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

We would like to report a rare case of pontine glioma with unusual neuroimaging features. The patient was a 3-year-old girl who suffered from chronic nausea and gait disturbance for several months. Computed tomography (CT) demonstrated ventricular dilatation, and ventricular peritoneal (VP) shunt was performed for idiopathic hydrocephalus at another hospital. Fever of unknown origin continued for a month after the VP shunt. At our hospital, cerebrospinal fluid examination showed bacterial meningitis, and it was assumed that shunt infection lead to shunt failure. Magnetic resonance imaging (MRI) revealed hydrocephalus and pontine swelling, and serial MRI suggested brainstem tumor extending to the bilateral thalamus. The patient underwent stereotactic biopsy of the left thalamic tumor, under general anesthesia, and the histological diagnosis was anaplastic astrocytoma. Diffuse pontine glioma rarely increases without cranial nerve deficits. In the present case, pontine glioma extended to the bilateral thalamus symmetrically. It was difficult to diagnose the presented lesion as pontine glioma in the early period because of its unusual neuroimaging.
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PMID:[A case of pontine astrocytoma with unusual neuroimaging features]. 1988 61

Higher rates of glucose usage generally correlate with poor prognosis in several types of malignant tumours. Experimental studies (both in vitro and in vivo) have shown that 2-deoxy-D-glucose (2-DG), a glucose analog and glycolytic inhibitor, enhances radiation-induced damage selectively in tumor cells while protecting normal cells, thereby suggesting that 2-DG can be used as a differential radiomodifier to improve the efficacy of radiotherapy. Clinical trials undertaken to study the feasibility, safety, and validity of this suggested approach will be described. Based on 2-DG-induced radiosensitization observed in primary organ cultures of cerebral glioma tissues, clinical trials were designed taking into consideration the radiobiology of gliomas and pharmacokinetics of 2-DG. Phase I/II clinical trials have unequivocally demonstrated that a combination of 2-DG (200-300 mg 2-DG per kg body weight orally administered after overnight fasting, 20 min before irradiation) with large weekly fractions (5 Gy/fraction) of low-LET radiotherapy is well tolerated without any acute toxicity or late radiation damage to the normal brain tissue. Nonserious transient side effects similar to hypoglycemia induced disturbances like restlessness, nausea, and vomiting were observed at the 2-DG doses used. Data from these trials involving more than 100 patients have clearly indicated a moderate increase in the survival, with a significant improvement in the quality of life with clinicopathological evidence of protection of normal brain tissue. A phase III multicentric trial to evaluate the efficacy of the combined treatment is in progress. Directions for future studies are discussed.
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PMID:Clinical studies for improving radiotherapy with 2-deoxy-D-glucose: present status and future prospects. 2000 89

PURPOSE Concomitant temozolomide (TMZ)/radiotherapy followed by adjuvant TMZ has increased survival in patients with glioblastoma multiforme (GBM). However, few options are effective for patients who experience treatment failure. We conducted a multicenter, phase II study to assess the efficacy and safety of continuous dose-intense TMZ for recurrent GBM. PATIENTS AND METHODS Patients with malignant glioma at progression after standard TMZ 150 to 200 mg/m(2) x 5 days in a 28-day cycle for three or more cycles were stratified by tumor type (anaplastic glioma group A, GBM, group B). Ninety-one patients with GBM were prospectively divided into three groups (early [B1], extended [B2], and rechallenge [B3]) according to the timing of progression during adjuvant therapy. All patients received continuous dose-intense TMZ 50 mg/m(2)/d for up to 1 year or until progression occurred. Response was assessed by using RECIST (Response Evaluation Criteria in Solid Tumors). Results A total of 116 of 120 patients were evaluable for efficacy. For patients with GBM, 6-month progression-free survival (PFS) was 23.9% (B1, 27.3%; B2, 7.4%; B3, 35.7%). One-year survival from time of study entry was 27.3%, 14.8%, and 28.6% for the B1, B2 and B3 groups, respectively. For patients with anaplastic glioma, 6-month PFS was 35.7%; 1-year survival was 60.7%. The most common grades 3 and 4 nonhematologic toxicities were nausea/vomiting (6.7%) and fatigue (5.8%). Grades 3 and 4 hematologic toxicities were uncommon. CONCLUSION Rechallenge with continuous dose-intense TMZ 50 mg/m(2)/d is a valuable therapeutic option for patients with recurrent GBM. Patients who experience progression during the first six cycles of conventional adjuvant TMZ therapy or after a treatment-free interval get the most benefit from therapy.
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PMID:Phase II trial of continuous dose-intense temozolomide in recurrent malignant glioma: RESCUE study. 2030 52

A 45-year-old man with a new diagnosis of low grade glioma was started on an escalating dose of levetiracetam (Lev) for seizure management. He gradually developed intractable nausea/vomiting and a high creatinine concentration due to acute renal failure which was attributed to Lev-induced interstitial nephritis. The medication was changed and his renal function rapidly improved to his baseline.
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PMID:Levetiracetam-induced interstitial nephritis in a patient with glioma. 2207 60

Gliomas may rarely arise in the leptomeninges without any evidence of intraaxial involvement. A case of primary diffuse leptomeningeal gliomatosis (PDLG) histologically diagnosed as oligoastrocytoma is presented. A 50-year-old woman presented with nausea, vomiting and headache. Magnetic resonance imaging (MRI) of cranium and cervical region revealed dural thickening starting from the craniocervical junction to the level of C4 without any parenchymal lesions. CSF examination showed an increase in protein and decrease in glucose levels. There were neither any kind of atypical cells nor any kind of growth in bacterial cultures. The patient underwent biopsy at the level of C1 for diagnosis. The specimen was diagnosed as primary diffuse leptomeningeal gliomatosis, with phenotypic features of astrocytoma and oligodendroglioma.
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PMID:A case of primary diffuse leptomeningeal gliomatosis predominantly involving the cervical spinal cord and mimicking chronic meningitis. 2227 77

A 5-year-old female presented to the emergency department with a 24-hour history of nausea, vomiting and mental status changes. Imaging demonstrated hemorrhage from a suprasellar mass consistent with an optic nerve glioma. The patient was taken to surgery for an open biopsy and hematoma evacuation. Pathology revealed a grade 3 fibrillary astrocytoma. She was subsequently treated with carboplatin and vincristine and is now 2 years out without evidence of recurrence. Apoplexy is a rare presentation for an optic pathway glioma. To the authors' knowledge, this is the first reported case in the pediatric population. Clinical presentation appears similar to the symptoms observed in pituitary apoplexy. Timely surgery should be directed at obtaining diagnostic tissue, prevention of long-term nervous system damage and evacuation of the hematoma where feasible.
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PMID:Opticochiasmatic apoplexy in a five-year-old. 2245 72

Single-agent sunitinib, an oral small molecule inhibitor of multiple tyrosine kinase receptors, was evaluated for treatment of patients with recurrent glioblastoma (GB) and anaplastic astrocytoma (AA). Fourteen AA and 16 GB patients, all previously treated with surgery, radiotherapy, and temozolomide, were enrolled in a prospective phase II study at either first or second relapse. Patients were treated with daily sunitinib for 4 consecutive weeks, followed by a 2-week break. For AA patients, the most common side effects were fatigue (86 %), diarrhea (43 %), hand-foot syndrome (36 %), neutropenia (36 %), thrombocytopenia (36 %), and nausea (29 %). In the GB cohort, the most common side effects were fatigue (56 %), diarrhea (44 %), neutropenia (31 %), and thrombocytopenia (25 %). Six of 14 (43 %) AA and 5 of 16 (31 %) GB patients experienced grade 3 or greater toxicities. Five patients discontinued study due to drug toxicities. There were no partial or complete responses in either cohort; 8/14 (57 %) AA and 5/16 (31 %) GB patients had stable disease at the first planned assessment. Progression-free survival at 6 months was 21.5 % (AA) and 16.7 % (GB). Median overall survival was 12.1 months (AA) and 12.6 months (GB). These results are comparable to those reported in the literature in patients treated with standard cytotoxic therapies. This is the largest reported trial of sunitinib in recurrent malignant astrocytic gliomas to date, as well as contains the largest AA cohort. Nonetheless, sunitinib did not demonstrate significant anti-glioma activity in patients with recurrent malignant astrocytic gliomas.
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PMID:A prospective phase II single-institution trial of sunitinib for recurrent malignant glioma. 2283 97

A multicenter, two stage phase II study, investigated irinotecan plus temozolomide in children with newly diagnosed high grade glioma. The primary endpoint was tumor response during a two-cycle treatment window, confirmed by external review committee. Patients received oral temozolomide 100 mg/(m(2) day) (days 1-5) and intravenous irinotecan 10 mg/(m(2) day) (days 1-5 and 8-12) for two 21-day cycles (three cycles for patients exhibiting objective tumor response). Standard treatment was then administered according to local investigator choice. In total 17 patients were enrolled and treated by local investigators. However, central pathology review found three patients did not have a diagnosis of high grade glioma and another four patients did not have evaluable disease according to independent central radiological review. The primary endpoint was based on the first ten evaluable patients as determined by the external review committee. Recruitment was stopped for futility after there were no complete or partial responses during the two-cycle treatment window in the first ten evaluable patients. Five patients had stable disease, and five progressed. Data for secondary endpoints including; time to tumor progression, time to treatment failure, and overall survival is reported. The safety profile of the treatment showed the combination was tolerable with two patients (11.8 %) having grade three nausea, and one (5.9 %) experiencing a grade four neutropenia, leading to permanent discontinuation from adjuvant treatment. Irinotecan plus temozolomide, although well tolerated did not improve outcome over historical controls in this setting.
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PMID:A phase II single-arm study of irinotecan in combination with temozolomide (TEMIRI) in children with newly diagnosed high grade glioma: a joint ITCC and SIOPE-brain tumour study. 2345 95

A 44-year-old man presented with the rare complication of remote cerebellar hemorrhage (RCH) after removal of a supratentorial glioma without the loss of a large volume of cerebrospinal fluid (CSF). He presented with severe headache, nausea, and vomiting for a few days, then he developed neurological deterioration including progressive disturbance of consciousness and left hemiparesis. Magnetic resonance imaging revealed a large tumor with intratumoral hemorrhage in the right frontal lobe that led to subfalcial and transtentorial herniation. The tumor was removed en bloc without excessive loss of CSF throughout the perioperative period. Although the level of consciousness remained unchanged from the preoperative level and no new neurological deficit was detected, routine postoperative computed tomography showed a bilateral RCH. Careful conservative therapy was provided and follow-up computed tomography demonstrated no further progression of hemorrhage. Compensatory acute engorgement of venous sinuses derived from the rapid decrease in intracranial pressure that occurred due to removal of the huge tumor might have caused cerebellar hemorrhagic venous infarction.
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PMID:Remote Cerebellar Hemorrhage after Removal of a Supratentorial Glioma without Perioperative CSF Loss: A Case Report. 2353 10

Pleomorphic xanthoastrocytoma (PXA) is a World Health Organization Grade 2 glioma that is uncommon (<1 % all adult gliomas) and seen primarily in children and young adults. PXA has been demonstrated to manifest the V600E BRAF mutation in nearly 70 % of all tumors, a mutation that constitutively activates the BRAF/MEK signaling pathway. Assess response and toxicity of a BRAF inhibitor, vemurafenib, in recurrent PXA manifesting the V600E mutation. Four adults [2 males; 2 female: median age 45 years (range 34-53)] with surgery, radiation and alkylator refractory recurrent PXA demonstrating the BRAF mutation (V600E) were treated with vemurafenib. A cycle of vemurafenib was defined as 4 weeks of continuous therapy. All toxicities seen were grade 2 and included arthralgia, photosensitivity, fatigue and nausea (1 patient each). The median number of cycles of therapy was 5 (range 2-10). Radiographic response was progressive disease in 1, stable disease in 2 and partial response in 1. Median progression free survival was 5 months (range 2-10 months). Median overall survival was 8 months (range 4-14 months). In this small retrospective series of select patients with recurrent PXA manifesting the BRAF V600E activating mutation, vemurafenib appears to have single agent activity with manageable toxicity. Confirmation in a larger series of similar patients is required.
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PMID:Salvage therapy with BRAF inhibitors for recurrent pleomorphic xanthoastrocytoma: a retrospective case series. 2375 28


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