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)

Eflornithine (DFMO), an irreversible inhibitor of ornithine decarboxylase, and mitoguazone (MGBG), a competitive inhibitor of S-adenosylmethionine decarboxylase, were evaluated in a phase I-II study for patients with primary recurrent malignant brain tumors. All patients had failed prior radiation therapy and most had also failed prior chemotherapy. Two dose schedules were used, with the second schedule (Group II) a modification of the first schedule (Group I). The Group II schedule, with different dose levels, was better tolerated than the Group I schedule. Gastrointestinal and myelotoxicity were dose-limiting in most patients, and tinnitus was dose-limiting in two patients. Nineteen of 33 evaluable patients had anaplastic gliomas, in whom response was observed in 21%, stable disease in 53%, and immediate progression after one course of therapy in 26%. Of six patients with glioblastoma multiforme, two had brief stabilization of disease. An additional patient with brainstem glioma and ependymoma also had disease stabilization. Four patients with medulloblastoma, a spinal cord mixed glioma, and one with oligodendroglioma failed DFMO-MGBG. Based on this study, we believe that a combination of DFMO and MGBG is well-tolerated and deserves further evaluation for patients with anaplastic gliomas, particularly those that appear to be biologically slow growing.
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PMID:Phase I-II study of eflornithine and mitoguazone combined in the treatment of recurrent primary brain tumors. 310 81

Pontine gliomas have been considered to be out of indication for operative treatment. However, in case of a cystic type, evacuation of the cyst alone can possibly extend the survival time of the patient. Since the advent of high resolution CT the nature of the pontine tumor, whether cystic or solid, can be easily differentiated, and cystic ones subjected to operation will be increasing in number. We report a case of cystic pontine glioma associated with von Recklinghausen's disease. The patient showed a remarkable improvement in her neurological status after evacuation of the cyst. A 16-year-old girl was admitted to our clinic with complaints of tinnitus and hearing difficulty of the left ear, progressive gait disturbance and double vision. Neurological examination revealed a sensory disturbance on the left side of the face, left abducens palsy, left facial paresis, left deafness, left cerebellar ataxia, right hemiparesis and right hemisensory disturbance excluding the face. Signs and symptoms of increased intracranial pressure were absent. There were many cafe-au-lait spots and several subcutaneous nodules. CT scan demonstrated a cystic lesion with a mural nodule in the left cerebello-pontine angle. The patient underwent left suboccipital craniectomy, and a puncture of the cyst between the trigeminal and facial-acoustic nerves which were displaced dorsally yielded yellowish fluid. The content of the cyst was evacuated and its wall was widely opened. After the operation the patient showed a remarkable improvement in her neurological deficits only with left deafness remained unchanged. Histological examination showed anaplastic astrocytoma. She was discharged after irradiation of 5000 rads.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cystic pontine glioma associated with von Recklinghausen's disease--report of a case]. 643 42

A retrospective review of 314 cases of contrast-enhanced magnetic resonance imaging (MRI) of the posterior fossa and internal auditory canals was carried out correlating the presenting symptoms with the scan findings. 7.2% of the cases showed findings on the MRI which could account for the patients' symptoms. Patients with sensorineural hearing loss were more likely to have a positive scan than those presenting with vertigo and/or tinnitus without hearing loss. Acoustic schwannoma was the most common lesion detected. Labyrinthine lesions e.g. cochlear schwannoma, labyrinthitis, congenital labyrinthine abnormality, and central lesions e.g. multiple sclerosis, brainstem glioma were some of the other lesions detected.
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PMID:Contrast-enhanced magnetic resonance imaging of the internal auditory canals and posterior fossa. 966 3

Although the efficacy of the nitrosourea-based combination chemotherapy procarbazine, N-(2-chloroethyl)-N'-cyclohexyl-N-nitrosurea, and vincristine (PCV) has been previously demonstrated in the setting of anaplastic/intermediate-grade gliomas, the benefit for glioblastoma patients remains unproven. In the current study, we sought to determine whether the addition of alpha-difluoromethylornithine (eflornithine), an inhibitor of ornithine decarboxylase, which has shown encouraging results in the setting of recurrent glioma patients, to a nitrosourea-based therapy (PCV) would constitute a more effective adjuvant therapy in the treatment of glioblastoma multiforme patients in the postradiation therapy setting. Following conventional radiation therapy, 272 glioblastoma (GBM) patients were randomized to receive either alpha-difluoromethylornithine-PCV (DFMO-PCV; 134 patients) or PCV alone (138 patients), with survival and time to tumor progression being the primary endpoints. The starting dosage of DFMO was 3.0 g/m2 p.o. q8h for 14 days before and after treatment with N-(2-chloroethyl)-N-cyclohexyl-N-nitrosurea; PCV was administered as previously described1. Clinical and radiological (Gadolinium-enhanced MRI) follow-ups were nominally at the end of each 6 or 8 week cycle (PCV at 6 weeks; DFMO-PCV at 8 weeks). Laboratory evaluations for hematologic and other adverse effects were at 2 week intervals. There was no difference in median survival or median time-to-tumor progression between the two treatment groups, as measured from day of commencement of postradiotherapy chemotherapy [MS (months): DFMO-PCV, 10.5; Overall survival, as measured from time of tumor diagnosis at first surgery, was 13.3 and 14.2 months at the median and 6.2 and 8.7% at 5 years, respectively, for the DFMO-PCV and PCV arms. The treatment effect was unchanged after adjustment for age, performance status (KPS), extent of surgery, and other factors using the multivariate Cox proportional hazard model. Adverse effects associated with DFMO consisted of gastrointestinal (diarrhea nausea/vomiting), cytopenias, and minimal ototoxicity (limited to tinnitus) at the dose range tested. The addition of DFMO to the nitrosourea-based PCV regimen in this phase III study demonstrated no additional benefit in glioblastoma patients, underscoring the resistance of glioblastoma multiforme tumors to alkylating agents. For patients with anaplastic (intermediate grade) gliomas, in which the previously demonstrated benefit of post-radiation chemotherapy is more substantial, the evaluation of DFMO-PCV vs. PCV is still ongoing and hopefully will yield more encouraging results.
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PMID:Phase III randomized study of postradiotherapy chemotherapy with alpha-difluoromethylornithine-procarbazine, N-(2-chloroethyl)-N'-cyclohexyl-N-nitrosurea, vincristine (DFMO-PCV) versus PCV for glioblastoma multiforme. 1105 Dec 33

The aim of this study was to describe adult patients' experiences of falling ill and being diagnosed with low-grade glioma. Information concerning such experiences is lacking in the literature. The study population were adults identified within a well-defined population. Interviews were conducted with 27 patients. The interview texts were analyzed using inductive content analysis. Illness onset was described as a sequence of events. Nineteen patients narrated rapid onset and 8 patients prolonged onset. The most commonly described symptoms in both types of onset were headache, epileptic seizures, vomiting, and vision changes. Racing thoughts, depression, and tinnitus were 3 of the more uncommon symptoms. The most prominent negative experiences regarding healthcare included disrespectful encounters and a lack of opportunity for participation. The salient negative life-situation consequences included a lack of social support and attitudes expressing a lack of understanding. However, to some extent, positive experiences also emerged in the interviews concerning healthcare and life situation despite the onset of the illness. In conclusion, most of the patients in the study experienced the illness onset as stressful. Healthcare staff need particular knowledge to understand the vulnerability of the patient in the onset of low-grade glioma.
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PMID:Experiences of onset and diagnosis of low-grade glioma from the patient's perspective. 1700 16

A rare case of neurosarcoidosis presenting as an isolated quadrigeminal plate mass without systemic manifestation of this disease is reported. This 26-year-old man presented with symptoms of acute intracranial hypertension including headache, morning vomiting as well as a right homonymous hemianopsia. Magnetic resonance imaging (MRI) showed an expansive tectal mass causing hydrocephalus secondary to an aqueductal obstruction. An external ventricular drainage was inserted and the mass, postulated to be a glioma, was removed through an occipital transtentorial craniotomy. Histopathological examination revealed numerous sarcoid granulomas. Postoperative course was relevant for bilateral hypoacusis and tinnitus, blurred vision, bilateral palpebral ptosis and bilateral internuclear ophthalmoplegia. Chest X-ray was normal. Postoperative thoracic computed tomography (CT) scan showed mediastinal adenopathies. Lung function tests were normal. Angiotensin converting enzyme (ACE) cerebrospinal fluid (CSF) blood ratio was normal. Postoperative treatment and follow-up included corticosteroids, serial lung function tests and cerebral MRI. Neurosarcoidosis may present with protean clinical manifestations and unusual radiological features. This rare diagnosis has to be kept in mind when facing isolated intracerebral mass lesions.
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PMID:Neurosarcoidosis presenting as an isolated mass of the quadrigeminal plate. 1863 66

Glioblastoma (GBM) of the internal auditory canal (IAC) is exceedingly rare, with only 3 prior cases reported in the literature. The authors present the fourth case of cerebellopontine angle (CPA) and IAC GBM, and the first in which the lesion mimicked a vestibular schwannoma (VS) early in its natural history. A 55-year-old man presented with tinnitus, hearing loss, and imbalance. MRI identified a left IAC/CPA lesion measuring 8 mm, most consistent with a benign VS. Over the subsequent 4 months he developed facial weakness. The tumor grew remarkably to 24 mm and surgery was recommended; the main preoperative diagnosis was malignant peripheral nerve sheath tumor (MPNST). Resection proceeded via a translabyrinthine approach with resection of cranial nerves VII and VIII, followed by facial-hypoglossal nerve anastomosis. Intraoperative frozen section suggested malignant spindle cell neoplasm, but final histopathological and molecular testing confirmed the lesion to be a GBM. The authors report the first case in which absence of any brainstem interface effectively excluded a primary parenchymal tumor, in particular GBM, from the differential diagnosis. Given the dramatic differences in treatment and prognoses between malignant glioma and MPNST, this case emphasizes the importance of surgical intervention on an aggressively growing lesion, which provides both the best probability of local control and the critical tissue diagnosis.
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PMID:Glioblastoma of the cerebellopontine angle and internal auditory canal mimicking a peripheral nerve sheath tumor: case report. 3057 79