1/294. Bilateral wilms tumor in a boy with severe hypospadias and cryptochidism due to a heterozygous mutation in the WT1 gene.Mutations in the WT1 gene causing Wilms tumors were first reported in wagr syndrome (wilms tumor, aniridia, Genitourinary malformation, mental Retardation) and Denys Drash syndrome (pseudohermaphroditism, wilms tumor, nephropathy), but only in a few patients with hypospadias and cryptorchidism without other signs of Denys Drash (DDS) or wagr syndrome WT1 mutations were identified. We report a boy, who was born in 1989 with hypospadias and bilateral cryptorchidism. Previous karyotyping and endocrine studies had ruled out any known cause of male pseudohermaphroditism. Subsequently, he developed a bilateral wilms tumor, which was detected by palpation at the age of 15 months during a routine visit by the general pediatrician. Because of its extensive size, surgery and chemotherapy were needed for treatment. Analysis of the WT1 gene was performed 5 y after diagnosis and revealed a C to T transition in one allele generating a stop codon at codon 362 and subsequently leading to a truncated protein with loss of its ability to bind to dna. No signs of DDS or wagr syndrome are present in the boy. The work up of this patient and the so far known few comparable cases from the literature lead to the conclusion that in newborns with severe urogenital malformations not due to known chromosomal or endocrine disorders mutational screening of the WT1 gene should be performed, to evaluate the high risk of developing a wilms tumor. We favor mutational screening in these patients as an easy tool for investigation, because in the future it will probably decrease the necessity of frequent control visits in patients without a WT1 mutation.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
2/294. Renal adenomatosis associated with carcinoma of the lower urinary tract: a case report with immunohistochemical study.A case of renal adenomatosis of the left kidney associated with a carcinoma of the ipsilateral ureter in a 49-year-old man is examined. One hundred and eight adenomas, which were smaller than 15 mm in diameter, and a single microcarcinoma, which measured 1 mm in diameter, were found in the kidney. Further, there were more than 800 hyperplastic lesions which could be classified into three groups: (i) 792 of distal origin; (ii) 24 of proximal origin; and 10 of collecting duct origin. The serial sections obtained from 19 paraffin blocks were stained using Leu M1 as the proximal marker and epithelial membrane antigen (EMA) as the distal/collecting marker to assist in determining the origins. Ten of the small adenomas (15 lesions), which did not exceed 3 mm in diameter, were predominantly positive for EMA and five were predominantly positive for Leu M1. Further, hyperplastic lesions of distal and collecting duct origins were diffusely positive for EMA and sporadically positive for Leu M1. The lesions of proximal origin were predominantly positive for Leu M1 and sporadically positive for EMA. These findings suggest that a progression from hyperplasia and a direct transition from a single tubule to adenoma occurred multifocally in different segments of the nephrons throughout the left kidney.- - - - - - - - - - ranking = 4keywords = urinary (Clic here for more details about this article) |
3/294. myelolipoma of the renal sinus. An unusual site for a rare extra- adrenal lesion.Extra-adrenal myelolipomas are rare; approximately 36 cases have been reported to date. We document a case of myelolipoma presenting as a localized mass in the renal sinus of a 66-year-old man. The chief clinical and radiologic differential diagnostic considerations in this case included a malignant renal tumor arising in the hilum. The patient was being investigated for recurrent urinary tract infections and vague abdominal pains. Histologically, the lesion showed features characteristic of a myelolipoma. There was also marked chronic inflammation in and around the mass. The uneventful follow-up of 62 months is in keeping with the benign nature of this lesion. This report expands the possibilities of the differential diagnoses of renal hilar neoplasms, particularly in view of the increased use of imaging techniques that are bound to detect many incidental lesions in this region.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
4/294. Renal cell carcinoma presenting as a pseudo-carcinoid tumour.A 38-year-old woman presented with hypertension and a renal cell carcinoma. Raised urinary 5-hydroxyindoleacetic acid values >300 micromol/l, predictive of carcinoid tumour, were found during the perioperative period. The values returned towards normal 2 weeks post-surgery. Immunohistochemical examination of the renal cell carcinoma with neuroendocrine markers was negative. Despite a diligent search, no carcinoid tumour could be identified and the patient was well 4 years later. There was no dietary or drug explanation for this pseudocarcinoid.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
5/294. adenocarcinoma on renal allograft as a complication at 5 years.We report a new case of renal carcinoma in allograft kidney, 5 years after transplantation, which presented as a suspect allograft abscess in a complex history of recurrent urinary infection. Only surgical management with peroperative histological evaluation permitted us to confirm the diagnosis of malignancy.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
6/294. Double cancers of the kidney and ureter complicated with emphysematous pyelonephritis within the parenchyma of the renal tumour.Emphysematous pyelonephritis and double cancers of the kidney and urinary tract are rare. We describe here a case of a diabetic man who had simultaneous renal cell carcinoma of the right kidney and transitional cell carcinoma of the right ureter complicated with emphysematous pyelonephritis within the tumour parenchyma of renal cell carcinoma. Imaging and pathology are demonstrated.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
7/294. Lymphoepithelioma-like carcinoma of the renal pelvis.We report the first documented case of undifferentiated carcinoma of the renal pelvis with a prominent lymphoid stroma (lymphoepithelioma-like carcinoma [LELC]). LELCs are morphologically identical to nasopharyngeal carcinoma and are rarely seen in the urinary tract, with only isolated cases involving the urinary bladder and ureter. The tumor was composed entirely of large pale staining malignant epithelial cells with ill-defined borders arranged in syncytial sheets separated by mainly reactive lymphocytes, occasional plasma cells and histiocytes. Tumor cells were immunoreactive to cytokeratin and were negative for leukocyte common antigen. awareness of LELC is important, as it should be distinguished from lymphoma or inflammatory lesions including, xanthogranulomatous pyelonephritis.- - - - - - - - - - ranking = 2keywords = urinary (Clic here for more details about this article) |
8/294. A malignant rhabdoid tumor of the kidney occurring concurrently with a brain tumor: report of a case.Malignant rhabdoid tumor of the kidney (MRTK) is one of the most lethal neoplasms to occur in young infants. Cases of MRTK accompanying an embryonal tumor in the central nervous system have occasionally been described. We present herein an interesting case of MRTK that was clinically diagnosed preoperatively. A male infant aged 6 months with both a midline brain tumor and a renal neoplasm was transferred to our institution. Although roentgenographic evaluation suggested that the renal lesion was a Wilms' tumor, midkine (MK), a growth and differentiation factor characteristically present in the urine of patients with Wilms' tumor, was not detected. A preoperative diagnosis of MRTK was established based on the lack of urinary MK in addition to the typical clinical features of the young age and the concurrent brain tumor.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
9/294. Primary adenosquamous and squamous cell carcinoma of the upper urinary tract. Report on three cases.Three cases of adenosquamous cell carcinoma and squamous cell carcinoma of the upper urinary tract are presented. The fact that the urothelium normally has no glandular or squamous structures renders the pathogenesis of these tumours interesting. The process is assumed to begin with an urothelial metaplasia resulting from a reaction to chronic irritation, leading to dedifferentiation, dysplasias and, in the end, to a squamous cell carcinoma or adenocarcinoma. The relevant medical histories include chronic episodes of pyelonephritis or nephrolithiasis. diagnosis, therapeutic approaches and prognosis of these rare tumours are discussed.- - - - - - - - - - ranking = 5keywords = urinary (Clic here for more details about this article) |
10/294. renal artery stenosis and pheochromocytoma: coexistence and treatment.PURPOSE: The aim of this study was to develop an optimal treatment plan for coexisting pheochromocytoma and renal artery stenosis. methods: A retrospective analysis of the evaluation and management of a hypertensive 14-year-old boy was conducted. RESULTS: Secondary causes of hypertension were excluded initially including intracranial, cardiac, and endocrine abnormalities. A renal scan showed right renal function at 39%. angiography confirmed a 90% subsegmental stenosis, as well as noting a blush suggesting a tumor. A computerized tomography (CT) scan of the abdomen showed a right adrenal mass. serum epinephrine was 3,006 pg/mL (normal, <1,009 pg/mL) and 24-hour urinary norepinephrine was 2,001 microg (normal, <105 microg/24 h), suggesting the presence of a pheochromocytoma. During the operation for resection, a right subsegmental renal artery was found to be draped around a renal hilar mass; the adrenal gland was normal. The tumor was resected, and pathology confirmed a pheochromocytoma. Postoperatively, the patient had some mild hypertension that gradually resolved. CONCLUSIONS: Extraadrenal pheochromocytomas (paragangliomas) occur more frequently in children and are most commonly located in the renal hilum. In this location, they may present initially as renal artery stenosis as a result of direct arterial compression, fibrous bands, or catecholamine-induced vasospasm. Our experience supports tumor resection of extraadrenal pheochromocytomas as the definitive treatment for both conditions.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
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