CNS Embryonal Tumor with PLAGL Amplification, a New Tumor in Children and Adolescents: Insights from a Comprehensive MRI Analysis.

Background And Purpose: CNS embryonal tumor with pleomorphic adenoma gene-like 1 ()pleomorphic adenoma gene-like 2 ( amplification (ET, PLAGL) is a newly identified, highly malignant pediatric tumor. Systematic MRI descriptions of ET, PLAGL are currently lacking.

Materials And Methods: MRI data from 19 treatment-naïve patients with confirmed ET, PLAGL were analyzed.

Determinants of impairments in functioning, fatigue, and participation ability in pediatric brain tumor survivors.

Background: Pediatric brain tumor survivors (PBTS) experience disease- and treatment-related sequelae. We aimed to investigate the occurrence of participation limitations, impairments in functioning, fatigue, and the association between patient, tumor- and treatment-related factors and these outcomes.

Methods: Children (4-18 years) after treatment for a brain tumor between 2005 and 2014 at the Erasmus Medical Center, Rotterdam, the Netherlands, were eligible.

Ketogenic diet treatment in recurrent diffuse intrinsic pontine glioma in children: A safety and feasibility study.

Background: The mean overall survival rate of children with diffuse intrinsic pontine glioma (DIPG) is 9-11 months, with current standard treatment with fractionated radiotherapy and adjuvant chemotherapy. So far, novel therapeutic strategies have not yet resulted in significantly better survival. The main source of energy for glioblastoma cells is glucose.

Survival prediction model of children with diffuse intrinsic pontine glioma based on clinical and radiological criteria.

Background: Although diffuse intrinsic pontine glioma (DIPG) carries the worst prognosis of all pediatric brain tumors, studies on prognostic factors in DIPG are sparse. To control for confounding variables in DIPG studies, which generally include relatively small patient numbers, a survival prediction tool is needed.

Methods: A multicenter retrospective cohort study was performed in the Netherlands, the UK, and Germany with central review of clinical data and MRI scans of children with DIPG.

Improving chemotherapy processes with a protocol-based information system: a pre and post-implementation study.

Background: The medical application domain has been a great challenge for information technology solutions for decades, especially when the target process has been complex and multidisciplinary such as chemotherapy processes.

Objective: To evaluate the impact of a homegrown protocol based information system on the efficiency of chemotherapy workflow processes in an outpatient setting.

Methods: A day care unit of the Hematology/Oncology outpatient clinic of Erasmus Medical Center was the setting for this study.

Hyperfractionated versus conventional radiotherapy followed by chemotherapy in standard-risk medulloblastoma: results from the randomized multicenter HIT-SIOP PNET 4 trial.

Purpose: To compare event-free survival (EFS), overall survival (OS), pattern of relapse, and hearing loss in children with standard-risk medulloblastoma treated by postoperative hyperfractionated or conventionally fractionated radiotherapy followed by maintenance chemotherapy.

Patients And Methods: In all, 340 children age 4 to 21 years from 122 European centers were postoperatively staged and randomly assigned to treatment with hyperfractionated radiotherapy (HFRT) or standard (conventional) fractionated radiotherapy (STRT) followed by a common chemotherapy regimen consisting of eight cycles of cisplatin, lomustine, and vincristine.

Results: After a median follow-up of 4.

Pitfalls in molecular analysis for mismatch repair deficiency in a family with biallelic pms2 germline mutations.

Heterozygous germline mutations in the mismatch repair (MMR) genes MLH1, MSH2, MSH6 and PMS2 cause Lynch syndrome. Biallelic mutations in the MMR genes are associated with a childhood cancer syndrome [constitutional mismatch repair deficiency (CMMR-D)]. This is predominantly characterized by hematological malignancies and tumors of the bowel and brain, often associated with signs of neurofibromatosis type 1 (NF1).

Differential expression and prognostic significance of SOX genes in pediatric medulloblastoma and ependymoma identified by microarray analysis.

The objective of this study was to identify differentially expressed and prognostically important genes in pediatric medulloblastoma and pediatric ependymoma by Affymetrix microarray analysis. Among the most discriminative genes, three members of the SOX transcription factor family were differentially expressed. Both SOX4 and SOX11 were significantly overexpressed in medulloblastoma (median, 11-fold and 5-fold, respectively) compared with ependymoma and normal cerebellum.

Various components of the insulin-like growth factor system in tumor tissue, cerebrospinal fluid and peripheral blood of pediatric medulloblastoma and ependymoma patients.

The insulin-like growth factor (IGF) system plays an important role in neuronal development and may contribute to the development of brain tumors. In this study, we studied mRNA expression levels of IGFs, insulin-like growth factor binding proteins (IGFBPs) and insulin-like growth factor receptors (IGFRs) in 27 pediatric medulloblastomas, 13 pediatric ependymomas and 5 control cerebella. Compared to normal cerebellum, mRNA levels of IGFBP-2 and IGFBP-3 were significantly increased in medulloblastomas and ependymomas.

Increased total-Tau levels in cerebrospinal fluid of pediatric hydrocephalus and brain tumor patients.

Total Tau (t-Tau), hyperphosphorylated Tau (p-Tau(181P)) and beta-amyloid(1-42) in cerebrospinal fluid (CSF) have shown to be markers of neuronal and axonal degeneration in various neurological and neurodegenerative diseases. The aim of this study was to evaluate the influence of the presence of a brain tumor and hydrocephalus on t-Tau, p-Tau(181P) and beta-amyloid(1-42) levels in CSF of pediatric patients. t-Tau, p-Tau(181P) and beta-amyloid(1-42) levels were simultaneously quantified by xMAP technology in 22 lumbar and 15 ventricular CSF samples from newly diagnosed pediatric brain tumor patients and 39 lumbar and 12 ventricular CSF samples from pediatric patients without a brain tumor.

Identification of novel biomarkers in pediatric primitive neuroectodermal tumors and ependymomas by proteome-wide analysis.

The aim of this study was to identify aberrantly expressed proteins in pediatric primitive neuroectodermal tumors (PNETs) and ependymomas. Tumor tissue of 29 PNET and 12 ependymoma patients was subjected to 2-dimensional difference gel electrophoresis. Gel analysis resulted in 79 protein spots being differentially expressed between PNETs and ependymomas (p < 0.

Biallelic germline mutations of mismatch-repair genes: a possible cause for multiple pediatric malignancies.

Background: Heterozygous defects in mismatch-repair (MMR) genes cause hereditary nonpolyposis colorectal cancer (HNPCC). In this syndrome, tumors typically arise from age 25 years onward. Case reports have shown that homozygosity or compound heterozygosity for MMR gene mutations can cause multiple tumors in childhood, sometimes combined with neurofibromatosis type I (NF1)-like features.

Identification of apolipoprotein A-II in cerebrospinal fluid of pediatric brain tumor patients by protein expression profiling.

Background: Our aim was to detect differences in protein expression profiles of cerebrospinal fluid (CSF) from pediatric patients with and without brain tumors.

Methods: We used surface-enhanced laser desorption/ionization time-of-flight (SELDI-TOF) mass spectrometry and Q10 ProteinChip arrays to compare protein expression profiles of CSF from 32 pediatric brain tumor patients and 70 pediatric control patients. A protein with high discriminatory power was isolated and identified by subsequent anion-exchange and reversed-phase fractionation, gel electrophoresis, and mass spectrometry.

Functional outcome after low-grade astrocytoma treatment in childhood.

Background: The relatively high survival rate of patients with low-grade astrocytoma necessitates increasing attention to physical and psychosocial outcomes. The objective of the current study was to investigate functional outcomes among children who were treated for low-grade or pilocytic astrocytoma in different areas of the brain.

Methods: Functional outcomes were evaluated in the following domains: impairments, disabilities, handicaps, and quality of life (QOL).

Differential patterns of insulin-like growth factor-I and -II mRNA expression in medulloblastoma.

Insulin-like growth factors (IGFs) play an important role in tumour growth and development. We hypothesized that this is also the case for medulloblastomas, which are highly malignant cerebellar brain tumours usually occurring in children. In these tumours the expression patterns of IGF-I and -II mRNA were studied.

Adsorption of erythropoietin and growth hormone to peritoneal dialysis bags and tubing.

Objective: To study the adsorption of erythropoietin and growth hormone to dialysis bags and tubing.

Design: In vitro study in which radiolabeled erythropoietin and recombinant human growth hormone were added to small-volume (50- and 250-mL) dialysis bags. Recovery was measured after 15-minute dwells.

Growth hormone treatment of children with brain tumors and risk of tumor recurrence.

GH is increasingly used for treatment of children and adults. It is mitogenic, however, and there is therefore concern about its safety, especially when used to treat cancer patients who have become GH deficient after cranial radiotherapy. We followed 180 children with brain tumors attending three large hospitals in the United Kingdom and treated with GH during 1965-1996, and 891 children with brain tumors at these hospitals who received radiotherapy but not GH.

Intraperitoneal hypercoagulation and hypofibrinolysis is present in childhood peritonitis.

An increased rate of obstruction of peritoneal dialysis catheters is observed during peritonitis. Hypercoagulation and hypofibrinolysis may explain this increased occurrence. We studied plasminogen activator inhibitor type 1 antigen (PAI-1), tissue-type plasminogen activator antigen (t-PA), D-dimer (DD), plasmin-alpha2-antiplasmin complexes (PAP), and thrombin-antithrombin III complexes (TAT) in 7 children with peritonitis (group A) and 12 children during stable peritoneal dialysis (group B).

Peritoneal protein loss in children with nephrotic syndrome during peritoneal dialysis.

Background: The passage of proteins across the glomerular filtration barrier is mainly determined by the size of the protein. In nephrotic syndrome (NS) the glomerular permselectivity is affected, causing proteinuria. Some authors suggest the existence of a generalized basement membrane defect.

Increase of the bioavailability of intraperitoneal erythropoietin in children on peritoneal dialysis by administration in small dialysis bags.

Objective: To establish the effectivity of administration of erythropoietin intraperitoneally in a small amount of fluid in children with renal anemia on continuous ambulatory peritoneal dialysis (CAPD).

Design: Prospective study in which children with renal anemia on CAPD were treated with erythropoietin intraperitoneally, administered in a specially designed bag containing 50 mL NaCl 0.9%.

Dural sinus thrombosis in children with cancer.

Dural sinus thrombosis (DST) has been reported in association with cancer in both adults and children. We describe the seven patients seen with this complication in our centre between 1981 and 1995. Diagnosis was confirmed by either cerebral CT scanning, MRI or angiography.

Adequate dialysis? Measurement of KT/V in a pediatric peritoneal dialysis population.

Objective: To measure the urea and creatinine kinetics in a pediatric population.

Patients And Methods: In 19 children treated with peritoneal dialysis (PD) KT/V, urea and creatinine clearances (Ccr) were measured. Thirteen children were on continuous ambulatory peritoneal dialysis (CAPD) and 6 on highly intermittent peritoneal dialysis (NIPD).

Growth hormone therapy and malignancy.

The possibility that human growth hormone (GH) replacement therapy might either increase the risk of cancer recurrence in a child who has previously been treated for a brain tumour or leukaemia, or induce de novo cancer, has worried paediatricians for a number of years. Concern arises from animal experiments, the association of acromegaly with malignancy, and the Japanese experience of a cluster of de novo leukaemia cases in children treated with GH. It is reassuring that so far the results from single centre studies and from the pharmaceutical industry surveillance programmes have shown no evidence of an increased risk of malignancy, recurrent or de novo.