) had several cysts, including a complex one (3.3 ⫻ 3.5 cm) with thick septations and prominent vascularity. In the left kidney (11.4 ⫻ 5.7 cm), there was a simple cyst (4.3 ⫻ 2.6 cm). Hemodialysis therapy was started, and a computed tomographic (CT) scan was obtained.
Figure 44A. CT scan of the abdomen obtained on admission, showing bilateral complex cysts with thickened septae.
Figure 44C. Light microscopy from kidney tissue obtained at the time of bilateral nephrectomy.
Figure 44B. Light microscopy from kidney tissue obtained at the time of bilateral nephrectomy.
What is your diagnosis? The diagnosis is acquired cystic kidney disease (ACKD) with associated bilateral renal cell carcinoma (RCC; Figs 44A to C). The right kidney contained both papillary and clear cell cancer (Fig 44B); the left kidney had a pure clear cell cancer (Fig 44C). Unique to this case was the presence of concurrent bilateral cancers and morphologically distinct tumors in a patient only recently started on hemodialysis therapy.
AJKD
QUIZ PAGE ANSWERS (continued)
RCC in the setting of cystic kidney disease generally develops on a background ACKD. Dunnill et al1 first described this condition in 1977 in a series of autopsies. It was studied further by Ishiwaka et al,2 who used CT scans to characterize its natural history. In more than 20 years of follow-up, these investigators have shown a male preponderance, a progressive increase in kidney volume, and an association with RCC.2 Other investigators, by using chemical analysis of cystic fluid and ultrastructural analysis, have implicated the proximal tubular cell as the cell of origin of both ACKD and ACKD-associated RCC.2,3 Although a toxic effect of uremia on ACKD-associated RCC has been postulated,4 the pathogenesis remains undetermined.5 Close to 8% of persons starting maintenance dialysis therapy (hemodialysis or peritoneal dialysis) have been shown to have ACKD.6 This incidence repeatedly has been reported to approach 90% during 5 to 10 years.2,6,7 The importance of understanding ACKD is underscored by the high rate of neoplastic transformation. Basille et al8 reported that in the presence of ACKD, there is a 12- to 18-times greater incidence of renal tumors compared with the general population. Although most of these tumors are benign and include papillary adenomas, oncocytomas, angiomyolipomas, and epithelial hyperplasia, 18% have been reported to be malignant.9,10 ACKD-associated RCCs usually are unilateral, multifocal, less than 3 cm in diameter, and of low malignant potential.11 Nevertheless, up to 27% have been found to be metastatic,12 with even a fatal sarcomatoid variant having been reported.13 The present case is the fourth report of bilateral ACKD-associated RCC and the only known case with morphologically distinct tumors.7,14,15 In this patient, the cystic lesions were noted to be complex on initial ultrasound, and on subsequent CT scan, were assigned a Bosniak rank of category III. Although there is little controversy in the management of this patient, it was agreed that surgical exploration and likely bilateral nephrectomies were warranted. For lower grade lesions, current suggested clinical practice is to reexamine by means of serial CT scans, at first after 3 to 6 months, then annually for at least 5 years.16 There presently is no consensus on which dialysis patients should be screened for ACKD, but it has been suggested that young patients who have been on dialysis therapy for years and are transplant candidates may benefit from such screening.
Final diagnosis: ACKD with associated bilateral RCC. Case provided by Mohammad Vaseemuddin, MD, and Mark A. Kraus, MD, PhD, John H. Stroger Jr Hospital of Cook County, Chicago, IL. If you have an interesting case you would like to submit for consideration, please go to http://ajkd.edmgr. com to do so. REFERENCES 1. Dunnill MD, Millard PR, Oliver D: Acquired cystic kidney disease of the kidneys: A hazard of long term intermittent maintenance hemodialysis. J Clin Pathol 30:868877, 1977 2. Ishiwaka I, Saito Y, Asaka M: Twenty year follow-up of acquired renal cystic disease. Clin Nephrol 59:153-159, 2003 3. Truong LD, Krishnan B, Cao JTH, Barrios R, Suki WN: Renal neoplasm in acquired cystic kidney disease. Am J Kidney Dis 26:1-12, 1995 4. Pecqueux JC, Schwarz A, Dieckmann KP, Offermann G: Cancer incidence in patients on chronic dialysis and in renal transplant recipients. Urol Int 45:290-292, 1990 5. Peces R, Martinez-Ara J, Miguel JL, et al: Renal cell carcinoma co-existent with other renal disease: Clinicopathologic features in pre dialysis patients and those receiving
dialysis or renal transplantation. Nephrol Dial Transplant 19:2789-2796, 2004 6. Matson MA, Cohen EP: Acquired cystic kidney disease: Occurrence, prevalence, and renal cancers. Medicine (Baltimore) 69:217-226, 1990 7. Sant GR, Ucci AA: Acquired renal cystic disease and adenocarcinoma following renal transplantation: A current urologic perspective. Urol Int 60:108-112, 1998 8. Basille JJ, Mc Cullogh DL, Harrison LH, Dyer RB: End stage renal disease associated with acquired cystic disease and neoplasia. J Urol 140:938-943, 1998 9. Satoh T, Nagata M, Nukui F, et al: A case of renal oncocytoma associated with acquired cystic disease of the kidney. Hinyokika Kiyo 44:493-496, 1998 10. Bretan PN, Busch MP, Hricak H, Williams RD: Chronic renal failure: A significant risk factor in the development of acquired renal cysts and renal cell carcinoma. Case reports and review of the literature. Cancer 57:1871-1879, 1986 11. Sasagawa I, Nakada T, Kubota Y, Suzuki Y, Ishigooka M, Terasawa Y: Renal cell carcinoma in dialysis patients. Urol Int 53:79-81, 1994 12. Ishiwaka I: Renal cell carcinoma in chronic hemodialysis patients: A 1990 questionnaire study in Japan. Kidney Int Suppl 41:2167-2169, 1993
AJKD
QUIZ PAGE ANSWERS
13. Suvarna SK, Ahuja M, Brown CB: Sarcomatoid renal cell carcinoma arising in hemodialysis-associated acquired kidney disease presenting with disseminated bone marrow infiltration. Am J Kidney Dis 24:581-585, 1994 14. Gotoh A, Gohji K, Mizuno Y: A case of acquired cystic disease of bilateral kidneys with renal cell carcinoma following long term hemodialysis. Nippon Hinyokika Gakkai Zasshi 82:1986-1989, 1991
(continued)
15. Rioux-Leclercq NC, Epstein JI: Renal cell carcinoma with intratumoral calcium oxalate crystal deposition in patients with acquired cystic disease of the kidney. Arch Pathol Lab Med 127:E89-E92, 2003 16. Israel GM, Bosniak MA: Follow up CT of moderately complex cystic lesions of the kidney (Bosniak category II F). AJR Am J Roentgenol 181:627-633, 2003