Renal tumors in children aged 10-16 Years: a report from the United Kingdom Children's Cancer and Leukaemia Group.

Wilms tumor is the most common renal tumor of childhood. However, other epithelial, mesenchymal, and neuroectodermal neoplasms may also arise in the kidney during childhood, several of which show specific age distributions; in the 1st year of life, mesoblastic nephroma and rhabdoid tumor are more common, whereas renal cell carcinoma, primitive neuroectodermal tumor, and anaplastic Wilms tumors are relatively more frequent in older children and adolescents. The aim of this study is to describe the spectrum of renal tumors in children aged 10-16 years using data from 1492 patients registered in the UK Wilms Tumour 3 Trial (1991-2001) and International Society of Paediatric Oncology Wilms Tumour Trial 2001 (2002-2008) clinical trials of renal tumors in childhood.

Sarcomatoid chromophobe renal cell carcinoma: Cytohistopathological correlation of a case.

Sarcomatoid renal cell carcinomas of the kidney are rare neoplasms constituting about 1-5% of all renal malignant neoplasms. These are aggressive tumors and are commonly associated with conventional (clear cell) renal cell carcinomas, but cases associated with chromophobe renal cell carcinomas are sparse. Cytological features of such lesions have rarely been reported.

Anaplastic variant of clear cell sarcoma of the kidney: a rare case report.

Clear cell sarcoma (CCSK) of the kidney is an uncommon but distinctive pediatric renal tumor with a characteristic histological pattern and marked propensity for bone metastasis. The rare anaplastic variant constitutes about 3% of cases of CCSK and carries an unfavorable prognosis, with increased tumor recurrence and resistant to chemotherapy. This variant show high frequency of p53 gene mutation and p53 over expression in comparison to the usual CCSK.

Pediatric non-Wilms renal tumors: subtypes, survival, and prognostic indicators.

Background: To determine the outcomes and predictors of survival for pediatric non-Wilms renal tumors (NWRT).

Methods: The SEER database (1973-2005) was queried for all patients < 20 y of age.

Results: Overall, 349 cases of NWRT were identified.

[Clear-cell sarcoma of the kidney: about a paediatric case].

Clear-cell sarcoma of the kidney (CCSK) is a rare malignant tumor of childhood, known for its aggressiveness, its tendency to recurrence and to metastasis to bone. We report an observation of a child of 48 months carrying a large abdominal mass. The diagnosis of the SCCR was made on biopsy, since imaging remained uncertain as to the renal origin of the mass.

Clear cell sarcoma of the kidney in children: experience in a developing country.

Introduction: Clear cell sarcoma of the kidney (CCSK) is a rare tumour comprising 4% of primary renal tumours in children. It has a unique constellation of chromosomal and molecular features and should no longer be viewed as an unfavourable histological variant of Wilms tumour. Little is known of its clinical presentation and pathological profile in children living in a developing country.

Vascular characterization of clear cell sarcoma of the kidney in a child: a case report and review.

Clear cell sarcoma of the kidney (CCSK) is uncommon pediatric renal tumor and can present a significant therapeutic challenge in those patients whose tumors spread beyond the kidney. Thus, identifying potential novel targets for treatment may be clinically important. Clear cell sarcoma of the kidney is characterized by a unique vascular pattern, in which nests of tumor cells are separated by regularly-spaced, fine fibrovascular septa.

Outcome of pediatric renal tumor treated using the Japan Wilms Tumor Study-1 (JWiTS-1) protocol: a report from the JWiTS group.

Purpose: In 1996, the Japan Wilms Tumor Study (JWiTS) group was founded to elucidate the efficacy and safety of the regimen established by the National Wilms Tumor Study (NWTS) group in the USA, and a multicenter cooperative study (JWiTS-1) was started in Japan. This report reviews the results of JWiTS-1.

Methods: A total of 307 patients with malignant renal tumor were enrolled in the JWiTS-1 study between 1996 and 2005.

The cytological diagnosis of paediatric renal tumours.

Fine needle aspiration cytology (FNAC) is used for preoperative diagnosis of paediatric renal tumours, especially in centres where preoperative chemotherapy is advocated in Wilms' tumour. This review focuses on salient cytological features in specific paediatric renal tumours, the approach to resolving a differential diagnosis and the role of ancillary methods in diagnosis of paediatric renal tumours. Crucial differential diagnoses include distinguishing: Wilms' tumour from benign tumours in the kidney like multicystic nephroma or congenital mesoblastic nephroma; aggressive non-Wilms' tumours of kidney like rhabdoid tumour of kidney; and Wilms' tumour from other paediatric round cell sarcomas like neuroblastoma, non-Hodgkin lymphoma etc.

Metanephric stromal tumor: a novel pediatric renal neoplasm.

Metanephric stromal tumor of kidney is a novel pediatric benign stromal specific renal neoplasm. A few cases have been reported in adults also. This tumor is usually centered in the renal medulla with a characteristic microscopic appearance which differentiates this lesion from congenital mesoblastic nephroma and clear cell sarcoma of the kidney.

Diagnostic utility of nestin expression in pediatric tumors in the region of the kidney.

Nestin is an intermediate filament that was first identified in neuroepithelial stem cells. During embryogenesis, nestin is expressed in a number of cell types, including neural crest cells and developing myocytes. We have recently shown that nestin is expressed in human podocytes and nephrogenic blastema.

Intracranial metastasis from synovial sarcoma.

Synovial sarcomas comprise between 5% to 10% of all soft tissue sarcomas in adults. It has a predilection for young adults and is one of the more frequently misdiagnosed soft tissue sarcomas. It is not unusual for synovial sarcoma to present initially after a traumatic event.

Paediatric renal tumours: recent developments, new entities and pathological features.

Paediatric renal tumours represent a relatively common group of childhood solid neoplasms, in which both diagnosis and treatment are highly dependent on the histopathological findings. In addition to Wilms' tumour (nephroblastoma), a number of specific distinct entities are now reported, including (congenital) mesoblastic nephroma, clear cell sarcoma of the kidney, rhabdoid tumour of the kidney, specific paediatric variants of renal cell carcinoma, and others such as renal primitive neuroectodermal tumour and desmoplastic small round cell tumour. Recent advances in both molecular biological findings and immunohistochemistry allow reliable diagnosis of most of these entities even on the basis of small needle biopsy specimens.

[Clear-cell sarcoma of the kidney. Two pediatric cases].

Clear-cell sarcoma of the kidney is a rare pediatric renal sarcoma with poor prognosis and propensity to metastasize to bone. It is a distinctive renal malignancy regarded as a morphologic feature of Wilms' tumor. We report two cases occurring in four-year and 12-month-old boys, explored for abdominal masses.

[Radiologic findings in non-Wilms' renal tumors in children].

Objectives: To analyze the radiologic findings of non-Wilms' renal tumors in children.

Material And Methods: This is a retrospective study of 26 patients with histologically confirmed non-Wilms' renal tumors. The following variables were evaluated on radiological studies: location, size, margins, necrosis, hemorrhage, calcifications, subcapsular hematoma, adenopathies, and metastases.

c-KIT overexpression, without gene amplification and mutation, in paediatric renal tumours.

Aims: To investigate the presence and prognostic relevance of KIT expression in paediatric renal tumours, and to determine whether receptor overexpression is associated with gene amplification and/or mutation.

Methods: Immunohistochemistry without antigen retrieval for CD117 was carried out on tissue microarrays consisting of 274 Wilms' tumours, 13 clear cell sarcomas of the kidney (CCSK), 10 mesoblastic nephromas (MN), and 7 rhabdoid tumours of the kidney (RTK). In addition, gene copy number was investigated by chromogenic in situ hybridisation (CISH), and overexpressing tumours were sequenced for KIT mutations in exons 9, 11, 13 and 17.

Clear cell sarcoma of the kidney--a study of seven cases over a period of three years.

Unlabelled: Clear cell sarcoma of the kidney (CCSK) can display diverse morphological patterns and mimic various other pediatric renal tumors. An accurate diagnosis of this tumour is important considering the therapeutic and prognostic implications.

Aim: The present study was undertaken to describe the various histological patterns of CCSK.

Clear cell sarcoma, cellular mesoblastic nephroma and metanephric adenoma: cytological features and differential diagnosis with Wilms tumour.

Wilms' Tumour (WT) is the most common kidney tumour in childhood, this fact and the embryonic complexity of WT create, whenever one of its three classical components predominates in cytologic smears, difficulties in the differential diagnoses with other less common entities. In the present study, we review the cytological and immunohistochemical characteristics of three children renal tumours, a Clear Cell Sarcoma of the Kidney (CCSK-case1), a Cellular Mesoblastic Nephroma (CMN-case2) and a Metanephric Adenoma (MA-case3) and compare them, for differential diagnostic purposes, with smears of blastematous, mesenchymal and epithelial predominant WTs, previously diagnosed in our Department. In all cases a mass was detected in the abdomen (2 and 8 year old children-cases 1 and 3, respectively), and pre-birth in case 2 (the tumour was detected during pregnancy).

Multifaceted dysregulation of the epidermal growth factor receptor pathway in clear cell sarcoma of the kidney.

Purpose: Epidermal growth factor receptor (EGFR) is a receptor tyrosine kinase overexpressed in a variety of human malignancies, against which targeted therapies have shown efficacy in lung and brain tumors. Clinical responses to EGFR inhibitors have been found to be highly dependent on the presence of activating mutations, whereas gene amplification, downstream activation of Akt, and abnormalities in PTEN are also reported predictive factors. We sought to evaluate these variables in pediatric renal tumors.

Recurring translocation (10;17) and deletion (14q) in clear cell sarcoma of the kidney.

Context: Clear cell sarcoma of the kidney (CCSK) is a prognostically unfavorable renal neoplasm of childhood. Previous cytogenetic studies of CCSK have reported balanced translocations t(10;17)(q22;p13) and t(10;17)(q11;p12). Although the tumor suppressor gene p53 is located at the chromosome 17p13 breakpoint, p53 abnormalities are rarely present in these tumors.

Bone metastases of clear cell sarcoma of the kidney diagnosed by fine needle aspiration cytology. A case report.

Background: Clear cell sarcoma of the kidney (CCSK) was first recognized from its predilection to metastasize to bone. While there are many case reports in documenting the diagnosis of CCSK by fine needle aspiration cytology (FNAC), this is the first case in which FNAC was instrumental in diagnosing the condition from a metastatic site.

Case: A 2-year-old presented with multiple bone lesions.

Treatment of recurrent clear cell sarcoma of the kidney with brain metastasis.

Background: Clear cell sarcoma of the kidney (CCSK) is known for its propensity to metastasize to bone, but it also spreads to other sites including the brain. This study was undertaken to describe the treatment and outcomes of patients with recurrent CCSK involving the brain.

Methods: A retrospective records review was conducted on eight patients with CCSK who developed brain metastases after complete responses to initial therapy.

Association between the HER2 expression and histological differentiation in Wilms tumor.

Human epidermal growth factor receptors (HER) play a critical role in the branching morphogenesis of renal tubules. In the current study, we analyzed the expression of HER2 in Wilms tumor and assessed the role of this gene in the tumorgenesis of Wilms tumor. During the period from 1960 to 2005, 40 patients with Wilms tumor were treated in our department.