Cases reported "Glioma"

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1/793. Complete remission of a diffuse pontine glioma.

    A patient is described in whom a large diffuse glioma of the pons extending into the midbrain was diagnosed at the age of 2 years. biopsy showed a fibrillary astrocytoma. After shunting of a hydrocephalus, the clinical symptoms abated without conventional therapy. Repeated MRI studies showed a continuous decrease of the tumour which was no longer visible when the patient was 6.6 years old. In reviews on spontaneous remissions of oncologic disorders we were unable to find a case of a biologically benign brain stem tumour. There is one isolated report on a similar case, though without histologic documentation.
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ranking = 1
keywords = glioma
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2/793. Secondary glioblastoma remarkably reduced by steroid administration after anaplastic transformation from gliomatosis cerebri--case report.

    A 45-year-old female presented with gliomatosis cerebri manifesting as hemiballismus-like involuntary movement in the arm, motor weakness in the leg, and hypesthesia in her left side. Computed tomography showed only diffuse swelling of the right cerebral hemisphere, but T2-weighted magnetic resonance imaging revealed a diffuse lesion spreading from the right thalamus to the temporal, parietal, and occipital lobes on the same side. No abnormal enhancement was recognized. cerebral angiography showed no specific finding. A right occipital lobectomy was performed to confirm the diagnosis of gliomatosis cerebri. Anaplastic transformation was recognized 5 months later. The disease did not resolve with radiation or interferon administration, but steroid therapy achieved remarkably effective tumor regression. The patient died due to pneumonia. autopsy showed the features of diffuse glioblastoma. Steroid therapy may be an effective treatment for gliomatosis cerebri before the terminal stage.
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ranking = 1.4
keywords = glioma
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3/793. MRI diagnosis of gliomatosis cerebri.

    Until recently the diagnosis of gliomatosis cerebri has been made on postmortem examination. This article reviews the use of serial magnetic resonance imaging studies to suggest premorbid diagnosis of this condition. The following is a case report of a 14-year-old female who had a subtotal cortical resection of tumor and several years later developed a progressive dementia. At postmortem examination the diagnosis of gliomatosis cerebri was made. Diffuse progressive white matter changes involving both hemispheres and brainstem, with increased thickness of the corpus callosum and without changes in cortical markings on T2-weighted magnetic resonance images, in this patient were highly suggestive of the diagnosis of gliomatosis cerebri.
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ranking = 1.4
keywords = glioma
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4/793. Molecular analysis of glioma and skin-tumour alterations in a xeroderma-pigmentosum child.

    xeroderma pigmentosum (XP) is a rare hereditary disease characterized by a very high frequency of skin tumours due to a defect in the nucleotide-excision-repair process. Some of these patients have also been reported to develop internal tumours with higher frequency than the normal population. Reported here are the clinical features and molecular analysis of an XP patient who developed multiple skin cancers as well as a thalamic glioma. Complementation analysis with recombinant retrovirus, cloning efficiency and unscheduled DNA synthesis after UV-C indicate that the patient belongs to the C group. Characterization of the p53 mutations in the 2 tumours of the patient leads to speculation on the aetiological agents involved in tumour initiation. The skin tumour is clearly induced by the presence of unrepaired UVB-induced dna damage on the non-transcribed strand of the p53 gene, while the glioma may be induced by unrepaired DNA lesions produced by free radicals.
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ranking = 1.2
keywords = glioma
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5/793. Diffuse leptomeningeal gliomatosis with oligodendroglioma.

    Leptomeningeal gliomatosis is a primary glioma residing mainly in the leptomeninges. We describe a rare case of diffuse leptomeningeal glioma, occurring in a six year old girl, in which the morphological features of the cellular infiltrates were those of an oligodendroglioma. To our knowledge, this is the fifth case of diffuse oligodendrogliomatosis reported in the English language literature.
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ranking = 2.6
keywords = glioma
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6/793. color Doppler image of central retinal artery of eyes with an intraconal mass.

    PURPOSE: Retinal ischemia secondary to hypoperfusion of the central retinal artery is recognized as one factor that may contribute to the development of loss of vision in eyes with intraorbital tumors. We study intraorbital tumors which produce motility disturbances and visual problems by color Doppler imaging to evaluate this factor. methods: We examined the central retinal artery velocities of 3 patients with disc edema caused by intraconal masses (2 cavernous hemangiomas and 1 presumed optic nerve glioma) via color Doppler imaging. RESULTS: The time-velocity waveform demonstrated abnormally high vascular resistance in the central retinal artery of all affected eyes in the primary position compared with the normal waveform seen in the other eyes. We compared the pulsatility index of eyes with an intraconal mass and contralateral, control eyes using Student's t test for paired samples and significant differences were noted between both groups (p< 0.01). CONCLUSIONS: Intraconal tumors could produce increased pressure in the optic nerve sheath and the optic nerve tissue which could be associated with impaired retinal and optic nerve blood flow and the subsequent amaurosis encountered with intraorbital tumors.
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ranking = 0.2
keywords = glioma
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7/793. Transnasal endoscopic repair of congenital defects of the skull base in children.

    OBJECTIVE: To examine imaging findings and methods of endoscopic treatment of congenital skull base defects in children. DESIGN: Retrospective study and case series. SETTING: Academic tertiary care center. patients: Four patients (aged 12 and 14 months and 8 and 13 years) were included from 1995 to 1997. Three presented with a nasal glioma, which was recurrent in 1 case. The fourth patient presented with bacterial meningitis due to a spontaneous cerebrospinal fluid leak. Computed tomography and magnetic resonance imaging were used to locate the defect of the skull base. INTERVENTION: Transnasal endoscopic resection of the glioma or the meningocele, with immediate repair of the skull base defects using free mucosal flaps and/or pediculized mucosal flaps and/or conchal cartilage together with fibrin glue and nasal packing during a 3-week period. RESULTS: None of the 4 patients has experienced recurrent cerebrospinal fluid leaks or postoperative meningitis. CONCLUSIONS: The transnasal endoscopic repair of congenital meningoceles is a reliable technique in select pediatric patients. Computed tomography and magnetic resonance imaging provide information that can be used to help the surgical procedure.
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ranking = 0.4
keywords = glioma
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8/793. A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including "rosetted") islands: report of 4 cases.

    Four examples of a novel glioneuronal neoplasm are presented. All tumors affected adults (including two males and two females aged 25-40 years) as supratentorial, cerebral hemispheric masses with associated seizure activity and, in one case, symptoms of raised intracranial pressure and progressive hemiparesis. CT scans in two cases revealed hypodense masses without calcification. MRI scans at presentation demonstrated, in all cases, solid T1-hypointense and T2-hyperintense tumors with mass effect in one instance but no edema or contrast enhancement. Only one was relatively circumscribed on neuroradiologic study. All were infiltrative in their histologic growth pattern and predominantly glial in appearance, being composed mainly of fibrillary, gemistocytic, or protoplasmic astroglial elements of WHO grade II to III. Their distinguishing feature was their content of sharply delimited, neuropil-like islands of intense synaptophysin reactivity inhabited and rimmed in rosetted fashion by cells demonstrating strong nuclear immunolabeling for the neuronal antigens NeuN and Hu. These cells included small, oligodendrocyte-like ("neurocytic") elements as well as larger, more pleomorphic forms. Two cases contained, in addition, well-differentiated neurons of medium to ganglion-cell size. Proliferative activity was observed principally within the glial compartment; two cases contained mitotic figures and exhibited relatively elevated MIB-1 indices (6.8% and 8.2%). One of the latter progressed and proved fatal at 30 months following subtotal resection and radiotherapy. The three other patients are alive at intervals of 14 to 83 months, two tumor-free and one with extensive disease associated with the appearance of enhancement on MRI. This glioneuronal tumor variant may pursue an unfavorable clinical course.
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ranking = 0.00033333070463259
keywords = glial
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9/793. Gliomatosis cerebri with secondary glioblastoma formation: report of two cases.

    The clinicopathological features of two cases of gliomatosis cerebri associated with secondary glioblastoma formation are reported. In both cases, glial cells were diffusely distributed in the supra- and infratentorial regions and underlying brain structures were preserved from the onset. In spite of such diffuse distribution of neoplastic glial cells, similar to that observed in low-grade astrocytoma, in both cases the tumor underwent complete remission after radiotherapy. However, the tumor recurred as a localized glioblastoma in both cases, 37 months (case 1) and 7 months (case 2) after the radiotherapy. In both cases, recurrence was accompanied by prominent dissemination of CSF. The recurrent tumors were radiation resistant, and the patients' conditions deteriorated rapidly after recurrence. The present two cases demonstrated that gliomatosis cerebri, classified among brain tumors of unknown origin by the world health organization, may transform into highly proliferative circumscribed tumors, in spite of their good response to radiotherapy. Examination of pathological features and their correlation with MRI findings may allow us to better understand the response to radiotherapy and the process of recurrence.
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ranking = 0.40162450400542
keywords = glioma, glial cell, glial
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10/793. A case history of glioma progression.

    Low-grade diffuse astrocytomas have an intrinsic tendency for malignant progression but the factors determining the kinetics of this process are still poorly understood. We report here the case of a male patient who developed a fibrillary astrocytoma at the age of 33 years and who underwent six surgical interventions over a period of 17 years without radiotherapy or chemotherapy. The first three biopsies spanned a period of 11 years and led to the diagnosis of low-grade, diffuse astrocytoma (WHO grade II), with a growth fraction (MIB-1 labeling index) of 2.3-3.7%. The fourth to sixth biopsies showed histological features of anaplastic astrocytoma (WHO grade III), with growth fractions between 5.0 and 10.5%. The fraction of gemistocytic neoplastic astrocytes also increased, from 0.3% in the first biopsy to 17.5% in the last biopsy and preceded the increase in proliferative activity and transition to anaplastic astrocytoma. The fraction of tumor cells immunoreactive to BCL-2 increased from 0.3% to 8.2%. A p53 mutation in codon 273 (CGT-->TGT, Arg-->Cys) was identified in the first biopsy and persisted throughout the course of the disease. However, the fraction of cells with p53 protein accumulation increased significantly during progression, from 3.2% in the first biopsy to 13.7% in the last. The absence of additional genetic alterations (PTEN mutations, loss of chromosome 10 and 19q) may be responsible for the slow progression and lack of glioblastoma features even after a 17-year disease duration.
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ranking = 0.8
keywords = glioma
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