Analysis of the mutational status of SIX1/2 and microRNA processing genes in paired primary and relapsed Wilms tumors and association with relapse.

Whereas 90% of patients with Wilms tumor (WT) reach cure, approximately half of patients developing a recurrent tumor die of the disease. Therefore, to disclose events leading to recurrence represents a clinical need. To study paired primary/recurrent tumor samples, being aware of the intra-tumoral heterogeneity, might help finding these answers.

Results of the Third AIEOP Cooperative Protocol on Wilms Tumor (TW2003) and Related Considerations.

Purpose: TW2003, the third Italian prospective study on Wilms tumor, aimed to improve survival in patients with stage III-IV tumors, de-escalate therapy for stage I-II nonanaplastic tumors, refine the risk stratification of therapy, and develop a national infrastructure for biobanking and central pathology review.

Materials And Methods: TW2003 recruited children 18 years old or younger with primary intrarenal tumors. Local physicians chose nephrectomy with or without preoperative chemotherapy as the initial treatment based on the risk of unsafe and/or incomplete immediate surgery.

Factors possibly affecting prognosis in children with Wilms' tumor diagnosed before 24 months of age: A report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) Wilms Tumor Working Group.

Background: Children with Wilms' tumor (WT) aged under 24 months (infants) have a better prognosis than older patients. Our aim was to study the epidemiology of this age group, with focus on the modality of diagnosis, tumor size, and association with malformations/syndromes, seeking to understand if any of these factors might be related to prognosis.

Patients And Methods: Infants diagnosed with WT between 2003 and February 2010 were evaluated.

Neuroblastoma presenting with symptoms of epidural compression at birth: a case report.

Background: Five to 10 % of children with neuroblastoma present with symptoms of epidural compression (EC). More than half these patients are diagnosed in the first year of life. The case of a neuroblastoma presenting symptoms of EC at birth is exceptional and deserves to be reported.

Whole transcriptome sequencing identifies BCOR internal tandem duplication as a common feature of clear cell sarcoma of the kidney.

Purpose: Clear cell sarcoma of the kidney (CCSK) is a rare pediatric renal tumor that is frequently difficult to distinguish among other childhood renal tumors due to its histological heterogeneity. This work evaluates genetic abnormalities carried by a series of CCSK samples by whole transcriptome sequencing (WTS), to identify molecular biomarkers that could improve the diagnostic process.

Methods: WTS was performed on tumor RNA from 8 patients with CCSK.

Loss of heterozygosity analysis at different chromosome regions in Wilms tumor confirms 1p allelic loss as a marker of worse prognosis: a study from the Italian Association of Pediatric Hematology and Oncology.

Purpose: The specific aims of the AIEOP-TW-2003 protocol included prospectively investigating a possible association of tumor loss of heterozygosity with outcomes in children treated for Wilms tumor.

Materials And Methods: We analyzed 125 unilateral favorable histology Wilms tumors registered between 2003 and 2008 in the Italian cooperative protocol for microsatellite markers mapped to chromosomes 1p, 7p, 11q, 16q and 22q.

Results: The 3-year disease-free survival and overall survival probabilities were 0.

Genomic profiling by whole-genome single nucleotide polymorphism arrays in Wilms tumor and association with relapse.

Despite the excellent survival rate of Wilms tumor (WT) patients, only approximately one-half of children who suffer tumor recurrence reach second durable remission. This underlines the need for novel markers to optimize initial treatment. We investigated 77 tumors using Illumina 370CNV-QUAD genotyping BeadChip arrays and compared their genomic profiles to detect copy number (CN) abnormalities and allelic ratio anomalies associated with the following clinicopathological variables: relapse (yes vs.

Heterogeneity of disease classified as stage III in Wilms tumor: a report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP).

Purpose: We analyzed whether the prognosis can differ among Wilms tumors (WT) labeled as Stage III according to currently adopted classification systems.

Methods And Materials: Patients with nonanaplastic Stage III WT consecutively registered in two Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials (CNR-92, TW-2003) were the subjects in the present analysis. The steady mainstay of therapy was primary nephrectomy, followed by three-drug chemotherapy with vincristine, dactinomycin, doxorubicin, and abdominal radiotherapy (RT).

Clostridium difficile-associated disease in children with solid tumors.

Goals Of Work: The goal of this study was to describe the incidence of Clostridium difficile-associated disease (CDAD) in children with solid tumours.

Patients And Methods: After documentation of a case of C. difficile-associated pseudomembranous colitis in a patient with neuroblastoma, the presence of C.

Functional inactivation of the WTX gene is not a frequent event in Wilms' tumors.

For many years the precise genetic etiology of the majority of Wilms' tumors has remained unexplained. Recently, the WTX gene, mapped to chromosome Xq11.1, has been reported to be lost or mutated in approximately one-third of Wilms' tumors.

Broviac catheter-related bacteraemias due to unusual pathogens in children with cancer: case reports with literature review.

Among 102 episodes of intravenous catheter related bacteraemias documented between January 1989 and July 1996 in children receiving antineoplastic chemotherapy or bone marrow transplantation at G. Gaslini Children's Hospital, Genoa, Italy, were identified seven episodes due to unusual pathogens: Bacillus circulans, Bacillus licheniformis, Brevibacterium casei, Flavimonas oryzihabitans, Porphyromonas asaccharolytica, Comamonas acidovorans and Agrobacterium radiobacter. Susceptibility to different antibiotics of all strains are reported.

Multiple inflammatory fibrosarcoma of the abdominal cavity in a child.

Inflammatory fibrosarcoma is a rare condition in childhood. In the abdominal location, its behaviour is often aggressive and potentially metastasizing. We report a case of a 3-year-old female with abdominal inflammatory fibrosarcoma who relapsed after 1 month from radical surgery.

Is chemotherapy effective therapy for intracranial immature teratoma? A case report.

Background: Intracranial immature teratomas (IT) are very rare germ cell tumors (GCT). The value of chemotherapy in their treatment has not been defined.

Methods: A child was referred to our hospital for consultation regarding the need for adjuvant treatment after being operated upon twice (at the age of 7 months and 11 months) for a large supratentorial intracerebral mass.

Changing pattern of pathogens causing broviac catheter-related bacteraemias in children with cancer.

The incidence of pathogens causing catheter-related bacteraemias in children undergoing antineoplastic chemotherapy with or without bone marrow transplantation at G. Gaslini Children's Hospital, Genoa, Italy, was analysed by comparing data from a retrospective study (1985-1988) with that obtained from a prospective one (1989-1992). In both periods catheter-related bacteraemias one (1989-1992).

Neuroblastoma IV-S in a patient with bilateral microphthalmia.

Neuroblastoma, a tumor of post-ganglionic sympathetic neurons, may be associated with a variety of genetic defects and congenital malformations (1). We report a case of neuroblastoma (NB) stage IV-S (2) in an infant with bilateral microphthalmia and other ocular malformations.

[Respiratory physio-kinesitherapy in cystic fibrosis: the parents' viewpoint].

The aim of this study was to evaluate the problems found in performing chest physiotherapy (PKT) by patients with Cystic Fibrosis (CF) and by their families. The research has been based upon processing 389 questionnaires (46 items) that were compiled by the families. Thirteen CF centers all over the nation have participated in this study.

Evaluation of ceftazidime in the treatment of 80 infectious episodes in compromised children.

The efficacy of ceftazidime in the treatment of infections in compromised children was evaluated in 80 such episodes occurring in 64 patients with various underlying diseases. Among the patients treated, 9 were newborns with severe neonatal distress, 21 were children with cancer and neutropenia, 8 were surgical patients, 22 had cystic fibrosis and 4 were suffering from meningitis. The following types of infections were treated: 19 bacteriologically documented and 8 possible septicemias (the latter only in newborns and neutropenic cancer patients); 2 severe upper respiratory tract infections in cancer patients; 8 soft tissue or skin infections; 1 cholangitis; 1 pneumonia; 1 osteomyelitis; 1 mediastinitis; 35 infectious exacerbations of underlying pulmonary disease in cystic fibrosis patients; and 4 meningitides.