Cases reported "Wilms Tumor"

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1/284. Subcapsular hemorrhage of adult Wilms' tumor.

    Wilms' tumor is a rare malignancy of kidney in adults. It usually cannot be differentiated from other renal masses preoperatively. This is a presentation of a case of adult Wilms' tumor who developed spontaneous hemorrhage during radiological evaluation. As tumor rupture can change the stage of the tumor and alter the prognosis of the patient, urgent work-up and treatment is advised.
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2/284. Extrarenal Wilms' tumor of the uterus.

    Wilms' tumor (nephroblastoma) is rare in adults. Its occurrence as a primary tumor outside the kidney is exceptional. Only 4 cases [1-4] have occurred in the uterus. This report adds the fifth instance of primary uterine Wilms' tumor. The patient, a 77-year-old woman (G2P2), is the oldest ever described with a Wilms' tumor.
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3/284. Spectrum of cystic variants of Wilm's tumour: cystic nephroma (multilocular cyst) and cystic partially differentiated nephroma--a report of four cases.

    Two cases of cystic nephroma (multilocular cyst of the kidney), and one case each of cystic partially differentiated nephroblastoma (CPDN) and rhabdomyomatous Wilms' tumour are described. All were male and in the pediatric age group. Grossly tumours were unilateral, unicentric and multiloculated. The need for proper designation of these lesions is highlighted because of difference in the treatment and prognosis of these tumours.
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4/284. Bilateral renal cancer in children: a difficult, challenging and changing management problem.

    Bilateral disease occurs in 6% of patients with Wilms' tumour. Bilateral renal involvement is present in 25%-50% of children with B-cell non-Hodgkin's lymphoma. Paediatric oncologists therefore encounter bilateral renal disease relatively frequently. A patient with bilateral Wilms' tumour is presented, in whom at least 16 synchronous tumours in the kidneys were treated successfully by primary chemotherapy and 'nephron-sparing' surgery, without renal radiotherapy. We believe the successful treatment without radiotherapy will allow greater potential for normal growth in the future. The case history of a patient with renal failure due to the infiltration of both kidneys by lymphoma and who was treated successfully by chemotherapy, is also presented. One kidney has completely returned to normal function and growth; the other has completely failed and almost disappeared, demonstrating complete reversibility of the damage up to some critical point, beyond which, failure and atrophy result. These two case histories demonstrate extreme examples of clinical dilemmas involving bilateral renal tumours and allow discussion of modern management aimed at preserving renal function.
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5/284. Intracranial metastasis of Wilms' tumor involving the tectal plate without pulmonary involvement. Case report.

    Intracranial metastasis without pulmonary involvement of Wilms' tumor is very rare, and most previously reported metastatic sites have been in the cerebral parenchyma. We experienced a rare case of metastasis of Wilms' tumor in the tectal plate without pulmonary involvement. A 3-month-old boy was admitted to our hospital due to hydrocephalus. After insertion of a ventriculoperitoneal shunt, there were no neurological deficits. Five months after the operation, a mass 5 cm in diameter extending from the tectal plate into the third ventricle was found. A mass 10 cm in diameter within the retroperitoneal space, which occupied the right kidney, was also found. Both tumors were resected and diagnosed histologically as Wilms' tumor. Despite every effort including chemotherapy and radiotherapy, the patient died at 14 months old. The majority of children with hydrocephalus showing thickening of the tectal plate would most likely have tectal glioma; however, this unusual case report reminds us that metastasis of Wilms' tumor can occur in the tectal plate causing hydrocephalus.
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6/284. Transcriptional regulation of PDGF-A and TGF-beta by KTS WT1 deletion mutants and a mutant mimicking denys-drash syndrome.

    denys-drash syndrome (DDS) and frasier syndrome (FS) are rare diseases caused by the mutations of wilms tumor gene, WT1. The common denominator in these syndromes is a nephropathy which is manifested by early-onset proteinuria, nephrotic syndrome and end stage renal failure. Although these syndromes are genetic models of nephropathy and the mutations of WT1 gene are characterized in these patients the mechanism how mutations of WT1 gene affect the embryonic kidney adversely has not been elucidated. Recently, there was a report that FS is caused by mutations in the donor splice site of WT1. These mutations predicted loss of KTS isoform, which is one of the four splicing variants of WT1. In this study, two KTS deletion mutants of WT1 were made as well as a WT1 mutant mimicking a mutation found in a patient who had diffuse mesangial sclerosis, end stage renal failure and wilms tumor. Mutant embryonic kidney cell lines were established by transfection of 293 embryonic kidney cells with WT1 mutants. We investigated the transcription regulation of mutant WT1 among these cell lines using the reporter vectors containing PDGF-A and TGF-beta promoter sequence. Our results showed that the promoter activity of PDGF-A and TGF-beta, which are related to the progression of glomerular diseases, was modestly increased in the mutant cell mimicking the patent, while those activities were markedly increased in other two deletion mutant cell lines. This study demonstrated that KTS WT1 mutation found in DDS affected the cytokine expression adversely in vitro. From these results, we suggest that the alteration of KTS WT1 expression be responsible for the rapid progression of renal diseases in DDS and FS.
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7/284. Clinicopathologic and cytologic features of a metanephric adenoma of the kidney: a case report.

    Metanephric adenoma is a recently described rare and benign renal neoplasm. Our patient, a 37-year-old woman, suffered from flank pain for five months and was found to have a renal mass. Ultrasound, computerized tomography and angiography findings were consistent with a hypovascular renal cyst. Wilms' tumor was the initial misdiagnosis, based on needle biopsy and aspiration cytology. A radical nephrectomy was performed. Histologically, the tumor was well defined and was composed of uniform small cells arranged in a solid, tubular or rosette-like pattern. The prognosis is good for metanephric adenoma. The tumor was first considered a benign counterpart of papillary carcinoma or Wilms' tumor; however, recent cytogenetic evidence suggested that the tumor might be related to papillary adenoma and papillary renal cell carcinoma. The clinical, radiologic, histologic and cytologic features presented here should help to promote the correct preoperative diagnosis and to avoid unnecessary aggressive treatment.
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8/284. Botryoid wilms tumor: case report and review of literature.

    A rare case of botryoid wilms tumor is presented. The main clinical manifestations were persistent low-grade fever, malaise, and proteinuria associated with microhematuria. ultrasonography revealed an echogenic mass in the right kidney, and a contrast-enhanced mass was found in the dilated collecting system by contrast-enhanced computed tomography. The surgically resected tumor was a polypoid, light-yellow, glistening mass that occupied a large part of the renal pelvis and originated from the pelvicaliceal wall. Part of the tumor extended to the proximal ureter, resulting in hydronephrosis in the involved kidney. No parenchymal lesion was observed. Microscopic examination revealed epithelial, stromal, and blastemal components, which indicated wilms tumor. Infection had occurred in the hydronephrotic kidney, which presumably had caused the major presenting symptoms. The prognosis of our patient and previously reported cases of botryoid wilms tumor was good compared with that of typical wilms tumor, since the botryoid type can be detected at an early stage.
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9/284. 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.
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10/284. wilms tumor in a child with trisomy 13.

    A 4-year-old black boy with trisomy 13, a history of frequent urinary tract infections, and a horseshoe kidney with painless gross hematuria was examined. An abdominal mass was detected and surgically resected. Examination of the surgical specimen revealed a wilms tumor. Given the concurrence of trisomy 13 and wilms tumor and the presence of another such case in the literature, there may be just cause to suspect a locus on chromosome 13 that affects the probability of developing wilms tumor. Given the increasingly longer survival of patients with trisomy 13, clinicians may need to be aware of the possibility of renal malignant disease in this population of patients.
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