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)

Interferons alpha and beta have been reported to cause tumor regression in a small proportion of patients with recurrent glioma. Eflornithine, an irreversible inhibitor of ornithine decarboxylase, reduces cellular polyamine levels and has also been reported to cause tumor regression in patients with recurrent anaplastic astrocytoma and glioblastoma multiforme. In vitro evidence suggests that interferon and eflornithine are synergistic. In this phase II trial, we investigated the combination of recombinant alpha interferon (36 x 10(6) units/m2 subcutaneously days 3 to 7) and eflornithine (2.25 g/m2 QID PO days 1 to 7) repeated every 28 days. All 29 patients entered in the study were evaluable for toxicity and efficacy. Toxicity consisted primarily of fever, chills, myalgia, weakness and fatigue as well as cortical dysfunction including somnolence, confusion, and exacerbation of underlying neurologic deficits. One patient died from cerebral herniation attributable to interferon. None of the patients experienced objective tumor regression. Seven patients (24%) were stable for more than six months, but the disease stability could also be explained by indolent underlying disease or inability to distinguish recurrent tumor from delayed radiation effects. Intermittent high-dose recombinant interferon alpha plus eflornithine demonstrated no definite antitumor effects in this trial.
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PMID:Phase II trial of recombinant interferon-alpha-2a and eflornithine in patients with recurrent glioma. 952 27

In modern clinical neuro-oncology, histopathological diagnosis affects therapeutic decisions and prognostic estimation more than any other variable. Among high-grade gliomas, histologically classic glioblastomas and anaplastic oligodendrogliomas follow markedly different clinical courses. Unfortunately, many malignant gliomas are diagnostically challenging; these nonclassic lesions are difficult to classify by histological features, generating considerable interobserver variability and limited diagnostic reproducibility. The resulting tentative pathological diagnoses create significant clinical confusion. We investigated whether gene expression profiling, coupled with class prediction methodology, could be used to classify high-grade gliomas in a manner more objective, explicit, and consistent than standard pathology. Microarray analysis was used to determine the expression of approximately 12000 genes in a set of 50 gliomas, 28 glioblastomas and 22 anaplastic oligodendrogliomas. Supervised learning approaches were used to build a two-class prediction model based on a subset of 14 glioblastomas and 7 anaplastic oligodendrogliomas with classic histology. A 20-feature k-nearest neighbor model correctly classified 18 of the 21 classic cases in leave-one-out cross-validation when compared with pathological diagnoses. This model was then used to predict the classification of clinically common, histologically nonclassic samples. When tumors were classified according to pathology, the survival of patients with nonclassic glioblastoma and nonclassic anaplastic oligodendroglioma was not significantly different (P = 0.19). However, class distinctions according to the model were significantly associated with survival outcome (P = 0.05). This class prediction model was capable of classifying high-grade, nonclassic glial tumors objectively and reproducibly. Moreover, the model provided a more accurate predictor of prognosis in these nonclassic lesions than did pathological classification. These data suggest that class prediction models, based on defined molecular profiles, classify diagnostically challenging malignant gliomas in a manner that better correlates with clinical outcome than does standard pathology.
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PMID:Gene expression-based classification of malignant gliomas correlates better with survival than histological classification. 1267 Sep 11

Several types of mass lesions may occur in the third and lateral ventricles. Typically they arise from the lining of the ventricular cavity or from contiguous structures, by extension into the ventricle. The authors describe two patients, each of whom presented with a different rare lesion of the ventricular system. The first was a 53-year-old woman with a history of hypertension who sustained a blunt traumatic injury to the occipital region and subsequently developed a progressively worsening right-sided headache. Radiological examinations over the next 2 years revealed an enlarged right lateral ventricle and, ultimately, a choroid plexus cyst in its anterior and middle third, near the foramen of Monro, which is a rare location for these lesions. The cyst was removed en bloc, and follow-up examinations showed a significant improvement in her headache and a minimal differences in size between right and left ventricles. The authors also describe a 57-year-old man with hypertension, diabetes mellitus, and an old mycardial infarct, who presented to an outside institution with a progressively worsening headache, generalized malaise, and loss of olfactory sensation. Diagnostic imaging revealed a 1.5-cm oval lesion centered in the lamina terminalis region, an open craniotomy was performed, and evaluation of a biopsy sample demonstrated the mass to be a chordoid glioma of the third ventricle, a recently described glioma subtype. Two days after surgery, he suffered a left parietal stroke and an anterior mycardial infarction. After convalescing, he presented to The University of Texas M. D. Anderson Cancer Center for radiotherapy and follow up; 7 months later he was readmitted complaining of headache, short-term memory loss, and worsening confusion and disorientation. Neuroimaging revealed progression of the tumor (now 2 cm in diameter), which was removed by gross-total resection. His headache resolved immediately, and 2 months later his only complaint was of episodes of confusion. Three weeks later he died of a massive myocardial infarction. These two patients represent the sixth case of an adult with a choroid plexus cyst in the anterior lateral ventricle and the 19th case of an adult with a chordoid glioma of the third ventricle, respectively.
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PMID:Choroid plexus cyst and chordoid glioma. Report of two cases. 1672 23

The etiology of cerebral hemorrhage is not always easy to diagnose and modern neuroimaging methods may be deceptive. The authors report on a 48-year-old previously healthy male admitted to hospital with a first-time severe generalized epileptic seizure. The patient presented with aphasia, confusion, headaches, nausea, right hemianopia and early papilledema. CT revealed an intracerebral hematoma in the left occipital lobe but MRI and MRA failed to provide any further insight into the etiology of the hemorrhage. Due to persisting intracranial hypertension, the patient underwent neuroendoscopic removal of the hematoma. After blood clots were evacuated, pathologic vascularity was found. Tissue samples were collected from the walls of the hematoma cavity. Neoplastic cells adjacent to the blood vessels were identified in one of the samples. The patient was reoperated and a glioma-like tumor was removed. The diagnosis of cerebral intratumoral hemorrhage is difficult especially when there are no previous clinical signs of a tumor. Neuroendoscopy may speed up the diagnostic process which usually extends past resorption of the haematoma and the technique may be an alternative to stereotactic surgery.
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PMID:[Endoscopic method in etiological diagnosis of spontaneous intracerebral hemorrhage. Case report]. 1687 45

Interferon-beta (IFN-beta) is a pleiotropic cytokine with antitumoral activity. In an effort to improve the therapeutic index of IFN-beta by providing local, sustained delivery of IFN-beta to gliomas, the safety and biological activity of a human IFN-beta (hIFN-beta)-expressing adenovirus vector (Ad.hIFN-beta) was evaluated in patients with malignant glioma by stereotactic injection, followed 4-8 days later by surgical removal of tumor with additional injections of Ad.hIFN-beta into the tumor bed. Eleven patients received Ad.hIFN-beta in cohorts of 2 x 10(10), 6 x 10(10), or 2 x 10(11) vector particles (vp). The most common adverse events were considered by the investigator as being unrelated to treatment. One patient, who was enrolled in the cohort with the highest dose levels, experienced dose-limiting, treatment-related Grade 4 confusion following the post-operative injection. Ad.hIFN-beta DNA was detected within the tumor, blood, and nasal swabs in a dose-dependent fashion and hIFN-beta protein was detectable within the tumor. At the highest doses tested, a reproducible increase in tumor cell apoptosis in post-treatment versus pre-treatment biopsies with associated tumor necrosis was observed. Direct Ad.hIFN-beta injection into the tumor and the surrounding normal brain areas after surgical removal was feasible and associated with apoptosis induction.
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PMID:A phase I trial of Ad.hIFN-beta gene therapy for glioma. 1818 Jul 70

Chordoid glioma of the third ventricle constitutes a rare, very recently recognized histological entity. Most reports of this neoplasm, focused on its distinct histological features, have hypothesized about a probable origin of the lesion at the third ventricle floor and/or the lamina terminalis. We report on a new case, presenting neuroradiological and intraoperative pictorial evidences of the tumoral attachment, limited to the chiasm-lamina terminalis junction. A 53-year-old woman debuted with acute symptoms of obstructive hydrocephalus, visual disturbances and confusion. MRI investigation showed a large solid-cystic third ventricle mass bulging through the lamina terminalis and ventricular floor. After placing a ventriculoperitoneal shunt, the tumor was completely removed through a trans-lamina terminalis approach. A tight tumoral attachment to the junction of the posterior chiasm to the lamina terminalis was identified and dissected. No other adhesions to the third ventricle boundaries were found. A chordoid glioma was diagnosed on histological examination. One year after the surgical procedure the patient does not present new neurological deficits, and there are no signs of tumoral regrowth on the follow-up postoperative MRI. Chordoid glioma should be included in the differential diagnosis of third ventricle tumors. Preoperative neuroradiological suspicion of this lesion should alert the neurosurgeon about the presence of a tight tumoral adherence at the level of the chiasm-lamina terminalis junction. The trans-lamina terminalis approach provides a suitable route for an early control of this attachment under direct vision, allowing a safe dissection of the mass from the third ventricle.
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PMID:Chordoid glioma of the third ventricle attached to the optic chiasm. Successful removal through a trans-lamina terminalis approach. 1859 29

A 39-year-old man presented with multiple basaloid follicular hamartomas involving the right side of his body in a systematized pattern following Blaschko's lines. His right leg was 22.5 cm shorter than the left, and rudimentary pre-axial polydactyly was noted on the left hand and the right foot. The teeth of the right maxilla were hypoplastic. DNA analysis of blood lymphocytes and fibroblasts from lesional skin did not reveal any mutation in the Patched gene. On account of this case and of 8 similar cases found in th e literature, the spectrum of a distinct syndrome is delineated. Ipsilateral extracutaneous defects include cervical ribs, polydactyly, malformed thumb and disproportionate overgrowth or deficient growth of limb bones; dental anomalies in the form of anodontia, hypodontia or ameloblastoma; and cerebral defects such as mental retardation, unsteady gait, meningioma and optic glioma. The cutaneous lesions of this syndrome should not be called "basal cell naevus" as this will lead to continuing confusion with Gorlin syndrome. The molecular basis of the disorder remains to be elucidated.
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PMID:Segmentally arranged basaloid follicular hamartomas with osseous, dental and cerebral anomalies: a distinct syndrome. 1870 10

CCN proteins are key regulators of signaling pathways that are essential for the control of normal life, from birth to death. As such, they make use of their unique mosaic structure to interact with several other regulatory proteins and ligands that control the fate of living cells. The various functions attributed to the CCN proteins may sometimes appear contradictory, but this situation reflects the complexity of the multimolecular scaffolds in which CCN proteins are engaged and the critical impact of the microenvironment that dictates the bioavailability of the elementary building blocks. CCN3 is one of the best examples of a CCN protein showing biological properties which may at first glance appear opposite or contradictory. Indeed, CCN3 acts both as a tumor suppressor and is associated with higher metastatic potential. Furthermore, the physical interaction of CCN3 with VEGF and its potential antiangionenic activity in glioma cells are in apparent contradiction with its proangiogenic activity in rabbit cornea. In this communication, I am revisiting the observations that led us to these apparent contradictions. After pointing out how the methodologies that were employed might have contributed to the confusion, I briefly discuss the dual biological activities of CCN3 in the context of tumor cell engineering and survival prognosis.
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PMID:CCN3: Doctor Jekyll and Mister Hyde. 1878 88

Leptomeningeal (LM) dissemination arises rarely in patients with low-grade gliomas. Few cases of LM dissemination of spinal cord low-grade gliomas have been reported in children. Here, we report a case of an intramedullary spinal cord low-grade glioma occurring in an adult presenting by LM dissemination. A 57-year-old man presented with a history of headaches and visual acuity deterioration. Neurological examination revealed mental confusion, decreasing left limb strength and left positive Babinski sign. Lumbar puncture showed low CSF glucose, high CSF protein and no tumor cells. Spinal MRI revealed an intramedullary spinal lesion with a diffuse leptomeningeal enhancement. A spinal meningeal biopsy showed arachnoiditis with isolated atypical cells which were cytokeratin immunopositive. A differential diagnosis was proposed between arachnoiditis close to an infectious process or close to meningeal carcinomatosis. An intramedullary biopsy was programmed but the patient died 1 month after biopsy. The post-mortem examination showed thickened leptomeninges and an ill-circumscribed intramedullary grey lesion. Histological examination revealed a low-grade glial neoplasm composed of monomorphous spindle "piloid" cells with diffuse dissemination to leptomeninges. Glial fibrillary acidic protein immunostaining showed scattered immunoreactive cells. Clinical and pathological features of this case are unusual. Pathogenesis and prognosis of low-grade gliomas with LM dissemination are poorly understood. This case, like cases occurring in children, cannot easily be classified in the present WHO system of classification of CNS tumors. Spinal low-grade gliomas with LM dissemination may represent a possible distinctive clinico-pathological entity.
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PMID:Leptomeningeal dissemination of low-grade intramedullary gliomas: about one case and review. 1912 71

We report four cases of biopsy-proven B-cell-rich primary angiitis of the central nervous system (PACNS). The mean age of the patients was 29 years (range, 23-37 years). The patients suffered from unilateral weakness (n = 2), seizure (n = 1), and hypersomnia, anorexia and confusion (n = 1). The vital signs and the results of laboratory tests were within normal limits in all the four cases except erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). ESR was elevated in one patient and CRP was elevated in two patients. The magnetic resonance imaging (MRI) scans revealed single (n = 2) or multiple (n = 2) irregularly enhancing lesions. Radiological studies initially indicated tumors such as glioma (n = 2) or lymphoma (n = 1), except in one case, in which the radiological analysis indicated vasculitis or demyelinating disease. All the cases involved both medium-sized (50-250 microm in diameter) and small-sized vessels (20-49 microm in diameter). The vascular, perivascular and parenchymal lymphocytes were polymorphous; however, CD20-positive B-cells were predominated in blood vessels while the CD8-positive T-cells infiltrated predominantly in brain parenchyma. Therefore, our patients revealed B-cell dominant lymphocytic vasculitis. Two patients who underwent active treatment (corticosteroid alone or with cyclophosphamide) showed remarkable clinical and radiological improvement but two patients still have initial neurological symptoms, namely, confusion and newly developed seizures, respectively, during the 19-101-month follow-up periods; this effect can be attributed to irreversible brain damage. Therefore, although early brain biopsy may be associated with histopathologic diagnostic pitfalls, it is a mandatory procedure for obtaining a confirmative diagnosis as well initiating early therapy, thereby reducing brain damage.
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PMID:B-cell dominant lymphocytic primary angiitis of the central nervous system: four biopsy-proven cases. 1973 59


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