The influence of central review on outcome in malignant gliomas of the spinal cord: the CCG-945 experience.

OBJECT The impact of central pathology review on outcome has been described in pediatric patients with high-grade glioma (HGG). The objective of this report was to analyze the impact of the central pathology review on outcome in the subgroup of patients with institutional diagnosis of HGG of the spinal cord enrolled in the Children's Cancer Group 945 cooperative study. METHODS Five neuropathologists centrally reviewed the pathology of the 18 patients with HGG of the spinal cord who were enrolled in the study.

Pathologist interobserver variability of histologic features in childhood brain tumors: results from the CCG-945 study.

In the Children's Cancer Group-945 trial, study design allowed estimation of overall interpathologist observational agreement for 6 histologic features frequently used in brain tumor diagnoses. We evaluated agreement between pairs of 5 experienced neuropathologists, who had knowledge of the general diagnoses prior to slide readings. We performed this study in an attempt to further improve pathologist interinstitutional agreement.

Severe, fetal-onset form of olivopontocerebellar hypoplasia in three sibs: PCH type 5?

We present three siblings with a precise onset of fetal seizure-like activity who had severe olivopontocerebellar hypoplasia (OPCH) and degeneration. Autopsies at 20, 27, and 37 weeks gestation showed diffuse central nervous system volume loss that was most marked for the cerebellum and brain stem structures. Neuropathological abnormalities included dysplastic, C-shaped inferior olivary nuclei, absent or immature dentate nuclei, and cell paucity more marked for the cerebellar vermis than the hemispheres.

Outcome of children with centrally reviewed low-grade gliomas treated with chemotherapy with or without radiotherapy on Children's Cancer Group high-grade glioma study CCG-945.

Background: The objectives of the current study were to determine the outcome of children who were treated with chemotherapy and radiotherapy on the Children's Cancer Group (CCG) high-grade glioma protocol (CCG-945) who were diagnosed with low-grade gliomas on post hoc central pathologic review and to identify clinical and biologic features associated with prognosis.

Methods: Between 1985 and 1991, 250 children with institutionally classified high-grade gliomas were enrolled on CCG-945. Patients older than 24 months with intracranial lesions were assigned randomly to receive either lomustine, vincristine, and prednisone (control regimen) or the 8-drugs-in-1-day regimen (experimental regimen); younger patients and those with primary spinal cord tumors were assigned nonrandomly to the experimental regimen.

Transcriptional profiling of medulloblastoma in children.

Object: Although medulloblastoma is the most common malignant brain tumor found in children, little is known about its molecular pathogenesis. The authors have attempted to compare patterns of gene expression in medulloblastoma samples with those in the healthy cerebellum.

Methods: The authors used complementary (c)DNA microarray analysis to compare the expression of genes in samples of medulloblastoma and normal cerebellum.

Doublecortin immunoreactivity in giant cells of tuberous sclerosis and focal cortical dysplasia.

Cerebral cortical lesions of tuberous sclerosis (TSC) and focal cortical dysplasia (FCD) show disturbances in laminar architecture and cellular differentiation. We immunohistochemically studied the expression of doublecortin, a fetal neuronal protein that regulates neuronal migration, in the surgical specimens of five TSC and eight FCD patients. In both TSC and FCD, bizarre giant cells showed a variable degree of doublecortin immunoreactivity.

Elevated expression of beta-site amyloid precursor protein cleaving enzyme 2 in brains of patients with Down syndrome.

The gene encoding the beta-site amyloid precursor protein cleaving enzyme 2 (BACE2) has been determined to be located on the long arm of chromosome 21 at 21q22.3. BACE2 cleaves the amyloid precursor protein at the beta-secretase site and is thought to contribute to amyloid beta protein production.

Excessive expression of synaptojanin in brains with Down syndrome.

We investigated the expression of synaptojanin, which has been mapped on 21q22.2 on human chromosome, in the cerebral cortex of patients with Down syndrome (DS), using immunohistochemistry and immunoblotting. Synaptojanin expression was observed in Cajal-Retzius cells, cortical plate neurons, subplate neurons, intermediate neurons, germinal matrix cells and the ventricular neuroepithelium of the fetal cerebrum in both controls and DS.