1/14. Severe intrarenal fibrosis, infundibular stenosis, renal cysts, and persistent perilobar nephrogenic rests in a patient with beckwith-wiedemann syndrome 27 years after diffuse nephroblastomatosis and wilms tumor: natural progression or a consequence of treatment?A27-year-old woman presented with back and abdominal pain. She was diagnosed in infancy with beckwith-wiedemann syndrome and bilateral multifocal perilobar nephrogenic rests that progressed to diffuse nephroblastomatosis with neoplastic nephroblastomatous rests at 14 months of age and subsequently to a right wilms tumor at 5 years of age. Computed tomography of the abdomen during the current admission showed multiple obstructed calices. Ureteroscopic inspection of the left kidney revealed severe intrarenal scarring with multiple infundibular stenosis, hydrocalices, and nephrocalcinosis. Renal biopsy showed sclerotic glomeruli with calcification and scarring and persistent subcapsular nodular renal blastema. Electrocautery incision and balloon dilatation provided temporary pain relief. After discharge, the patient has had two or three episodes of recurrent pain associated with new areas of infundibular stenoses and renal cysts. Bilateral nephrectomy and renal transplantation is being considered for management of progressive disease and relief of intractable pain. The potential causes of progressive and severe intrarenal fibrosis, infundibular stenosis and nephrocalcinosis, and renal cysts in this patient may include abnormal renal development secondary to beckwith-wiedemann syndrome itself, radiation or chemotherapy damage, or a combination.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
2/14. Case report: simple renal cyst in child.A case of a large renal cyst of the right kidney suggesting a nephroblastoma is reported. Preoperative differentiation was diffucult.- - - - - - - - - - ranking = 0.16666666666667keywords = nephroblastoma (Clic here for more details about this article) |
3/14. Ischiospinal dysostosis with cystic kidney disease: report of two cases.Ischiospinal dysostosis (ISD) is a recently described entity characterized by ischial hypoplasia and spinal segmental anomalies. Nephroblastomatosis that may manifest radiologically as 'polycystic kidney disease' has been regarded as a syndromic constituent of ISD. We report two children with ISD associated with 'renal cystic disease'. One child had multiple renal cysts resembling radiologically adult polycystic kidney disease, leading to renal dysfunction, whereas the other a solitary renal cyst with normal renal function. Renal malformations in ISD may be variable, ranging from diffuse nephroblastomatosis to a solitary renal cyst.- - - - - - - - - - ranking = 0.16666666666667keywords = nephroblastoma (Clic here for more details about this article) |
4/14. adult Wilms' tumor mimicking hemorrhagic renal cyst.A 36-year-old man presented with macroscopic hematuria associated with right flank pain. Examination of the patient revealed a cystic mass in the right kidney. Because the mass had increased in size, enucleation of the mass was performed. Histopathological findings revealed nephroblastoma, therefore, radical nephrectomy was performed. We believe the pathogenesis of the cystic formation to be a process in which a tumor that had developed in the pericalyceal region spontaneously ruptured, exuding urine into the perinephric space, forming a cystic mass. The patient is alive with no evidence of disease 24 months after the operation.- - - - - - - - - - ranking = 0.16666666666667keywords = nephroblastoma (Clic here for more details about this article) |
5/14. Nephron-sparing surgery for multilocular cyst of the kidney in a child.Multilocular cyst of the kidney is an uncommon benign renal neoplasm. Because of its benign nature, this lesion is best managed by nephron-sparing surgery. This report describes a child who underwent a partial nephrectomy for a multilocular cyst of the kidney. After reviewing other cases, we discuss the clinical characteristics of multilocular cyst of the kidney and emphasize the cystic partially differentiated nephroblastoma as a differential diagnosis.- - - - - - - - - - ranking = 0.16666666666667keywords = nephroblastoma (Clic here for more details about this article) |
6/14. Cystic partially differentiated nephroblastoma.Cystic partially differentiated nephroblastoma is a relatively rare tumour of the kidney usually affecting infants. Cystic Wilms' tumour and multilocular cystic nephroma should be distinguished from cystic partially differentiated nephroblastoma. Multilocular cystic nephroma is a benign tumour whereas cystic Wilms' tumour is at the malignant end of the range of classification of such tumours. Cystic partially differentiated nephroblastoma may undergo local recurrence but there is no report of metastasis.- - - - - - - - - - ranking = 1.1666666666667keywords = nephroblastoma (Clic here for more details about this article) |
7/14. Nephroblastoma occurring in a multilocular cystic kidney.A case of a nephroblastoma in a multilocular cystic kidney in a 5-year-old child is reported and the related literature is reviewed. Because of the unreliability of preoperative and intraoperative diagnostic modalities in identifying a coincidental nephroblastoma nephrectomy is recommended for multilocular cystic disease of the kidney in children.- - - - - - - - - - ranking = 0.33333333333333keywords = nephroblastoma (Clic here for more details about this article) |
8/14. Multilocular renal cyst. Case report, ultrastructure and review of the literature.Multilocular renal cyst is an uncommon focal, unilateral, cystic, epithelial lesion of uncertain pathogenesis. Because nephroblastomatous foci have been found on microscopic examination of several of the reported cases, some authors have proposed that multilocular cysts are differentiated and cystic forms of Wilms' tumour. This proposition is analysed and it is concluded that three possibly interrelated lesions may be defined: (1) Wilms' tumours showing cystic differentiation. (2) Lesions macroscopically indistinguishable from multilocular cyst containing variably differentiated nephroblastomatous foci. All such cases have been described in infants. (3) Typical multilocular renal cysts, which have described in children and adults. review of the literature shows that several cases have been included in the second category solely because of the presence of small intraseptal tubules. Consequent anomalies of interpretation are highlighted and a further case of multilocular cyst in an adult female is reported in which ultrastructural examination, recorded for the first time, confirms the epithelial nature of the cyst lining cells which resemble simplified renal tubular epithelium.- - - - - - - - - - ranking = 0.33333333333333keywords = nephroblastoma (Clic here for more details about this article) |
9/14. Cystic partially differentiated nephroblastoma and polycystic wilms tumor: a spectrum of related clinical and pathologic entities.Cystic partially differentiated nephroblastoma is a rare neoplasm occurring in young children and demonstrating features of classic nephroblastoma (wilms tumor) and multilocular cysts. Cystic partially differentiated nephroblastoma actually represents a spectrum of cystic renal tumors in which varying amounts of blastema, stroma and epithelial structures are present. Some of these lesions should, perhaps, be classified more accurately as polycystic nephroblastoma. The clinical and pathologic findings, and management of 2 infants with cystic partially differentiated nephroblastoma are presented. The controversy over the histogenesis of these lesions and whether they should be considered benign or potentially malignant is discussed.- - - - - - - - - - ranking = 1.5keywords = nephroblastoma (Clic here for more details about this article) |
10/14. Multilocular renal cyst: electron microscopic evidence of pathogenesis.Two cases of multilocular renal cyst (MRC) are presented with electron microscopic findings. The epithelial cells of the cysts resembled renal collecting tubule cells and not convoluted tubule or Henle's loop cells. Intercystic areas contained only connective tissue elements. Simple MRC appears to be a congenital malformation that results from segmental maldevelopment of the ureteric bud. In some cases of MRC, the intercystic septa contain immature renal elements. The lesion is then called cystic poorly differentiated nephroblastoma (CPDN). However, in both MRC and CPDN, local recurrence or metastases have not been described. Treatment of these lesions should consist of nephrectomy, careful histologic evaluation to rule out obvious foci of Wilms' tumor, and conservative follow-up.- - - - - - - - - - ranking = 0.16666666666667keywords = nephroblastoma (Clic here for more details about this article) |
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