A case of acquired cystic disease of bilateral kidneys with renal cell carcinoma following long-term hemodialysis]

We report a case of bilateral renal cell carcinoma which developed during about 14 years of hemodialysis. The patient of male was a 39-year-old with a chief complaint of macrohematuria 14 years prior to dialysis therapy. Computed tomography revealed multiple cystic changes of bilateral kidneys and a high density area in the right kidney. He was admitted to our department in April 1988. He was suspected of renal cell carcinoma of the right kidney and underwent transperitoneal radical nephrectomy on the right kidney. The left kidney was also resected simultaneously in part because it failed to function and in part because the long-term hemodialyzed patients are considered to have a complication of renal cell carcinoma at a high frequency. Pathological diagnosis was bilateral acquired cystic disease of the kidney (ACDK) with renal cell carcinoma. The kidney develops multiple cystic changes following long-term hemodialysis, which forms a high risk for development of renal cell carcinoma. This is a case of bilateral renal cell carcinoma which occurred after long-term hemodialysis, was reported with discussion and reference to the literature.     

Cystic local recurrence of renal cell carcinoma after laparoscopic radical nephrectomy in a hemodialysis patient

Although local recurrence of renal cell carcinoma after laparoscopic radical nephrectomy is sometimes reported, cystic local recurrence of renal cell carcinoma has rarely been reported. We report the case of a 59‐year‐old man with hemodialysis who developed cystic local recurrence of renal cell carcinoma accompanied by acquired cystic disease of the kidney in the retroperitoneal space after laparoscopic radical nephrectomy. A cystic tumor of 5.1 cm in diameter occurred in the left retroperitoneal space 15 months after left laparoscopic radical nephrectomy, and enlarged to 7.2 cm in diameter with enhanced mass along the wall of the cyst 36 months after surgery. The cystic tumor was removed and showed local recurrence of renal cell carcinoma on pathological examination.     

EPO-producing gastric carcinoma in a hemodialysis patient

A case of erythrocytosis caused by gastric cancer that produced erythropoietin is described. To the authors’ knowledge, no case of erythropoietin-producing gastric cancer has been reported until now. A 73-year-old man with a 4-year history of maintenance hemodialysis for diabetic nephropathy required phlebotomy. Serum erythropoietin level was 181 mU/mL (181 IU/L). Gastroscopy results showed rough mucosa with hemorrhaging caused by gastric cancer. The patient underwent distal gastrectomy, and serum erythropoietin level decreased to 27.1 mU/mL (27.1 IU/L) by postoperative day 8. Existence of erythropoietin in the tumor tissue was confirmed immunohistochemically. The presence of severe acquired cystic disease of the kidney, renal cell carcinoma, and other malignant tumors should be investigated in hemodialysis patients displaying erythrocytosis.     

Bilateral renal cell carcinoma associated with polycystic kidney disease: case report and literature review

A 56-year-old Japanese man consulted a urologist because of urethral bleeding. He had been undergoing hemodialysis for the past 15 years due to polycystic kidney disease. Computed tomography revealed an irregular cyst wall in the left kidney. Since a neoplasm could not be ruled out, we removed the left kidney, by laparoscopic radical nephrectomy after obtaining the patient's consent. Histopathologic diagnosis was renal cell carcinoma. Fourteen months after the operation, urethral bleeding recurred. Further examination of the bladder and the urethra revealed no significant abnormalities. The patient insisted on right nephrectomy. Therefore, laparoscopic radical nephrectomy was performed. Histopathologic diagnosis was also renal cell carcinoma. Renal cell carcinoma in patients with end-stage renal disease is fairly common and is associated with acquired cystic kidney disease. However, renal cell carcinoma associated with polycystic kidney disease is extremely rare.     

Chromophobe cell renal carcinoma with acquired cystic disease of the kidney in a long-term hemodialysis patient

We report a rare case of chromophobe cell renal carcinoma found in a 52-year-old female who had received hemodialysis therapy for 13 years. She was diagnosed as having a left renal tumor 7.5 cm in diameter with acquired cystic disease of the kidney (ACDK) by ultrasonographic examination during periodical systemic screening. As abdominal computed tomography scanning and enhanced color Doppler ultrasonography suspected that the hypervascular tumor was renal cell carcinoma, she underwent translumbar nephrectomy in July 2000. The histopathological diagnosis was chromophobe cell carcinoma with pT2 and grade 2 malignancy. Chromophobe cell carcinoma is uncommon among renal tumors with ACDK found in long-term hemodialysis patients.     

Renal tumors in end‐stage renal disease: A comprehensive review

The incidence of end‐stage renal disease has increased owing to the greater prevalence of patients with chronic kidney disease and diabetes mellitus. End‐stage renal disease is usually accompanied by acquired cystic disease and is a risk factor for renal cell carcinoma. The present review discusses the etiology of renal cell carcinoma in end‐stage renal disease patients, focusing on two unique renal cell carcinoma histological subtypes: acquired cystic disease‐associated renal cell carcinoma and clear cell papillary renal cell carcinoma. Acquired cystic disease‐associated renal cell carcinoma occurs almost exclusively in patients who underwent hemodialysis, especially long‐term (>10 years) hemodialysis. Its histology is distinctive: a cribriform or sieve‐like architecture with intra‐ or intracystic lumina; tumor cells containing abundant eosinophilic cytoplasm and large nuclei with prominent nucleoli; and most notably, calcium oxalate crystal deposition. Recognition of the crystals is critical for diagnosing acquired cystic disease‐associated renal cell carcinoma. Acquired cystic disease‐associated renal cell carcinoma typically has an indolent clinical course, except in cases with sarcomatoid components. Clear cell papillary renal cell carcinoma also has an indolent course (no cases involving metastasis have been reported to date), and its features resemble those of both clear cell renal cell carcinoma and papillary renal cell carcinoma. Unlike acquired cystic disease‐associated renal cell carcinoma, which occurs only in end‐stage renal disease patients, clear cell papillary renal cell carcinoma occurs in non‐end‐stage renal disease patients as well. Additional renal tumors in end‐stage renal disease patients include anastomosing hemangiomas. Long‐term hemodialysis worsens the prognosis of end‐stage renal disease patients with renal cell carcinoma, regardless of its original histological subtype, presumably by inducing oxidative stress and sarcomatoid transformation.     

2 cases of renal cell carcinoma on long-term hemodialysis

Two cases of renal cell carcinoma on maintenance dialysis for chronic renal insufficiency are reported. The first case, a 40-year-old man, complained of hematuria after 9.3 years of dialysis. Nephrectomy was done and small renal cell carcinoma with acquired cystic disease of the kidney were observed. The second case, a 48-year-old man, was found to have renal cell carcinoma after 2.5 years of dialysis by means of routine examination without any symptoms. The kidney showed carcinoma with small cystic areas and four adenomas. Usefulness of routine examination with echogram is stressed for management of hemodialysis.     

Letter To The Editor: “Relationship between Insulin Resistance and Malondialdehyde Levels in Dialysis Patients”

A patient with renal cell carcinoma in primitive kidney with acquired cystic renal disease following hemodialysis for 9 years was discovered during a routine ultrasonographic follow-up. Differences with these tumors in transplanted patients are stressed.     

Uremic acquired renal cystic disease. Natural history and complications

Males on long-term dialysis tend to develop more severe forms of acquired cystic disease of the kidney than females. The severity of the disease is unaffected by therapeutic modality, either hemodialysis or CAPD, however, cystic transformation becomes less extensive after successful renal transplantation. The fundamental pathological change characteristic of acquired cystic disease is epithelial hyperplasia, with the uremic milieu and duration of uremia being the most important factors in its development. Renal cell carcinoma, the most important complication of acquired renal cystic disease, has a high prevalence and incidence, usually remains symptomless and sometimes reveals metastases. Therefore, many clinicians and investigators, but not all, accept the need for regular screening of all dialysis patients for the development of renal cell carcinoma. Major complications of acquired renal cystic disease, malignancy and retroperitoneal bleeding, will become increasingly important because of the growing number of patients on long-term dialysis.     

Renal oncocytoma with bilateral synchronous renal cell carcinoma in a patient undergoing long-term hemodialysis

Abstract:   We report a case of bilateral synchronous renal cell carcinoma and renal oncocytoma in a 56-year-old male who had been treated with hemodialysis for 32 years. Because anemia gradually worsened, computed tomography and magnetic resonance imaging were carried out and revealed bilateral renal tumors within acquired cystic disease of the kidney. Bilateral nephrectomy was carried out, and the patient was diagnosed with multiple renal cell carcinomas and a single renal oncocytoma. To our knowledge, this is the first reported case of renal oncocytoma with synchronous renal cell carcinoma in a patient undergoing long-term hemodialysis.     

Collecting duct carcinoma with acquired cystic disease of the kidney in a long-term hemodialysis patient

Abstract:   We report a very rare case of collecting or Bellini duct carcinoma (CDC) found in a 60-year-old male who had received hemodialysis therapy for 21 years. Screening with ultrasonography revealed a solid tumor originating from the cyst wall in the right kidney with acquired cystic disease of the kidney. Subsequent computed tomography (CT) and angiography could not detect another renal tumor. Right radical nephrectomy was performed. The tumor detected preoperatively was composed of papillary renal cell tumor (RCC) and multiple clear cell carcinoma, pathologically. In addition to the tumors, CDC was revealed in the central medulla with the involvement of regional lymph nodes. Three months later, left nephrectomy was performed because left RCC was suspected during CT. The histological diagnosis was multiple clear cell carcinomas. Peritonitis carcinomatosa appeared and the patient died 13 months later.     

A case of pheochromocytoma associated with incidental multiple renal cell carcinoma originating from acquired cystic disease of the kidney in hemodialysis

The patient was a 52-year-old woman maintained on peritoneal dialysis for 9 years and on hemodialysis for 7 years. This patient complained of palpitations and hypertensive attack during the hemodialysis. Computed tomography and magnetic resonance imaging demonstrated the presence of right adrenal tumor(1.5 x 1.5 cm) and bilateral multiple renal cysts. Right adrenal pheochromocytoma was confirmed by elevated serum level of catecholamines and diagnosed by 131I-MIBG scintigram. This patient underwent right adrenalectomy and nephrectomy in consideration of future generation of cancer in the acquired cystic kidney. Histological examination confirmed malignant pheochromocytoma and small multiple renal cell carcinomas. The patient was alive without any recurrence 6 months postoperatively. To our knowledge, this is the fifth case of adrenal pheochromocytoma and renal cell carcinoma associated ACDK in a hemodialysis patient in the Japanese literature.     

Acquired cystic disease of kidney associated with renal cell carcinoma in chronic dialysis patients.

We describe 2 cases of acquired cystic disease of the kidney (ACDK) associated with renal cell carcinoma in patients treated with long-term hemodialysis. Both patients have had dialysis for five and eight years, respectively. Renal cell carcinomas of these patients are small, averaging 2 cm in diameter. They are clear cell type. Atypical epithelial hyperplasia arising from cystic areas can be seen intermingling with carcinoma. This confirms that atypical epithelial hyperplasia is a precursor of renal cell carcinoma. Although the incidence of renal cell carcinoma arising in ACDK is on the rise, the issue of how to manage patients with ACDK remained unsettled and required further study.     

Multiple urothelial cancers accompanied by acquired cystic disease of the kidney in a long-term hemodialysis patient

A 41-year-old miller undergoing hemodialysis for 9 years presented with recurrent episodes of asymptomatic gross hematuria of a 7 years' duration and left flank pain with slight fever of a four months' duration. CT scans revealed bilateral acquired cystic disease of the kidney (ACDK) and a mass (phi 4 cm) in the medial portion of the left kidney, which was angiographically a hypovascular tumor. Cystoscopic examination revealed multiple papillary tumors arising from the entire bladder wall. These findings suggested that the renal mass was a renal pelvic tumor. Bilateral nephroureterectomy with left paraaortic lymph nodes dissection and radical cystectomy were carried out, followed by urethrectomy two weeks later. Histologically, multiple papillary transitional cell carcinomas with G2 greater than G3 anaplasia were found in the left renal pelvis, the lower portion of the left ureter and the bladder. The tumors were predominantly superficial, although the renal pelvic one focally invaded into the muscular layer. Lymph node metastasis and renal cell carcinoma were not recognized. Postoperative course was uneventful with normal blood pressure, though anemia was slightly progressive. He has been free of disease for six months.     

Ten-year prospective study on the development of renal cell carcinoma in dialysis patients

A prospective study was undertaken to investigate the development of renal cell carcinoma in dialysis patients. Three renal cell carcinomas were detected among 96 hemodialysis patients in 1979, and screening by computed tomographic (CT) scan was continued yearly until 1989. During this 10-year period, one renal cell carcinoma was found in the second year and another in the ninth year. Autopsy performed on seven of 19 patients who died showed one case of small clear cell carcinoma accompanying acquired cystic disease. In 33 males, kidneys were found to have enlarged 2.7 +/- 1.7 times over the 10-year follow-up due to acquired cysts, while no change in kidney volume was noted in 24 females. Native kidneys in nine of 12 patients who maintained functioning grafts were reduced in size. The patient with the largest kidney enlargement (11.5 times) died from retroperitoneal bleeding in 1989. These prospective study results suggest that both the incidence and prevalence of renal cell carcinoma in dialysis patients is high. Furthermore, major complications of acquired renal cystic disease seem to occur predominantly in males.     

Xanthogranulomatous pyelonephritis with acquired cystic disease of the kidney in a haemodialysis patient

SUMMARY:   A 54-year-old-female patient who had received regular haemodialysis therapy for 12 years was referred to our hospital for evaluation of a left renal mass. Imaging examinations revealed acquired cystic disease of the kidney (ACDK) and a tumour-like lesion in the left kidney. Because of the preoperative diagnosis of the left renal cell carcinoma, the patient underwent a left nephrectomy. Pathological examination revealed xanthogranulomatous pyelonephritis. It was difficult to distinguish xanthogranulomatous pyelonephritis from renal cell carcinoma in our case, because it is very rare for xanthogranulomatous pyelonephritis to occur in ACDK.     

Renal cell carcinoma in acquired cystic disease of the kidney manifested by spontaneous renal rupture

A 67-year-old male had been maintained on hemodialysis for 13 years because of chronic renal failure secondary to diabetes mellitus. The patient was referred to our hospital with sudden right flank pain. Computed tomography revealed multiple cysts in both kidneys and a right massive perirenal hematoma. Although there was no definite evidence of a renal tumor, a right nephretomy was performed. Histological study revealed acquired cystic disease of the kidney (ACDK) with a hematoma containing papillary renal cell carcinoma. He has been free of recurrence for 2 months. To our knowledge, this case is the fourth report of renal cell carcinoma in ACDK manifested by spontaneous rupture in the Japanese literature.     

Two cases of renal cell carcinoma arising in the native kidney following renal transplantation--clinical study and review of 26 cases reported in Japan

Renal transplant recipients have an increased risk of developing malignancies due to long-term immunosuppression. Two cases of renal cell carcinoma arising from the native kidney following renal transplantation are reported. Case 1; A 60-year-old man underwent cadaveric renal transplantation on July 31, 1995. He had resumed hemodialysis because of graft failure on June 5, 2000. He was referred to our hospital with macroscopic hematuria on March, 2002. Computed tomography (CT) demonstrated a right renal mass of about 3.0 cm in diameter with bilateral acquired cystic disease of the kidney (ACDK). The radical nephrectomy was performed. Case 2; A 55-year-old man underwent cadaveric renal transplantation on November 15, 1996. He had resumed hemodialysis because of graft failure on June 5, 1996. A left renal mass measuring 3.0 cm in diameter as well as bilateral ACDK were found by CT on July, 2002. Left radical nephrectomy was performed. The histological diagnosis of both renal tumors was renal cell carcinoma, clear cell type, G2, pT1a. Fifty-six renal transplantations (48 were renal allografts from cadavers, while 8 were from living donors) were performed in our hospital from 1980 to 2002. Two patients (3.6%) developed renal cell carcinoma after transplantations. We also discuss a clinical study and review of 26 cases reported in Japan. Since the incidence of malignant neoplasia is high, CT and ultrasonography should be performed routinely on the patients with renal transplantation.     

Erythropoietin-producing renal cell carcinoma in chronic hemodialysis patients: A report of two cases

Erythropoietin (EPO)-producing renal cell carcinomas in two hemodialysis patients are reported. Despite deteriorated kidney function, these patients did not manifest anemia at diagnosis and their elevated serum EPO levels rapidly returned to within the normal range after nephrectomy. Immunohistochemical staining of the resected specimens showed production of erythropoietin in the tumor cells in one case and in the lining cells of the cyst wall in the other case. Renal cell carcinoma could cause an increase of blood hematocrit level in dialysis patients.     

Twenty-year follow-up of acquired renal cystic disease

AIMS: Since 1979 the diseased kidneys of 96 patients on replacement therapy with chronic renal failure due to chronic glomerulonephritis have been followed to investigate the development of acquired cysts and tumors. This is a report of the 20-year follow-up. MATERIALS AND METHODS: Ninety-six patients were followed using periodic CT scan and were divided into hemodialysis, renal transplantation, bilateral nephrectomy and deceased groups during the follow-up. In the hemodialysis group, 36 patients (19 males, 17 females) were followed for 20 years. RESULTS: Kidney volumes which were 57.8 (1.51) (geometric mean (geometric SD)) ml at start of the follow-up had become 185.3 (2.03) ml 20 years later in males, and in females, 57.3 (1.64) ml had become 99.7 (2.36) ml. The increased rate was 3.2 (2.06) fold in males and 1.7 (2.57) fold in females. This enlargement of the kidneys was due to acquired cysts. Kidney volumes at the 20-year follow-up had increased more significantly than those at the 15-year follow-up in males; however, kidney volumes at the 20-year follow-up had not changed in females, if compared with data at the 15-year follow-up. Kidney volumes in males at 20-year follow-up were significantly larger than those in females (p = 0.0232). Males with more than 3.2-fold in kidney volume increase at the 20-year follow-up were under the age of 40 at entry into this study (p = 0.0055), although the correlation between the degree of kidney volume increase and age was not significant (p = 0.0910). Kidney volumes in the transplantation group remained small. There was no new renal cell carcinoma development after 15-year follow-up except for the local recurrence of a previous operated case. Although 7 of 44 patients died during the past 20 years due to malignancy, no patient died of renal cell carcinoma because of early detection and treatment. One patient died of retroperitoneal bleeding, which is a complication of acquired renal cystic disease. CONCLUSION: Male preponderance of acquired cysts was maintained at the 20-year follow-up. There was a tendency for the rate of increase in acquired renal cystic disease to be larger in young males. No one died of renal cell carcinoma, although the incidence of renal cell carcinoma was high.