Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We identified 39 patients with chronic epilepsy (seizures > or = 2 years) proven to have primary brain tumors. These cases represent approximately 12% of the surgery cases for epilepsy in the same period. Mean age of seizure onset was 13.2 years: mean duration before operation was 10.5 years. Thirty-eight of 39 had normal neurologic examination. Twenty-six tumors were temporal, 7 were frontal, 4 were parietal, and 2 were occipital. Nine of 26 (34.6%) of the temporal group had contralateral interictal EEG spikes. Pathology was 15 ganglioglioma, 13 low-grade astroctoma, 4 oligodendroglioma, 2 low-grade mixed glioma, 1 pleomorphic xanthoastrocytoma, 2 dysembryoplastic neuroepithelial tumor, and 1 ependymoma. Postoperative seizure frequency (minimum follow-up 6 months) ranged from 15 to 16 seizure-free auras only in patients with temporal tumors and total gross tumor removal (mean follow-up 28 months) to 0 of 6 seizure-free in patients with extratemporal tumors who underwent subtotal resection or biopsy.
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PMID:Chronic intractable epilepsy as the only symptom of primary brain tumor. 824 54

Ependymomas and astrocytomas commonly have allelic losses of chromosome 22q, which suggests the presence of a glioma tumor suppressor gene on 22q. A candidate tumor suppressor gene on 22q is the neurofibromatosis 2 (NF2) gene since NF2 patients have an increased susceptibility to ependymomas and astrocytomas. Using single strand conformation polymorphism analysis and direct DNA sequencing, we screened 8 ependymomas and 30 fibrillary astrocytomas from non-NF2 patients for mutations in the coding sequence and portions of the 3' untranslated region of the NF2 gene. Only one mutation was detected, a single base deletion in NF2 exon 7 from a spinal ependymoma, which had also lost the wild-type allele. These results suggest that the NF2 gene may be important in the formation of some ependymomas but the NF2 gene is probably not the critical chromosome 22q tumor suppressor gene involved in astrocytoma tumorigenesis.
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PMID:Analysis of the neurofibromatosis 2 gene in human ependymomas and astrocytomas. 826 60

Despite reported activity in many other solid tumors, high-dose ifosfamide produces few objective responses in recurrent pediatric brain tumors. Alkylating agents such as cyclophosphamide (CYCLO) possess good activity in many of solid tumors, including brain tumors. Although Ifosfamide (IFOS), a structural congener of CYCLO, has been suggested to have greater activity in several tumors, its activity in brain tumors is uncertain. We conducted a phase II trial of every-other day IFOS (3 gm/M2/qod x 3) in 87 recurrent pediatric brain tumors. Responses were evaluable in 71 patients. Partial responses occurred in 1/6 patients with low grade astrocytoma, 1/16 with malignant glioma, 1/16 with medulloblastoma, 1/3 with pineoblastoma and 1/12 patients with ependymoma. No responses occurred among 10 patients with brain stem gliomas or 8 patients with other brain tumors. Despite the poor objective response rate, 23/71 patients were clinically and imaging stable for periods of 8-62 weeks. There was no relationship between prior CYCLO treatment and subsequent response or failure with IFOS. The predominant toxicity was myelosuppression. Although generally reversible, prolonged suppression and sepsis were responsible for the deaths of 3 heavily pretreated patients. Renal toxicity was uncommon; 2 patients had grade III, and one grade IV renal tubular dysfunction. One patient had grade IV hematuria. Neurotoxicity was less common than in studies of daily ifosfamide; only 1 patient had grade IV neurotoxicity. Three patients had grade III or IV IFOS related hyponatremia. Despite the good stable disease rate, the poor rate of objective response suggests that IFOS monotherapy possesses little clinically meaningful activity in brain tumors.
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PMID:A phase II study of every other day high-dose ifosfamide in pediatric brain tumors: a Pediatric Oncology Group Study. 852 93

Ten patients with brainstem tumors (BST) underwent Gamma Knife radiosurgery. In 5 cases, there were definitive histological diagnoses; ependymoma (n = 3) and CNS lymphoma (n = 2). The others were diagnosed as ependymoma (n = 1), CNS lymphoma (n = 2) and glioma (n = 2) on clinical grounds. Of 4 cases with ependymoma, the tumor showed a marked response in 3 cases. Of 4 cases with CNS lymphoma, all responded rapidly and the clinical symptoms improved markedly. However, in the 2 cases with glioma, the tumor remained unchanged or continued to grow. Gamma Knife radiosurgery was effective in local growth control of BST without adverse effects. An alternative treatment design will be required in glioma cases.
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PMID:Gamma Knife radiosurgery for the treatment of brainstem tumors. 858 38

Glial tumors in the cerebellopontine angle (CPA) are rare. Four histologically distinct types of glial tumors of the CPA have been described in the literature as ependymoma, medulloblastoma, mixed glial tumor, and fibrillary astrocytoma. This case report describes a pilocytic astrocytoma of the CPA. A 58-year-old man with a hearing loss had an extra-axial tumor in the left CPA that extended into the internal auditory canal. The characteristics of the tumor on magnetic resonance imaging scans differed from those of typical CPA tumors. It adhered avidly to the cochlear and vestibular nerves, which had to be sacrificed for gross total resection. Microscopic examination showed the typical features of an adult-type pilocytic astrocytoma.
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PMID:Pilocytic astrocytoma presenting as an extra-axial tumor in the cerebellopontine angle: case report. 858 72

The rationale for obtaining surveillance computerized tomography (CT) scans or magnetic resonance (MR) images in pediatric patients with brain tumors is that early detection of recurrence may result in timely treatment and better outcome. The purpose of this study was to investigate the value of surveillance cranial images in a variety of common pediatric brain tumors managed at a tertiary care pediatric hospital. A retrospective chart review was performed of children with astrocytoma of the cerebral hemisphere, cerebellum, optic chiasm/hypothalamus, or thalamus; cerebellar or supratentorial high-grade glioma; supratentorial ganglioglioma; posterior fossa or supratentorial primitive neuroectodermal tumor (PNET); and posterior fossa ependymoma. Data were analyzed to determine the frequency with which recurrences were identified on a surveillance image and how the type of image at which recurrence was identified related to outcome. In 159 children, 17 of 44 recurrences were diagnosed by surveillance imaging. The percentage of recurrences identified by surveillance imaging was 64% for ependymoma, 50% for supratentorial PNET, 43% for optic/hypothalamic astrocytoma, and less than 30% for other tumors. The rate of diagnosis of recurrence per surveillance image varied from 0% to 11.8% for different tumor types. Only for ependymomas did there appear to be an improved outcome when recurrence was identified prior to symptoms. Our results indicate that, using the protocols outlined in this study, surveillance imaging was not valuable in identifying recurrence of cerebellar astrocytoma or supratentorial ganglioglioma during the study period, but was probably worthwhile in identifying recurrence of posterior fossa ependymoma and optic/hypothalamic astrocytoma and, possibly, medulloblastoma. Surveillance protocols could be made more effective by individualizing them for each type of tumor, based on current data on the patterns of recurrence.
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PMID:Value of postoperative surveillance imaging in the management of children with some common brain tumors. 862 43

To determine the frequency and nature of MRI lesions in children with neurofibromatosis type I (NF1), 50 patients aged 8 to 16 years were evaluated prospectively with cranial MRI. Forty-one children were asymptomatic with respect to central nervous system pathology, and 50% were macrocephalic. Sixteen patients (32%) had normal MRI examinations. Thirty-two patients (64%) had high intensity lesions on T2-weighted images and 16 patients (32%) had hyperintense lesions on T1-weighted images. Seven patients (14%) had ventricular dilatation (associated with increased intracranial pressure in 2) and 11 patients (22%) had optic pathway lesions (optic glioma in 7). MRI was superior to CT in differentiating optic sheath thickening and optic nerve tortuosity from optic glioma in four patients. An intracranial tumour (ependymoma) and sphenoid wing dysplasia were evident in individual patients. Findings previously unreported in NF1 included an aqueductal web resulting in hydrocephalus, intraocular neurofibroma resulting in retinal detachment, and asymptomatic enlargement of the septum pellucidum. T1 and T2 signal abnormalities in isolation were not associated with neurological deficits or the occurrence of macrocephaly, and all lesions that required intervention were suspected clinically. Macrocephaly in the absence of increased intracranial pressure or accelerated head growth is not an indication for neuroimaging in children with NF1. However, the majority of children (68%) had disease-specific abnormalities and thus MRI may provide a useful adjunct to clinical evaluation in the diagnosis of equivocal cases.
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PMID:MRI findings in children with neurofibromatosis type 1: a prospective study. 866 66

The increased frequency of glioma among neurofibromatosis 1 (NF1) patients suggests a general involvement of the NF1 gene in glioma tumourigenesis. Using the methodology of conventional Southern blotting with a complete panel of overlapping partial cDNAs covering the whole NF1 gene, we screened 31 gliomas of several different subtypes and 3 primitive neuroectodermal tumours (PNETs) from non-NF1 patients for aberrant restriction patterns in their tumour DNA samples. Clear evidence for somatic mutation events at the NF1 gene locus was found in 1 astrocytoma, 2 glioblastomas, 1 ependymoma and 1 PNET with an astrocytic component. These results suggest that the NF1 gene is important in suppressing tumours of neuroectodermal origin.
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PMID:Somatic mutations in the neurofibromatosis 1 gene in gliomas and primitive neuroectodermal tumours. 866 13

Tumor growth depends on cell division and cell death. To investigate the role of apoptosis in tumor cell death, we examined 83 cases of glial tumors using in situ nonradioactive tailing of DNA breaks. In addition, since p53 protein may participate in the regulation of apoptosis in glioblastoma, we compared the apoptosis ratio (AR) with the labeling index (LI) of p53 protein immunopositivity. The AR in glial tumor parenchyma ranged from 0 to 1.4%: mean AR +/- standard deviation was 0.4 +/- 0.4% (range, 0-1.4) for glioblastoma, 0.3 +/- 0.3% (range, 0.01-0.83) for anaplastic astrocytoma, 0.1 +/- 0.1% (range, 0-0.41) for low-grade astrocytoma, 0.006 +/- 0.008% (range, 0-0.02) for pilocytic astrocytoma, 0.2 +/- 0.2% (range, 0-0.62) for oligodendroglioma and 0.003 +/- 0.004% (range, 0-0.01) for ependymoma. ARs were significantly higher in higher-grade astrocytic tumors than in lower-grade tumors (Mann-Whitney U test: P = 0.0003), although wide variability in each group resulted in overlapping between the groups. p53 protein immunopositivity (more than 25% of nuclei) was found in 15 of 32 glioblastoma cases, while in the remaining 17 none or only a low percentage (up to 6%) of the nuclei were positive. In p53 protein-positive cases mean AR (0.51 +/- 0.47%) was not significantly higher than that in p53 protein-negative cases (0.22 +/- 0.23%; P = 0.1681).
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PMID:Apoptosis in glial tumors as determined by in situ nonradioactive labeling of DNA breaks. 877 55

The transmission of donor-related malignancies by organ transplantation is a rather rare event. There has only been one report on the development of a brain tumor metastasis in liver transplantation. From September 1988 to January 1993, 342 donor hepatectomies with subsequent transplantation were performed at our center. The main donor diagnoses included subarachnoidal bleeding (n = 128; 37.4%), isolated head injury (n = 114; 33.3%), multiple injuries (n = 55; 16.1%), primary cerebral neoplasia (n = 13; 3.8%), and other (n = 32; 9.4%). Primary cerebral neoplasia included glioblastoma (n = 4), meningioma (n = 3), astrocytoma (n = 2), angioma (n = 2), neurocytoma (n = 1), and ependymoma (n = 1). In the group of donors suffering from primary cerebral neoplasia, procured organs other than the liver included kidneys (n = 20), combined kidneys and pancreata (n = 1), pancreata (n = 2), hearts (n = 8), combined hearts and lungs (n = 1), and single lungs (n = 1). Follow-up of the respective graft recipients ranged from 28 to 68 months (median 43 months). Recurrent malignancy was observed once, in a liver graft recipient. The donor, a 48-year-old female, had undergone surgical resection of an intracerebral multiform glioblastoma and died 4 months later of a relapse in the brain stem. The 28-year-old female recipient had undergone transplantation for an autoimmune-hepatitic cirrhosis. Four months later, histopathological examination of an intraperitoneal and intrahepatic mass revealed a poorly differentiated, small-cell pleomorphic cancer, identified as a glioma metastasis by S100- and glial fibrillary acidic protein immunohistochemical staining. The patient died 6 months post-transplantation. On autopsy, no further neoplastic lesions were detected. Our review adds a second reported case of a liver graft-transmitted brain tumor to the literature and the fourth donor-related malignancy after hepatic transplantation in general.
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PMID:Liver graft-transmitted glioblastoma multiforme. A case report and experience with 13 multiorgan donors suffering from primary cerebral neoplasia. 900 60


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