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 present a 24-year-old patient with multiple chondromas of both hands, the pelvis, the left leg, and an associated brain stem glioma. There was no evidence of hemangioma or dyschromia, and the condition was diagnosed as Ollier's disease, a special type of dyschondroplasia like Maffucci's syndrome and Kast's disease. An increased overall risk for development of malignant skeletal and nonskeletal tumors is associated with Maffucci's syndrome. The risk of malignant degeneration is lower in Ollier's disease. A glioma in the pons and the right lobe of the cerebellum was found in this patient. The literature describes an association with gliomas in only 12 cases of dyschondroplasia and an infratentorial localization in just one case. Signs of malignancy were histologically confirmed in 7 cases without significant preponderance of any one type. Our patient had a low-grade brain stem astrocytoma with fibrillar and gemistocytic components. A stereotactic serial biopsy made it possible to rule out malignant degeneration. Stereotactic brain tumor biopsy as a routine neurosurgical procedure is particularly valuable for deep space-occupying processes and forms the basis for therapy. In the present case, irradiation was not recommended.
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PMID:A case of dyschondroplasia associated with brain stem glioma: diagnosis by stereotactic biopsy. 223 48

Carboplatin is one of a series of cisplatin analogs now undergoing clinical investigation. Phase I and II trials in adults demonstrate activity in a number of human cancers and less toxicity than might be expected with the parent compound. This phase I trial was undertaken to establish the maximum tolerated dose and the recommended phase II dose in children treated by a 1-hour iv infusion every 4 weeks. Twenty-nine patients with recurrent or progressive tumor were entered in this study at the Children's Hospital of Los Angeles and Children's Memorial Hospital in Chicago between April 12, 1983, and November 27, 1984. Beginning with a dose of 350 mg/m2 (about 80% of the adult phase II dose), we escalated the dose in groups of patients to 670 mg/m2; dose-limiting myelosuppression was encountered at this dose. Fifty-seven infusions are at least partially evaluable for toxicity. Asymptomatic hypomagnesemia, hypocalcemia, and ototoxicity were observed infrequently, and nausea and vomiting were mild. One patient with a mixed glioma of the posterior fossa achieved a good partial response lasting 9 months. Stable disease for greater than or equal to 6 months was observed in three patients: one each with ependymoma, brain stem glioma, and spinal cord astrocytoma. The recommended pediatric phase II dose is 560 mg/m2 given as a 1-hour iv infusion every 4 weeks.
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PMID:Pediatric phase I trial of carboplatin: a Childrens Cancer Study Group report. 331 95

Magnetic resonance (MR) and CT examinations of 26 patients with the established or clinically suspected diagnosis of brain stem glioma were reviewed. Eleven tumors were seen on both MR and CT. The entire extent of the abnormality was better outlined on MR, although CT was more advantageous in demonstrating cystic components and calcium deposition. Magnetic resonance and CT depicted focal intratumoral hemorrhage equally. Magnetic resonance was found to be particularly suitable to follow up the progression or regression of the disease. Of particular interest were two patients with evidence of aqueductal obstruction but normal CT appearance of the midbrain; the causative abnormality, believed to be a glioma, was clearly shown by MR imaging. In nine patients the normal appearance was helpful to exclude the possibility of a brain stem glioma. Thus far, results have shown 100% sensitivity (true positive ratio) and specificity (true negative ratio) with MR in the evaluation of brain stem gliomas. It is concluded that MR imaging should be the examination of choice and could be the definitive screening procedure in patients with suspected brain stem glioma.
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PMID:MR imaging of brain stem gliomas. 397 48

We report the findings at follow-up in 67 consecutive children with central nervous system tumors treated over a 5-year-period at a single institution. The diagnoses were supratentorial astrocytoma (n = 12), cerebellar astrocytoma (n = 10), ependymoma (n = 9), medulloblastoma (n = 9), brain stem glioma (n = 6), optic pathway glioma (n = 5), and others (n = 16). The survival rates were 83% for supratentorial astrocytomas at a median of 46.5 months, 90% for cerebellar astrocytomas and 55% for ependymomas at 40 months, respectively, 55% for medulloblastomas at 22 months, 33% for brain stem gliomas at 23 months, and 80% for optic pathway gliomas at 49 months. With regard to neurological sequelae, 13 patients were treated for epilepsy, 13 patients had mild to moderate neurological deficits, and 4 patients were severely disabled. Seventeen of 37 tested patients performed below average on formal neuropsychometric testing, one-fourth attended special education courses, and at least one-fourth suffered from behavioral and adjustment problems.
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PMID:Follow-up and quality of survival of 67 consecutive children with CNS tumors. 784 33

A case of brain stem glioma following radiotherapy for brain tumor is presented. A 44-year-old female was admitted with chief complaints of gait disturbance and disturbed consciousness. She had received radiation therapy with 60Gy following resection of a right temporal astrocytoma 20 years before, but until admission this time follow up CT scan had shown no regrowth. On admission CT scan and MRI showed an enhanced mass (2 x 2.5cm) in the mid-brain. Tumor biopsy was performed using the subtemporal approach. The histological diagnosis showed a fibrillary astrocytoma grade III. The patient received postoperative synchronized chemotherapy but expired 8 months after admission. The growth of glioma appeared in the previous radiation field after a long latent period. This case should be considered not as a case of the regrowth of a tumor but as a case of a radiation induced tumor. Although 77 cases of gliomas following radiation for intracranial tumor have been reported, this case of radiation-induced brain stem glioma is extremely rare.
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PMID:[Brain stem glioma induced by radiotherapy: report of a case]. 787 36

In a 20-year-old female patient with a brain stem glioma in the medulla oblongata in association with paraplegia and respiratory paralysis, bradyarrhythmias such as sinus bradycardia and sinus arrest repeated sporadically and transiently, but soon subsided as radiotherapy was being delivered to the glioma in the medulla oblongata. The bradyarrhythmias were differentiated from sick sinus syndrome in their sporadic and transient character. The patient responded normally to atropine, isoproterenol, and phenylephrine. Parasympathetic nerve reflexes induced by Aschner's, Czermak's, and Valsalva's maneuvers and sympathetic nerve reflex induced by change of body position were within normal limits. Although EKG abnormalities associated with diseases of the central nervous system are frequently due to intracranial hypertension and/or irritation of the hypothalamus, the bradyarrhythmias in this patient were possibly due to vagus stimulation caused by the glioma in the medulla.
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PMID:[A case of sporadic and transient bradyarrhythmias in a patient with a glioma in the medulla oblongata]. 835 39

A case of brain stem glioma, presenting with impairment of contralateral pain-temperature sense of ascending nature, is reported. A 38-year-old woman with documented neurofibromatosis type 1 (NF-1) was admitted to our hospital for treatment, complaining of diminished pain-temperature sense in the left lower extremity. On admission, the symptom was first evaluated to be due to cervical myelopathy although motor involvement was absent. An MRI, myelography and CT-myelography of the cervical spine were done, demonstrating no abnormality. Shortly after the admission the sensory impairment progressed to the upper chest level and then to the upper extremity on the left. Because of her documented NF-1 a brain CT was checked, revealing a small mass with ring enhancement in the dorsal midbrain on the right. On MRI, the tumor location was at the right dorsolateral tectal region of superficial situation. Under the diagnosis of midbrain glioma the tumor was partially removed by the occipital transtentorial approach. At operation, the tumor has grown intraaxially, having pinkish-gray color and central necrosis. Histologically the tumor was diagnosed as glioblastoma multiforme. Postoperatively she presented a definite improvement of the sensory impairment in the reverse order, that is from upper extremity and then to the lower extremity. Progression and post-treatment improvement of the impaired pain-temperature sense in this case suggested that the topography and lamination of the lateral spinothalamic tract might be present even in the dorsal midbrain, namely the posterior-superficial layer to be sacral segment and the anterior-deep layer to be cervical one.
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PMID:[Brain stem glioma presenting with contralateral sensory impairment of ascending nature]. 919 96

Between 1988 and 1997, 28 children have had iodine-125 implants for CNS tumors performed in our institution. Ten had stereotactic implantation in the brain stem region, and nine had the diagnosis of brain stem glioma (8 diffuse pontine, 1 midbrain tumor). Their ages ranged from 1.8 to 12 years. All patients had histological confirmation of malignancy (7 high-grade glioma, 2 low-grade glioma, 1 PNET). Diffuse pontine glioma patients received external beam radiation (50 Gy) followed by a fractionated stereotactic boost of 3 Gyx4 fractions. After 4-6 weeks, patients were reevaluated for stereotactic interstitial I-125 therapy. The planned implant dose was 82.9 Gy to the enhancing tumor (4 cGy per h). Preliminary results indicated that no surgical complications were associated with the catheter placement. Four patients have died (7-9 months from diagnosis) and four patients remain alive (5-38 months from diagnosis, median 10 months). Two autopsies confirmed the presence of progressive glioblastoma multiforme and intralesional necrosis. In one patient who received an implant alone for midbrain LGA, necrosis without tumor was found on biopsy after 36 months. He was successfully treated with hyperbaric oxygen therapy. The implementation of permanent I-125 implants appears to have a role in the management of pediatric CNS malignancy. This study confirms the results of previous reports regarding the safety of stereotactic interstitial brachytherapy in the brain stem. Tumor control for patients with high-grade brain stem glioma remains poor even with high focal radiation doses.
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PMID:Permanent I-125 brain stem implants in children. 984 Mar 81

KRN8602(MX2) is a newly developed morpholino-anthracycline that has been found to cross the blood-brain barrier and be distributed in brain tissue after intravenous administration and to be effective against human glioma cells and the intracerebrally transplanted tumors in vivo. In order to confirm these promising preclinical observations clinically, we performed a phase II trial of KRN8602 in patients with recurrent malignant glioma. The 44 patients enrolled received at least 2 cycles of KRN8602 35 mg/m2/day at 3-4 week intervals by intravenous bolus. Of the 44 patients, 37 could be evaluated for response, and 39 for toxicity. One patient with anaplastic astrocytoma had a complete response (1/37, 3%), and 2 patients with anaplastic astrocytoma and 1 with brain stem glioma had a partial response (3/37, 8%). The overall response rate was 11% (4/37). All patients who responded had received prior chemotherapy that included nitrosoureas. No response was observed in the patients with glioblastoma. Myelosuppression was moderately severe, with 72% of patients developing grade 3 or 4 leukopenia. Severe nausea/vomiting was observed in 31% of the patients. No severe cardiotoxicity was observed. The results indicate that KRN8602 has modest activity against recurrent malignant glioma with relatively severe, but manageable toxicity. It seems to be worthwhile to further assess the efficacy and toxicity of KRN8602 against malignant glioma, which is generally less sensitive to chemotherapy.
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PMID:A phase II study of KRN8602(MX2), a novel morpholino anthracycline derivative, in patients with recurrent malignant glioma. 1042 Oct 76

Despite surgery and adjuvant cytotoxic therapy anaplastic astrocytoma, glioblastoma and diffuse intrinsic brain stem glioma continue to have dismal prognosis. Differentiation induction is a new approach taking into account that malignant glioma cells share many features with immature glial progenitor cells that are capable of terminal differentiation. The concept of differentiation therapy is currently evaluated for several pediatric malignancies with or without multimodal standard therapy. Valproic acid (VPA) is a branched chain fatty acid that is able to inhibit proliferation of neuroectodermal cells and to induce these cells along neuronal or glial lineage. Preclinical studies have shown that VPA inhibits growth of human and rodent glial tumor cells in vitro and induces a distinct mature glial phenotype. In addition, growth of human neuroblastoma cells is inhibited in vitro and in vivo and exhibits marked evidence of differentiation. Treatment of neuroblastoma and glioma cells with VPA was accompanied by changes of surface molecule expression that enhance immunogenicity and reduce their capability to metastasize. The antitumoral effects observed in preclinical studies were reached at concentrations that are readily achieved in patients treated with VPA for epilepsy. Epilepsy patients receiving VPA have significantly enhanced hemoglobin F levels, supporting the hypothesis that nontoxic levels of VPA can induce cellular differentiation. Broad clinical experience with VPA and its low toxicity encourage the evaluation of VPA in patients that have been submitted to postoperative combined chemo- and radiotherapy for pediatric malignant glioma.
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PMID:Valproic acid for the treatment of pediatric malignant glioma. 1047 71


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