Clear cell sarcoma of the kidney in a child with Fanconi anemia.

Patients with Fanconi anemia subgroup D1, attributable to biallelic mutations in BRCA2, have an increased risk of solid tumors. Tumors in the kidneys of these patients are almost exclusively Wilms tumor. We report the first recorded case, to our knowledge, of a Clear Cell Sarcoma of the Kidney in a patient with this cancer predisposition syndrome.

Clear cell sarcoma of the kidney demonstrates an embryonic signature indicative of a primitive nephrogenic origin.

Clear cell sarcoma of the kidney (CCSK) is a tumor affecting children with a median age of 3 years at diagnosis. The cell of origin of CCSK is unknown and data on the molecular changes giving rise to CCSK is scarce. This has hindered the identification of positive diagnostic markers and development of molecularly targeted treatment protocols for CCSK.

Stage I clear cell sarcoma of the kidney: is it the time for a less intensive adjuvant treatment?

Clear cell sarcoma of the kidney (CCSK) is a rare type of renal tumor, comprising 2% to 5% of all primary renal tumors in children. Despite the label of "unfavorable" tumor, with recent multimodality treatment schedules, including radiotherapy and multi-agent chemotherapy, disease free survival rates approaching 80% can be achieved. Younger age at tumor diagnosis and advanced-stage disease represent adverse prognostic factors.

Incidental capture of rarely diagnosed pediatric tumor: an infant boy with clear cell sarcoma of the kidney.

Clear cell sarcoma of the kidney (CCSK) is an uncommon neoplasm that accounts for less than 5% of all pediatric renal tumors. Compared with Wilms' tumor, CCSK has a higher rate of relapse, greater propensity for bone metastasis, and poorer overall survival. We present the case of a 19-month-old boy with a large renal mass diagnosed incidentally by ultrasonography during surveillance for vesicoureteral reflux.

Clear cell sarcomas of the kidney registered on International Society of Pediatric Oncology (SIOP) 93-01 and SIOP 2001 protocols: a report of the SIOP Renal Tumour Study Group.

Purpose: Clear Cell Sarcoma of the Kidney (CCSK) is a rare childhood renal tumour. Only a few homogeneously treated CCSK cohorts have been reported. This study aims to describe clinical characteristics and survival of CCSK patients treated according to recent International Society of Pediatric Oncology (SIOP) protocols.

[Clinical features of solid malignant tumors in childhood].

The pathogenesis of pediatric malignant tumors is associated with congenital abnormalities. Oncogenes and antioncogenes are identified in some of these cases. Neuroblastoma arises from the adrenal medulla and sympathetic ganglia.

DNA methylation profile distinguishes clear cell sarcoma of the kidney from other pediatric renal tumors.

A number of specific, distinct neoplastic entities occur in the pediatric kidney, including Wilms' tumor, clear cell sarcoma of the kidney (CCSK), congenital mesoblastic nephroma (CMN), rhabdoid tumor of the kidney (RTK), and the Ewing's sarcoma family of tumors (ESFT). By employing DNA methylation profiling using Illumina Infinium HumanMethylation27, we analyzed the epigenetic characteristics of the sarcomas including CCSK, RTK, and ESFT in comparison with those of the non-neoplastic kidney (NK), and these tumors exhibited distinct DNA methylation profiles in a tumor-type-specific manner. CCSK is the most frequently hypermethylated, but least frequently hypomethylated, at CpG sites among these sarcomas, and exhibited 490 hypermethylated and 46 hypomethylated CpG sites in compared with NK.

Clear cell sarcoma of the kidney misdiagnosed as mesoblastic nephroma: a case report and review of the literature.

Clear cell sarcoma of the kidney (CCSK) is a rare renal neoplasm of paediatrics, making up about 3% of all renal tumours in paediatrics, with a high tendency for developing bone metastasis. A seven year-old boy was referred to our clinic with two firm, large masses over the manubrium of the sternum and right frontal area, which pathologically were confirmed as a metastatic CCSK. The patient had a history of a renal mass three years earlier, for which radical nephrectomy had been performed, and histopathologic diagnosis was compatible with mesoblastic nephroma.

Outcomes of patients with revised stage I clear cell sarcoma of kidney treated in National Wilms Tumor Studies 1-5.

Purpose: To report the clinical outcomes of children with revised stage I clear cell sarcoma of the kidney (CCSK) using the National Wilms Tumor Study Group (NWTS)-5 staging criteria after multimodality treatment on NWTS 1-5 protocols.

Methods And Materials: All CCSK patients enrolled in the National Wilms Tumor Study Group protocols had their pathology slides reviewed, and only those determined to have revised stage I tumors according to the NWTS-5 staging criteria were included in the present analysis. All patients were treated with multimodality therapy according to the NWTS 1-5 protocols.

Clear cell sarcoma of the kidney: a review.

Clear cell sarcoma of the kidney (CCSK) is a rare renal tumour that is observed most often in children under 3years of age. Only a few large series of CCSK have been reported and patients with CCSK are often included among patients with other types of childhood renal tumours. The purpose of this paper is to review the published series and case reports of CCSK and to create an up-to-date overview of clinical and histological features, genetics, treatment, and outcome.

Clear cell sarcoma of kidney: A rare entity.

Clear cell sarcoma of the kidney is an uncommon renal neoplasm of childhood. It represents one of the most common unfavorable tumors included in National Wilms' Tumor Study Group clinical protocols. We came across this rare tumor in a 2-year-old male child.

Primary lung metastases in pediatric malignant non-Wilms renal tumors: data from SIOP 93-01/GPOH and SIOP 2001/GPOH.

Malignant non-Wilms renal tumors (NWRT) are a small but relevant subgroup of renal neoplasms in children. In this study we analyzed corresponding data from the trials SIOP 93-01/GPOH and SIOP 2001/GPOH of the Society of Pediatric Oncology and Hematology.Data of 22 patients with NWRT and primary lung metastases were retrospectively reviewed.

Genetics of pediatric renal tumors.

Wilms tumor (WT) accounts for approximately 95 % of all pediatric renal tumors, with a peak incidence between 2 and 3 years of age. It occurs in sporadic and congenital forms, the latter often occurring before 1 year of age. Incidence declines with age, and WT rarely is observed in adults.

Pediatric non-Wilms' renal tumors: a third world experience.

Background: Pediatric non-Wilms' renal tumors (NWRT) are poorly understood owing to their heterogeneity and relative rarity. This study aimed at auditing the outcome of the management of NWRT in a tertiary hospital in the Third World.

Methods: Records of all patients (n = 68) treated for NWRT over a 32-year period (1978-2010) were reviewed retrospectively.

Brain metastases in paediatric patients: characteristics of a patient series and review of the literature.

In contrast to the occurrence of brain metastases advanced malignant tumours in adult cancer patients, the dissemination of solid tumours to the brains of paediatric cancer patients is very uncommon. We present a neuro-pathological and clinical study of a group of children and adolescents with brain metastases (BM) from extracranial solid malignancies. The analysed patients were diagnosed with soft tissue sarcomas (three), germ cell tumours (three), or osteosarcoma, neuroblastoma, clear cell sarcoma of the kidney, or pleuropulmonary blastoma (one each).

Pediatric clear cell sarcoma of the kidney with cavoatrial thrombus.

Clear cell sarcoma of the kidney (CCSK) is a rare renal tumor. Only 4 cases of CCSK with vascular thrombus have been reported, and 2 of these were pediatric cases. One of the children had an intraatrial thrombus as well.

Pediatric and adult primary sarcomas of the kidney: a cross-sectional imaging review.

There is a wide pathological spectrum of kidney sarcomas that show characteristic histology, ontogeny, and clinical-biological behavior. While leiomyosarcomas commonly arise from the capsule, solitary fibrous tumors and clear cell sarcomas typically show renal sinus and medullary epicenter, respectively. Although distribution and imaging findings of some sarcomas may be characteristic, definitive diagnosis warrants histopathological examination following surgery.

The clinical course in pediatric solid tumor patients with focal nodular hyperplasia of the liver.

Background: Focal nodular hyperplasia (FNH) of the liver is a rare benign lesion that may be related to the vascular and hepatic damage induced by completion of tumor therapy and a reaction to localized vascular abnormality. The aim of this study was to analyze the clinical course in pediatric solid tumor patients with FNH.

Methods: We analyzed thirty-two patients with pediatric solid tumors who received multiagent chemotherapy (15 advanced neuroblastomas, 7 hepatoblastomas, 5 rhabdomyosarcomas, 2 nephroblastomas, 1 rhabdoid tumor of the kidney, 1 clear cell sarcoma of the kidney and 1 pancreatoblastoma).

Toxicology and carcinogenesis studies of isoeugenol (CAS No. 97-54-1) in F344/N rats and B6C3F1 mice (gavage studies).

Unlabelled: Isoeugenol is one of several structurally similar phenylpropenoid compounds produced by plants. It has been extracted from calamus, savory, basil, ylang-ylang, clove, tuberose, jonquil, nutmeg, tobacco, sandalwood, dill seed, mace, gardenia, petunia, and other flowers. Isoeugenol can also be produced by isomerization of eugenol, which occurs naturally in clove, pimento, bay leaf, and cinnamon.

Molecular analysis of clear cell sarcoma with translocation (1;6)(p32.3;q21).

A 23-month-old boy presented with an 11-month history of abdominal distention. Examination disclosed a large tumor in the right kidney and transabdominal radical nephrectomy was performed. Histopathological study demonstrated clear cell sarcoma of the kidney (CCSK).

A case of clear cell sarcoma of the kidney.

Clear cell renal sarcoma is a rare tumor and comprises 4% of primary pediatric malignant renal tumors. It is known as an aggressive tumor with poor prognosis. Clinically and radiographically, it resembles Wilms tumor.