Cases reported "Kidney Neoplasms"

Filter by keywords:



Filtering documents. Please wait...

1/267. 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 = 1
keywords = formation
(Clic here for more details about this article)

2/267. Renal cell carcinoma in children with diffuse cystic hyperplasia of the kidneys.

    We report the clinical, pathologic, and genetic features of renal malignancy in two children with diffuse cystic hyperplasia. Both presented with massive bilateral nephromegaly. Neither had a family history or clinical findings suggestive of tuberous sclerosis or von hippel-lindau disease. The kidneys of both children were extensively replaced by tubulocystic hyperplasia with large eosinophilic epithelial cells. The masses of hyperplastic tissue were nodular, compressing remnants of uninvolved renal parenchyma. Tubulopapillary carcinoma was present in both children, one of whom had bilateral multicentric carcinoma. No loss of heterozygosity was detected in the tumors at the TSC1, TSC2, or VHL gene regions, and no alterations in the VHL gene were detected using single-strand conformation polymorphism analysis. These cases of bilateral renal enlargement with diffuse cystic hyperplasia appear to represent a new clinical syndrome that may warrant bilateral nephrectomy because of the risk of malignancy.
- - - - - - - - - -
ranking = 0.5
keywords = formation
(Clic here for more details about this article)

3/267. Congenital mesoblastic nephroma (CMN) with an unusual immunohistochemical feature.

    OBJECTIVES: To describe a case of congenital mesoblastic nephroma (CMN) treated by radical nephrectomy with no evidence of relapses after five years in spite of an unusual positivity for proliferating cellular nuclear antigen (PCNA). methods: A three-month-old child presented a right renal mass with compression of the inferior vena cava. Excretory urography showed an intrarenal mass with distortion of the calyceal system. There was no evidence of metastasis. Radical nephrectomy was carried out; no adjuvant therapy was given. Histological and immunohistochemical studies were performed. RESULTS: The tumor was a 6 x 6 x 5 cm solitary mass extending into perirenal tissue, involving the hilar vessels but not the ureter. Histologically, it has been classified as a congenital mesoblastic nephroma of the classic variant. Positive reaction for vimentin and actin was observed. Strong positivity for PCNA and negativity for P53 were revealed. CONCLUSIONS: PCNA is considered to be a reliable marker of potential malignancy. This, however, contrasts with the biological behavior of our case. Further evaluation is required for correct interpretation of this additional information and to avoid inappropriate aggressive therapy.
- - - - - - - - - -
ranking = 0.5
keywords = formation
(Clic here for more details about this article)

4/267. Syndrome of microcephaly, Dandy-Walker malformation, and wilms tumor caused by mosaic variegated aneuploidy with premature centromere division (PCD): report of a new case and review of the literature.

    We report a male infant with multiple congenital anomalies and mosaic variegated aneuploidy; a rare cytogenetic abnormality characterized by mosaicism for several different aneuploidies involving many different chromosomes. He had prenatal-onset growth retardation, microcephaly, dysmorphic face, seizures, hypotonia, feeding difficulty, and developmental delay. In addition, he developed bilateral Wilms tumors. Neuroradiological examination revealed Dandy-Walker malformation and hypoplasia of the cerebral hemisphere and pons. cytogenetic analysis revealed various multiple numerical aneuploidies in blood lymphocytes, fibroblasts, and bone marrow cells, together with premature centromere division (PCD). Peripheral blood chromosome analysis from his parents also showed PCD, but no aneuploid cells. The clinical phenotype and multiple aneuploidies of the patient may be a consequence of the homozygous PCD trait inherited from his parents. Comparison with previously reported cases of multiple aneuploidy suggests that mosaic variegated aneuploidy with PCD may be a clinically recognizable syndrome with major phenotypes being mental retardation, microcephaly, structural brain anomalies (including Dandy-Walker malformation), and possible cancer predisposition.
- - - - - - - - - -
ranking = 3
keywords = formation
(Clic here for more details about this article)

5/267. Mesoblastic nephroma in adulthood: a case report.

    BACKGROUND: Mesoblastic nephroma is an uncommon renal tumor of infancy and rarely occurs in adults. We report an adult case of mesoblastic nephroma. methods: A 50-year-old woman was found incidentally to have a right renal mass by abdominal ultrasonography. Computed tomography and magnetic resonance imaging revealed a heterogeneous tumor and angiography showed a mixture of hypervascularity and hypovascularity. Right radical nephrectomy was performed. RESULTS: The tumor was an encapsulated yellowish solid mass. Microscopically, the tumor was composed of spindle cell proliferation. Atypia and mitoses were not identified. Among the tumor cells, there were tubular arranged epithelial elements. CONCLUSION: The patient was free of recurrence 14 months postoperatively. Mesoblastic nephroma is classified as a benign tumor but recurrence and malignant formation of this tumor have been reported so regular postoperative follow up is required.
- - - - - - - - - -
ranking = 0.5
keywords = formation
(Clic here for more details about this article)

6/267. Renal cell carcinoma with massive osseous metaplasia and bone marrow elements.

    Focal calcifications are frequently seen in renal masses and may be present in renal cell carcinomas. Metaplastic bone formation, on the other hand, is a rare event. We report a unique case of a large calcified renal cell carcinoma with massive osseous metaplasia and bone marrow elements. The clinical and pathologic differential diagnosis for this tumor is discussed along with a review of the literature on this unusual phenomenon.
- - - - - - - - - -
ranking = 0.5
keywords = formation
(Clic here for more details about this article)

7/267. Clear cell sarcoma of kidney in an adolescent and in young adults: a report of four cases with ultrastructural, immunohistochemical, and dna flow cytometric analysis.

    Clear cell sarcoma of the kidney is a distinct, highly malignant pediatric neoplasm. Its occurrence in adults is extremely rare and the subject of isolated case reports. We present a series of four cases (three males and one female) identified in an adolescent and in young adults (16, 18, 20, and 25 years) with flank mass (three cases), hematuria (two cases), flank pain (two cases), and hypertension (one case). Three patients had stage III disease and one had stage I disease (National Wilms' Tumor Study staging system). All tumors had predominantly or exclusively the classic histology of a monotonous proliferation of uniform small round cells with evenly distributed fine chromatin, although focal microcyst formation (two cases) and spindled architecture (one case) (variant patterns) were also noted. Therapy in all cases consisted of surgery and chemotherapy with or without radiation. Follow-up data (29-202 months) showed distant metastases in all four cases, including the lung (four cases), bone (two cases), and the liver (two cases). Three patients died of disease at 29, 59, and 63 months (mean, 50.3 months), and one patient is alive with no evidence of disease at 202 months. Ultrastructural features included scattered primitive junctions, short and irregular cytoplasmic extensions, and scant to a moderate amount of mitochondria. Immunohistochemical study (three cases) showed immunoreactivity with vimentin (two cases) and no reaction with cytokeratin, epithelial membrane antigen, S-100 protein, or desmin. Flow cytometric analysis showed diploid dna content in three primary tumors and tetraploidy in one metastatic tumor. The proliferative activity (S-phase fraction) was low to intermediate (mean, 9.8%). Our data suggest that clear cell sarcoma of the kidney in the young adult age group resembles its pediatric counterpart in ultrastructural and immunohistochemical characteristics, proclivity for skeletal and visceral metastasis, dna diploid status with relatively low S-phase, and aggressive clinical course. Clear cell sarcoma of the kidney in adult patients, although rare, must be differentiated from sarcomatoid carcinoma, sarcomas, and round cell tumors because of its unique characteristics in comparison to other renal neoplasms.
- - - - - - - - - -
ranking = 0.5
keywords = formation
(Clic here for more details about this article)

8/267. 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.
- - - - - - - - - -
ranking = 25.084989349112
keywords = rosette
(Clic here for more details about this article)

9/267. Unusual chromosome patterns of renal cell carcinomas common to two brothers.

    In this study, we describe two renal cell carcinomas (RCC) that occurred at the same time in two brothers, yielding information on the carcinogenic process. We used flow cytometry (FCM) to evaluate nuclear dna content, and performed cytogenetic analysis. We also carried out fluorescence in situ hybridization (FISH) with a panel of centromeric probes for chromosomes 3, 7, 8, 9, 12, 17, 20, and Y in interphase cells. flow cytometry analysis revealed diploid histograms in the tumor and "nonmalignant" samples of patient 1, while an aneuploid cell subpopulation was found in the tumor and "nonmalignant" samples of patient 2. Tumor samples from the two brothers were studied by FISH, and had common numerical chromosome aberrations: trisomy of chromosomes 3 and 7, and monosomy and trisomy of chromosomes 9 and 17. Moreover, in normal samples from both brothers, we found monosomy 9, and in a normal sample from patient 1, monosomy 17. cytogenetic analysis revealed trisomy 3 in some cells grown from normal kidney tissue of each brother. The identification of the same chromosome alterations in both brothers appears to provide evidence of an unusual process of carcinogenesis, probably due to a common genetic basis.
- - - - - - - - - -
ranking = 0.5
keywords = formation
(Clic here for more details about this article)

10/267. Solitary metachronous splenic metastases: an evaluation of surgical treatment.

    Splenic metastases occurring after primary tumor removal and apparently solitary have been documented only recently in literature. They are, most of the times, clinically asymptomatic and their presence is casually determined by ultrasonographic follow-up in subjects otherwise in good conditions. The belief that splenic metastases occur only in disseminated cancer is today no longer accepted. Some Authors consider solitary splenic metachronous metastases eligible for surgical treatment as well as pulmonary or hepatic metastases. In the case presented, surgery was required due to abscess formation of a splenic metastasis, which was not responding to chemotherapy. Our experience, like others reported in literature, verified a long-term post-operative survival in spite of limited disease-free time. Surgical treatment by splenectomy can be indicated in selected patients, considering that chemotherapy has been proved to be ineffective in the treatment of splenic metastases.
- - - - - - - - - -
ranking = 0.5
keywords = formation
(Clic here for more details about this article)
| Next ->


Leave a message about 'Kidney Neoplasms'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.