Acquired cystic disease of the kidneys and renal cell carcinoma in chronic renal insufficiency without dialysis treatment

We report 3 cases of acquired cystic disease of the kidneys with associated renal carcinoma in 2 of the cases. In all 3 cases, the patients had chronic renal insufficiency due to hypertension but had never required dialysis. Review of 176 reported cases of acquired cystic disease of the kidneys and renal tumors disclosed that 18 patients (including 1 previously reported by us) had never received dialysis treatment. These cases support the hypothesis that acquired cystic disease of the kidney is not restricted to patients treated with maintenance dialysis. Among the 18 patients, hypertension was the most common underlying cause of renal failure. Patients with chronic renal failure due to or associated with severe hypertension should be monitored carefully for the development of both renal cysts and tumors even though they have not started on chronic dialysis.     

Ex vivo renal artery reconstructions: indications and techniques

Ex vivo renal artery surgery has been reported by several investigators and has extended the role of revascularization in the treatment of lesions previously managed by nephrectomy alone. Several techniques are available for use, and selection of the most appropriate method can be tailored to the specific anatomy being managed. Our total experience included 27 kidneys that have been managed by ex vivo renal artery reconstruction. Lesions managed in this manner include two kidneys with renal artery stenosis and renal tumors, one kidney with a congenital branch arteriovenous malformation, and 24 kidneys with branch occlusive or aneurysmal disease from fibromuscular dysplasia. Postoperative angiography was performed in 22 cases and defined successful revascularization without technical error in 20 cases. One operative death occurred as a result of myocardial infarction. One patient required reoperation to control bleeding, and two patients had temporary acute tubular necrosis during the postoperative period. Techniques employed included ex vivo repair with autotransplantation to the iliac system (six kidneys), mobilization and perfusion without transection of the renal vein (10 kidneys), and ex vivo perfusion and repair with replacement into the original renal fossa (11 kidneys). The authors believe this latter technique of reconstruction to be preferable to autotransplantation for the usual patient undergoing ex vivo repair of complex renovascular lesions.     

Primary renal lymphoma presenting as acute renal failure

Diffuse bilateral infiltration of the kidneys by lymphoma cells is a rare but well-documented cause of acute renal failure (ARF). Only 51 such cases have been reported, 15 of which had ARF as the initial presentation of lymphoma. The clinical and pathologic features of these 15 cases and of two additional cases reported herein are reviewed. The diagnosis should be suspected in a patient with ARF, bilateral enlargement of the kidneys, minimal proteinuria, nonspecific findings on urinalysis, and absence of features of allergic tubulointerstitial nephritis. Renal imaging techniques may suggest the possibility of lymphomatous infiltration, but only renal biopsy or autopsy can provide a definitive diagnosis. Although modern chemotherapy and/or radiation therapy usually leads to a dramatic normalization of renal function, almost all patients eventually die of widespread recurrent lymphoma, despite the absence of clinical or pathologic involvement of the kidneys at the time of death.     

The long-term effect of simultaneous heart and kidney transplantation on native renal function.

BACKGROUND: It is unclear whether patients with heart failure and renal insufficiency should receive a simultaneous heart and kidney transplant or whether a single heart transplantation is sufficient to restore native renal function. METHODS: We analyzed the renal plasma flow and glomerular filtration of the native and transplant kidneys in eight patients long term after simultaneous heart and kidney transplantation using a dynamic MAG3 radioisotope scan and serum creatinine determinations. All subjects had been hemodialysis dependent before transplantation. Seven patients suffered from an intrinsic renal disease that were diabetic nephropathy in three cases, small fibrotic kidneys of undetermined origin in two cases, one lupus nephritis, and cyclosporine nephrotoxicity in one patient who had a previous heart transplant. In one patient renal insufficiency was considered to be solely due to renal hypoperfusion because no intrinsic renal disease could be detected. RESULTS: All patients were on cyclosporine-based triple immunosuppression, transplanted for 4 to 10 years, exhibited cardiac ejection fractions of more than 50% and had normal serum creatinine values. Radioisotopic scan showed no function of the native kidneys in all seven patients with intrinsic renal disease but exhibited normal function of the native kidneys as well as the renal transplant in the patient without intrinsic kidney disease before transplantation. CONCLUSIONS: These data suggest that a simultaneous heart and kidney transplantation is necessary in patients with cardiomyopathy and renal insufficiency due to primary kidney disease, but not in those with hemodynamically mediated renal failure, even if an immunosuppressive regimen with calcineurin inhibitors is used.     

End stage renal disease associated with acquired cystic disease and neoplasia

Acquired renal cystic disease is a recently recognized disease entity that primarily afflicts patients on chronic hemodialysis. The incidence of acquired renal cystic disease in patients with end stage renal disease ranges from 30 to 95 per cent. The major complications of acquired renal cystic disease appear to be neoplasia and hemorrhage. Neoplasia has been demonstrated in up to 45 per cent of all end stage kidneys with acquired cystic disease. Our surgical experience with 7 cases of end stage renal disease is reported. Acquired renal cystic disease was noted in 4 of the 7 cases (57 per cent) and renal neoplasia was evident in all 7. Worldwide experience to date in combined pathology and radiology series of patients undergoing hemodialysis demonstrates that acquired cystic disease, "tumor" (usually adenoma or oncocytoma) and renal carcinoma occur at respective rates of 47.1, 4.8 and 1.5 per cent. Since the pathologist will detect more tumors and cysts than the radiologist, these figures probably are an underestimate of the true incidence. Long-term surveillance of patients harboring end stage kidneys is recommended beginning with a baseline ultrasound.     

An unusual cause of acute renal failure: renal lymphoma

Renal involvement is a common finding in non-Hodgkins lymphoma (NHL). Acute renal failure at initial presentation due to lymphomatous infiltration of the kidneys has been described infrequently. We report a 17-year-old male who presented with acute renal failure due to massive lymphomatous infiltration of the kidneys, which necessitated hemodialysis. The diagnosis of B-cell NHL was established by tru-cut biopsy of the kidneys and the patient had an excellent response to high-dose chemotherapy with no major complication. The presence of extrarenal involvement in the testes and the retroperitoneal lymph nodes made the diagnosis of primary renal lymphoma debatable. However, considering the delay in diagnosis and the high proliferative rate of B-cell NHL, we might postulate that the disease had originated primarily in the kidneys. We recommend that in NHL cases with severe renal involvement, full-dose chemotherapy should be instituted with meticulous clinical and laboratory follow-up in order to improve clinical and renal failure status rapidly and to avoid further dissemination of NHL.     

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.     

Acquired renal cystic disease in the end stage kidney: urological implications

A unique form of acquired renal cystic disease occurs commonly in the end stage kidneys of patients with chronic renal failure. Recent experience with 3 cases of acquired renal cystic disease has made us aware that the condition has significant urological implications. The pathogenesis of this disease is unknown but may be related to tubular obstruction, ischemia or the accumulation of toxic products. The diagnosis of acquired renal cystic disease is established by either ultrasound or computerized tomography, both of which demonstrate bilateral multiple small cysts scattered throughout the cortex and medulla of the contracted end stage kidney. Acquired renal cystic disease usually is asymptomatic but may be associated with either hemorrhage or neoplasia. Autopsy studies have revealed renal tumors in up to 45 per cent of the patients with acquired renal cystic disease. These tumors usually are small but our case 3 was a renal cell carcinoma that measured 4 cm. in diameter. Also, there have been other recent reports of large tumors and deaths of metastatic renal carcinoma in patients with acquired renal cystic disease. Patients with chronic renal failure should undergo periodic examination of the native kidneys by either ultrasound or computerized tomography. It may be difficult to distinguish benign from malignant lesions radiologically, and nephrectomy may be indicated when the diagnosis is uncertain.     

Renal cell carcinoma in crossed fused renal ectopia

Crossed renal ectopia its a rare congenital malformation and in most cases it presents with fusion of both kidneys. Patients should be asynptomatic until 4a-5a decade and at that time they unfold urinary infection, urolithiasis, abdominal mass or pain that simulates a gastric disease. We report a case of renal cell carcinoma in a patient with crossed fused renal ectopia, showed in a Scan that was performed for gastric disease suspicion.     

Characterization of renal artery stenoses based on magnetic resonance renal flow and volume measurements

BACKGROUND: The benefits of percutaneous angioplasty of renal artery stenosis is not known. In an attempt to better define the patient subgroup most likely to benefit from a revascularization procedure, the relationship between renal arterial morphology, blood flow volume, and renal volume was investigated using magnetic resonance (MR) techniques. METHODS: Analysis was based on arterial flow volume and renal volume measurements of 130 kidneys in 65 patients with clinically suspected renal vascular disease. Significant renal arterial stenosis (RAS), documented by contrast-enhanced three-dimensional MR angiography, compromised blood supply to 31 kidneys. Renal volume measurements were related to the body mass index to derive the renal volume index (RVI). The RVI of 72 kidneys in 36 patients without evidence of RAS or severe renal insufficiency was used as a standard of reference to differentiate normal-volume from reduced-volume kidneys. RESULTS: Eighteen out of 31 RAS kidneys were significantly reduced in volume (3.08 +/- 0.75); the volume of the remaining 13 kidneys was within one standard deviation of the normal reference value. Flow volumes in kidneys with RAS were significantly reduced compared with kidneys without RAS (91.56 vs. 279.15 ml/min). Based on the RFI values (RFI = flow volume/renal volume), there was only minimal overlap between normal volume kidneys with RAS (0.73 +/- 0.34) and kidneys without RAS (2.02 +/- 0.59). RFI values of small volume kidneys with RAS (1.55 +/- 0.47), on the other hand, overlapped with both groups. CONCLUSIONS: Normal volume kidneys with impaired arterial flow caused by RAS can be differentiated from those without based on a flow index (RFI). These data suggest the existence of a critical cut-off value (flow index <1.2 ml/min per cm3 of renal tissue) beyond which the renal parenchyma starts shrinking.     

Bilateral renal inflammatory pseudotumour effectively treated with corticosteroid

Inflammatory pseudotumour (IPT) is a rare disease of unknown cause that most commonly involves the lung but can occur in almost any site in the body. Occurrence in the kidneys is very rare and bilateral renal involvement even rarer. There are 34 previously reported cases in the English-language medical literature between 1966 and 2008. Herein we report a case of IPT infiltrating both kidneys. We have also reviewed the clinical features, radiological findings, treatment and outcome of renal IPT. Clinical features at presentation are commonly non-specific. Features on imaging are inadequate to make a diagnosis of IPT or to clearly distinguish it from malignancy. Consequently diagnosis has frequently been made after nephrectomy and on a few occasions with the aid of percutaneous or open biopsies. The majority of renal IPT (83%) have been treated with nephrectomy and those cases with bilateral IPT have received corticosteroids.     

Bilateral renal oncocytomatosis in a patient with renal failure

Bilateral multifocal renal oncocytomas are very rare disorders with only 6 previously reported cases in the world literature, of which only 3 have had pathologic confirmation. We present the first reported case of diffuse, bilateral, multifocal renal oncocytomatosis in a patient with end-stage renal disease requiring hemodialysis. Our patient was found to have hundreds of nodular tumors in both kidneys on exploration, representing the second such reported finding.     

The diagnosis of renal tuberculosis

The incidence of renal tuberculosis is today less than in the past two decades due to the reduction in the incidence of other forms of tuberculosis. It has been fully established that renal tuberculosis is secondary to tuberculous infection elsewhere in the body and that the organism is carried to the kidneys by the blood stream in most instances.The question of bilateral involvement of both kidneys, at the outset, is not fully decided; however, the work of Medlar and Sasana demonstrated 88 per cent of their guinea-pigs to have bilateral renal lesions. Clinically, Braasch and Sutton were only able to demonstrate 291 cases (13 per cent) of bilateral involvement in a total of 2,200 cases of renal tuberculosis studied.The symptoms vary from nil to those of a very painful cystitis with later pain and tenderness in the kidney region. Occasionally, hematuria may be the first symptom. A deceptive feature in some cases is the occurrence of long remissions free of painful symptoms. No symptom or train of symptoms is pathognomonic of renal tuberculosis.The urinary findings give the most important evidence of the presence of renal tuberculosis. Pus cells are regularly found and red blood cells and albumin are usually present throughout the entire course of the disease. The acid fast bacillus is found on staining the centrifuged urinary sediment in a large percentage of the cases and guinea-pig inoculations are to be employed when the smears give no decisive results.The cystoscopic appearance of the bladder is quite characteristic. Two types of lesions exist: granulations and ulceration. The function of each kidney is to be determined as well as the microscopic contents in the urine of each kidney. The presence of a few organisms in the urine from one kidney in the absence of pus cells, red blood cells and albumin may represent a contamination in passing the ureteral catheter through an infected bladder.The pyelogram is of great value. A small defect may first direct the examiner's attention to the possibility of a tuberculous infection. Cortical necrosis is demonstrated when the lesion involves the renal papilla. The excretory urogram usually does not demonstrate early defects as well as the retrograde pyelogram. However, the excretory urogram is valuable in following the course of the disease and in demonstrating disproportion in the involvement of the kidneys.     

Diagnosis of renal trauma

We experienced 25 cases of renal trauma in our hospitals during the past 5 years, 20 cases were male and 5 cases were female, ranging in age 10 to 51 years old with average 26 years old, and the chief complaints were gross-hematuria and pain of renal region. We studied the IVP and CT patterns of these cases. IVP revealed 13 cases of renal contusion, 11 cases of renal lacerated wound and 1 case of renal fragmentation. The CT scan of 14 cases showed that it was useful for the diagnosis of the degree of renal trauma, especially, for hematoma surroundings the kidneys. The hematoma surrounding the kidneys was clearly noted in 7 cases by CT scan, but the unclearness of renal contours and iliopsoas muscle was considered to suggest the existence of hematoma surrounding the kidneys by IVP. All of the hematoma surrounding the kidneys was absorbed by conservative therapy, but 5 of the 7 cases were treated within 3 months.     

Analysis of cytodiagnostic urinalysis findings in 77 patients with concurrent renal biopsies.

We investigated the value of cytodiagnostic urinalysis in detecting and scoring the severity of the four types of renal lesions (glomerular, interstitial, tubular, and vascular). Both cytodiagnostic urinalysis and concurrent renal biopsy were performed in 77 patients (47 from native kidneys and 30 from transplants) and the scoring and findings assessed in a double-blinded fashion. Evaluation of the reproducibility for the counting of renal cells showed a low intraobserver and interobserver variation. Cytodiagnostic urinalysis correlated with the renal biopsy with respect to primary lesions in 42 (89%) of the native kidney cases, and in 23 (77%) of the transplant kidney cases. The accuracy of diagnosis of glomerular lesions in both native and transplanted kidneys was 0.91, and for acute rejection in transplanted kidneys, the accuracy of diagnosis was 0.73. Severity scores showed good correlation between cytodiagnostic urinalysis and renal biopsy in both transplanted and in native kidneys and cytodiagnostic urinalysis correlated well with the increase in creatinine levels. The most important components of cytodiagnostic urinalysis for the diagnosis of glomerular lesions were dysmorphic erythrocytes and proteinuria. The specificity and sensitivity of dysmorphic erythrocytes for a glomerular lesion were 0.89 and 0.88, respectively. In cases with biopsy-proven glomerular lesions, more severe changes were found by cytodiagnostic urinalysis when the biopsy showed proliferative lesions in the glomeruli than when normal glomeruli were found by light microscopy. Cytodiagnostic urinalysis has the advantage over renal biopsy that it can be repeated as often as necessary and thus allows observation of the development or regression of the renal lesion over time. We conclude that cytodiagnostic urinalysis is well correlated with the renal biopsy and that it provides valuable and quantitative information regarding the disease process in the kidney.     

C-erb B-2 amplification in cystic renal disease.

Gene amplification (overexpression) of c-erb B-2 was tested in a variety of cystic renal diseases, renal cell neoplasms (adenomas and carcinomas) and end stage kidneys without cysts. C-erb B-2 encodes a receptor-like protein that shares homology with, but is distinct from the epidermal growth factor (EGF) receptor. A monoclonal antibody that immunoprecipitates a protein of approximately 185 kD from a lysate of NIH/3T3 cells transfected with the c-erb B-2 gene was utilized for testing. Simple renal cysts, cystic renal dysplasia, autosomal recessive polycystic kidney disease (ARPKD), and non-cystic, essentially normal kidneys failed to show c-erb B-2 overexpression. In contrast, autosomal-dominant polycystic kidney disease (ADPKD), acquired (dialysis-associated) cystic disease (ACD), non-cystic end stage kidneys and renal cell neoplasms revealed overexpression of c-erb B-2 with some frequency (40% or more of cases tested). Three cystic disorders revealing c-erb B-2 overexpression also showed platelet-derived growth factors (PDGFs) expression in similar locations (cyst lining and adjacent tubules). Other growth factors [insulin-like growth factor (IGF-I), fibroblast growth factor (FGF) and beta transforming growth factor (TGF beta)] were not noted to be overexpressed in either c-erb B-2 positive or negative cystic diseases. C-erb B-2 may be a marker related to the proliferative/growth capabilities of selected cystic diseases, including potential for associated genesis of benign and malignant renal cell tumors.     

Acute renal failure in a patient with Rosai-Dorfman disease

Acute renal failure developed in a 57-year-old woman who had Rosai-Dorfman disease diagnosed 1 year previously on a cervical lymph node. Organ imaging showed diffuse masses infiltrating both kidneys. The renal biopsy showed a lymphoplasmacytic and histiocytic process extensively replacing the parenchyma, which is in keeping with Rosai-Dorfman disease of the kidneys. However, the typical lymphophagocytic cells were lacking. This case illustrates that diagnosis of Rosai-Dorfman disease in renal biopsy can be very difficult, requiring both exclusion of many benign and malignant lesions and a high index of suspicion for this condition. In particular, lymphoma was excluded based on the mixed polyclonal composition of inflammatory cells and the absence of atypical lymphoid proliferation. The renal function partially recovered after a course of therapy combining VP-16 (etoposide) and dexamethasone and remained stable over 4-year follow-up. This report emphasizes the importance of early diagnosis and intervention to safeguard renal function in extensive Rosai-Dorfman disease.     

Spontaneous renal rupture in pregnancy

A review of the literature reveals 9 cases of spontaneous renal rupture in pregnancy, to which we add another case. Most cases occurred in hydronephrotic kidneys. Four cases consisted of renal rupture without associated infection or tumor, 3 were associated with abscesses, and 3 ruptured through renal hamartomas. Previously, all ruptured kidneys were removed. In our case the kidney was salvaged satisfactorily by prompt placement of a nephrostomy tube and drainage of the perirenal tissue. Attempt at renal salvage is urged for renal rupture in pregnancy.     

Reflux nephropathy as a cause of end-stage renal failure

Reflux nephropathy can present as end-stage renal failure in adults without previous history of urinary infection. We describe four cases to illustrate this and suggest that in cases of end-stage renal failure with bilateral small kidneys a micturating cystogram should be performed prior to transplantation.