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

Acquired cystic disease of the kidney (ACDK) is a common phenomenon in long-term adult dialysis patients with end-stage renal disease. Renal hemorrhage and neoplastic transformation of the cyst are two major complications of this entity and these two can occur independently. Here we describe a 65-year-old man with a history of hemodialysis-dependent end-stage renal failure for 12 years presented with macroscopic hematuria and right flank pain. Investigations revealed right massive perirenal and subcapsular hematoma with ACDK. The hemorrhagic state required nephrectomy of the right kidney. Histological study showed ACDK with massive subcapsular and perinephric hematoma containing minimal clear cell carcinoma region(0.5 cm in diameter). Although a causal relationship between renal hemorrhage and renal cell carcinoma in this patient was unproven, the present case suggests radical surgery can be a recommendable treatment modality of hemorrhagic ACDK.     

Renal cell carcinoma associated with acquired renal cystic disease

A patient with eight years’ history of haemodialysis treatment developed gross haematuria. During evaluation severe abdominal pain with dyspnoea developed and he died suddenly. Autopsy revealed spindle cell type renal cell carcinoma (RCC) associated with acquired renal cystic disease and adenoma in the remaining kidney. Multiple embolization of RCC was considered to be linked to the cause of death. Although patients on long-term haemodialysis often have multiple renal cystic lesions, some of which are associated with tumour formation, RCC of this specific cell type in dialysed kidney has not been previously described.     

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.     

Metastatic renal cell carcinoma associated with acquired cystic kidney disease 15 years after successful renal transplantation

Renal cell carcinoma (RCC) is a relatively uncommon cancer in renal transplant patients. From 1968 to 1987, 101 cases of RCC of native kidneys have been reported to the Cincinnati Transplant Tumor Registry. We describe here a case of metastatic RCC associated with acquired cystic kidney disease (ACKD) 15 years after successful renal transplantation. The patient presented with a subcutaneous nodule, which led to discovery of a large primary tumor in the left kidney. ACKD was present in the atrophic right kidney. The reported cases of ACKD-associated RCC in renal transplant recipients were reviewed. Most of these cases are middle-aged men with a long posttransplant course, good graft function, and usage of azathioprine and prednisone as immunosuppressive agents. ACKD can develop or persist and progress to RCC many years after successful renal transplantation. Transplant patients with flank pain, hematuria, or other suspicious symptoms should have imaging studies of their native kidneys.     

Clinical evaluation of renal cell carcinoma and acquired cystic disease of the kidney in long-term dialysis patients

Long-term dialysis patients frequently develop acquired cystic disease of the kidneys (ACDK). One hundred and sixty-seven dialysis patients were classified into two groups according to the presence or absence of ACDK, and the two groups were compared with regard to the dialysis period, laboratory findings, and so forth. Among the 167 dialysis patients, 37.7% showed ACDK and had an average dialysis duration of 10.3 years, which was significantly longer than the 5.0 years for patients without ACDK. The proportion of patients with ACDK rose with increasing duration of dialysis; 64.6% of the patients on dialysis for more than 10 years had ACDK. The values of hemoglobin and hematocrit of patients with ACDK were significantly higher than those without ACDK. Of 12 dialysis patients who were operated upon because of suspected renal tumor, pathological examination confirmed renal cell carcinoma in 8 and adenoma in one. One of these patients had been treated with chronic ambulatory peritoneal dialysis, and another had bilateral renal tumor. Among the 8 patients with renal cell carcinoma, ACDK was detected in 5 by means of CT and ultrasound tomography, and cystic changes were observed in two on light microscopic examination of the resected kidneys. Pathological examination of the non-tumorous renal tissues revealed hyperplastic or adenomatous changes in the cyst epithelium in 5 patients undergoing dialysis for more than 7.5 years. These changes were thought to be pre-neoplastic. The high incidence of ACDK and the development of renal tumors in long-term dialysis patients indicates the necessity of intensive monitoring.     

Acquired cystic disease of the kidney and renal cell carcinoma

This book is a well-presented and data-rich compendium on theproblem of acquired cysts and kidney cancers that occur in failedend-stage kidneys. Its Japanese author, Dr Isao Ishikawa, isthe world's authority on this subject. This book shows his comprehensive     

Metastasizing renal cell carcinoma and acquired renal cystic disease in a hemodialysis patient

Acquired renal cystic disease is an entity which is characterized by the progressive substitution of the atrophic renal parenchyma by multiple cysts in patients with renal insufficiency. Its main complications are hemorrhage and tumorous degeneration. The case discussed is a 57-year-old patient with terminal renal insufficiency secondary to interstitial nephropathy, who, following 6 years of treatment with hemodialysis and renal transplantation, developed a state of persistent hematuria requiring nephrectomy of the left kidney. Histological study revealed multiple cysts of monostratified epithelium with intracavitary projections, multiform adenomas and multifocal malignant tumorous polymorphism. The patient died in a state of progressive cachexia with pleural and hepatic metastasis.     

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

Abstract:   Patients receiving long-term hemodialysis tend to develop renal cell carcinoma (RCC). Among such cases, chromophobe RCC and so-called 'capsulomas' are rarely reported. Here, we report a case of a Japanese woman in her early 70s, who developed both renal lesions after 17 years of hemodialysis. The patient received radical nephrectomy for enlarging renal mass. Grossly, the resected kidney showed a dominant tumor and small-sized subcapsular nodules. Histologically, two types of neoplasm, chromophobe RCC and 'capsuloma', existed with acquired cystic disease of the kidney. Chromophobe RCC had eosinophilic cytoplasm with perinuclear halos, and some tumor cells showed oncocytic features. Hale's colloidal iron staining showed a weakly positive cytoplasmic reaction. Immunohistochemistry was diffusely positive for cytokeratin 7, but negative for vimentin in the tumor cells. 'Capsulomas' were multiple subcapsular nodules composed almost entirely of smooth muscle-like cells with immunoreactivity for melanosome-associated antigen detected by HMB-45.     

Calcium oxalate deposition in renal cell carcinoma associated with acquired cystic kidney disease: a comprehensive study

The main complication of acquired cystic kidney disease (ACKD) is frequent development of renal tumors, including renal cell carcinoma (RCC). Intratumoral deposition of calcium oxalate (CaOx) is a distinct feature of ACKD-associated RCCs, but several features of this type of RCC are not known. Features of the 30 end-stage renal disease (ESRD)-associated RCCs identified within a 13-year period, including eight with CaOx deposition, were analyzed. Pathologic and clinical features of CaOx positive (+) and negative (-) RCCs were evaluated and compared. The CaOx+ RCCs showed higher tendency for bilaterality and multifocality. Seven tumors displayed distinctive morphologic features characterized by tumor cells with ill-defined cell membrane, abundant granular eosinophilic cytoplasm, large nuclei, and prominent nucleoli. One tumor was of clear cell type. Regardless of histologic type, all tumors displayed a proximal tubular differentiation. No significant difference was noted for tumors' stage, proliferation, and apoptosis rate between the CaOx+ and CaOx- RCCs. CaOx+ RCCs account for a significant portion of all ESRD-associated RCCs. The majority of these RCCs display a distinctive morphologic profile. Proximal tubular cell differentiation in conjunction with ESRD-mediated high serum level may be pathogenetically important for intratumoral CaOx deposition. These RCCs seems to have a relatively good prognosis.     

A case of spontaneous rupture of renal cell carcinoma

A case of spontaneous rupture of renal cell carcinoma is reported. A 53-year old man was admitted with the chief complaint of sudden gross hematuria and right flank pain on December 28, 1979. On the following day, the clinical impression was right ruptured kidney, and therefore right nephrectomy was done. Pathological diagnosis was renal cell carcinoma. He received the post-operative irradiation of a total of 5,000 rads. He was seen five years later, at which time there was no evidence of local recurrence or distant metastasis of cancer. Thirty three cases of spontaneous rupture of renal cell carcinoma were collected from Japanese and English literature. Most common chief complaint is abdominal or flank pain. Excretory urography, ultrasonography, CT scan and angiography are useful, but it is difficult to diagnose preoperatively when the tumor is small. Therefore, it is important to suspect occult cancer when a reasonable cause of rupture is undetermined. In these indeterminate cases primary nephrectomy should be considered strongly.     

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.