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 report a case of brain surface angiocentric glioma in a 6-year-old Japanese boy with medically intractable partial epilepsy. MRI showed somewhat ill-defined high-signal lesions on fluid-attenuated inversion recovery and T2-weighted imagings of the right occipitoparietal cortex. At surgery, a yellowish tumor was localized in the superficial cortex. Histologically, the tumor was predominantly composed of elongated astrocytic cells forming rings around blood vessels. Tumor cells circumferential to vessels predominanted in low cellurarity areas, whereas radial alignment with perivascular pseudorosettes was observed in more cellular regions. These perivascular pseudorosettes closely resembled those of ependymoma. The tumor cells showed variable cytoplasmic immunoreactivity with GFAP. These findings were more likely monomorphous angiocentric glioma, which was first described by Wang et al. in 2005. However, in our case the tumor had a small foci of polymorphous appearance and a comparatively high MIB-1 labeling index (8%). Therefore, the present case may be an atypical form of monomorphous angiocentric glioma. However, no de novo anaplastic monomorphous angiocentric glioma similar to our case has yet been reported in the literature. It remains to be determined whether the behavior of monomorphous angiocentric glioma is an example of benign biological characteristics or whether it more closely resembles a low-grade malignant tumor.
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PMID:Brain surface spindle cell glioma in a patient with medically intractable partial epilepsy: a variant of monomorphous angiocentric glioma? 1817 12

Astroblastoma is a historically traded microscopic diagnosis to denote a rare neuroepithelial tumor of uncertain nosology, involving a distinctive pattern of pseudorosette arrangement of neoplastic cells. While displaying some glial properties, the latter shall not - by definition - be either reducible to or part of any conventional glioma type. We report on clinicopathologic correlations in a case of astroblastoma involving an extensive rhabdoid phenotype of tumor cells. The male patient was operated on at the age of 53 and 59 years for a left parietal tumor measuring 5.8 cm in diameter at the first presentation. On magnetic resonance imaging and angiography, both the primary and its recurrence were discrete, highly vascularized, and contrast-enhancing. The second surgery was complemented with radiotherapy of 66 Gy, followed by chemotherapy with Temozolomide. Twelve years into clinical history, the patient has stable minimal residual disease at the age of 65. A review of pathology samples from both surgeries showed well-differentiated astroblastoma according to current standards, with an MIB-1 labeling index of 1% and 4%, respectively. Neither of the specimens involved cellular anaplasia, overt mitotic activity, microvascular proliferation, or palisading necrosis. Most tumor cells harbored paranuclear filamentous rhabdoid inclusions that were immunostained for vimentin and, in part, also for GFAP. No polyantigenic reactivity was observed. This example contributes another facet to the spectrum of the so-called composite rhabdoid tumors. Involving a low-grade parent neoplasm, it also further substantiates the incipient perception that the rhabdoid phenotype neither is a peculiar but nonspecific convergence point of anaplastic evolution, nor are such lesions indiscriminately bound for a relentless course.
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PMID:Astroblastoma with rhabdoid features and favorable long-term outcome: report of a case with a 12-year follow-up. 1828 55

We describe diffuse glioma-like infiltrates in excised tubers in five out of forty Tuberous sclerosis complex (TSC) patients undergoing excision of a tuber at our institution within the last 10 years. All patients presented with refractory seizures. Resection specimens from four patients had the pathognomonic histologic features of neuroglial hamartomas (tubers) and in one case there was cortical microdysgenesis lacking cells typical of TSC. All lesions were associated with an infiltrate of atypical, mostly elongate, glioma-like small cells, which were immunoreactive for GFAP in three, and pS6 (a marker for activity of the mTOR pathway), in two cases. MAP-2 and CD34, were negative and MIB-1 (Ki67) immunostains ranged from <1-21%. Array-based comparative genomic hybridization revealed that these proliferative phenomena were associated with 21 different copy number aberrations in comparison with a tuber without atypical infiltrates. Postoperatively (follow-up period ranging from 8 to 34 months) none of the patients have any evidence of a glioma. We report that tubers resected for treatment of seizures are sometimes associated with glioma-like lesions, which are indistinguishable from infiltrating gliomas by morphology and immunohistochemistry. Genomic analysis with SNP arrays revealed copy number changes which may be associated with the pathogenesis of such infiltrates.
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PMID:Glioma-like proliferation within tissues excised as tubers in patients with tuberous sclerosis complex. 1858 Nov 25

The objective of the present study was an evaluation of the incidence and risk factors for erroneous histopathological diagnosis of low-grade glioma after stereotactic biopsy. Twenty-eight tumors diagnosed as low-grade glioma after stereotactic biopsy and surgically resected thereafter were analyzed. There were 13 astrocytomas, 7 oligodendrogliomas, and 8 mixed gliomas. All neoplasms had a lobar location. Seven tumors had contrast enhancement on MRI. The number of tissue samples obtained during stereotactic biopsy was one in 19 cases, two in 4, and three or more in 5. Complete diagnostic agreement in tumor typing and grading after stereotactic biopsy and surgical resection was attained in 10 cases (36%). Agreement in tumor typing was marked in 16 cases (57%). Erroneous typing was more frequent in tumors with an MIB-1 index of less than 3% (P = 0.0629) and mixed gliomas (P = 0.0801). Overgrading of WHO grade I tumors was marked in 3 cases (11%) and undergrading of WHO grade III gliomas in 8 cases (28%). Tumor undergrading was more frequent in cases with an MIB-1 index of more than 3% (P = 0.0045). The MIB-1 index detected after stereotactic biopsy was nearly always lower compared with those established after surgical resection (P < 0.0001). In conclusion, the histopathological diagnosis of low-grade glioma established after stereotactic biopsy is associated with a substantial risk of inaccuracy. Tumors with low proliferative activity and mixed gliomas are especially susceptible for erroneous tumor typing. Undergrading of high-grade gliomas may be suspected if the MIB-1 index in the tumor specimen constitutes more, than 3%.
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PMID:Low-grade glioma on stereotactic biopsy: how often is the diagnosis accurate? 1885 92

We compared the tumor uptake of (11)C-methionine (MET) with positron emission tomography (PET) with the results of a pathological analysis to examine the proliferative potential and microvessel density measured with immunostaining for MIB-1 and factor VIII, respectively, from 33 patients with glioma. The MET uptake in oligodendrogliomas was significantly greater than that in grade 2 astrocytomas and comparable to those in grade 3 and 4 astrocytomas. The MIB-1 index of oligodendroglioma meanwhile was comparable to that of grade 2 astrocytoma. The microvessel area in oligodendroglioma was significantly greater than that in grade 2 astrocytomas and comparable to those in grade 3 and 4 astrocytomas. According to a multivariate statistical analysis, MET uptake ratio was closely correlated with the MIB-1 index among astrocytomas. An increase in the microvessel area in the oligodendrogliomas contributed to the higher MET uptake among the tumors with a low-proliferative index. This information is important for interpreting the results of MET-PET studies for clinical use.
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PMID:Contributions of biological tumor parameters to the incorporation rate of L: -[methyl-(11)C] methionine into astrocytomas and oligodendrogliomas. 1909 97

Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic neoplasm of the brain. Some PXAs are accompanied by anaplastic features and are difficult to manage because of frequent recurrences that lead to early death. No previous reports have demonstrated consistent efficacy of adjuvant radiotherapy or chemotherapy for this disease. We report a case of PXA with anaplastic features treated with stereotactic irradiation (STI) that resulted in long-term control of repeatedly recurring nodules throughout the neuraxis. A 47-year-old woman presented with an epileptic seizure due to a large tumor in the right frontal lobe. The tumor was resected and diagnosed as PXA with anaplastic features. Sixteen months later, a relapse at the primary site was noted and treated with stereotactic radiosurgery using Gamma Knife. Two years later, the patient developed a tumor nodule in the cervical spinal cord that histologically corresponded to a small-cell glioma with high cellularity and prominent MIB-1 (mindbomb homolog 1) labeling. In the following months, multiple nodular lesions appeared throughout the CNS, and STI was performed six times for eight intracranial lesions using Gamma Knife and twice using a linear accelerator, for three spinal cord lesions in total. All lesions treated with STI were well controlled, and the patient was free from symptomatic progression for 50 months. However, diffuse dissemination along the craniospinal axis eventually progressed, and she died 66 months after initial diagnosis. Autopsy showed that the nodules remained well demarcated from the surrounding nervous system tissue. STI may be an effective therapeutic tool for controlling nodular dissemination of PXA with anaplastic features.
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PMID:Long-term control of disseminated pleomorphic xanthoastrocytoma with anaplastic features by means of stereotactic irradiation. 1916 34

Pilocytic astrocytoma (PA) is the most common glioma in the pediatric population. PAs can exhibit variable behavior that does not always correlate with location, yet at present there is no way to predict which tumors will be more aggressive. To address this problem, an institutional cohort of 147 PAs (118 with outcome data) from both cerebellar and noncerebellar locations (spine, diencephalon, midbrain, brainstem and cortex) was utilized. Parameters included quantification of characteristic morphologic variables as well as genes previously shown to be of relevance in high-grade gliomas, including MIB-1, p53 and MGMT. In this cohort, the classic biphasic appearance was most common in cerebellar tumors, whereas noncerebellar tumors were predominantly microcystic. Associations with outcome suggest that the presence of degenerative atypia may be a favorable factor in PAs. Oligodendroglial morphology and the absence of leptomeningeal invasion are adverse histologic factors, but only in cerebellar tumors. Conversely, MIB-1 proliferation index and p53 and MGMT expression do not correlate with outcome. Morphologic biomarkers thus do exist for PAs, but the utility of each biomarker varies according to location. These results suggest that PAs differ fundamentally according to location; therefore, biological behavior may not simply depend on extent of resection.
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PMID:Impact of morphology, MIB-1, p53 and MGMT on outcome in pilocytic astrocytomas. 1983 38

Our aim was to extract the radiopathological features of cerebellar malignant glioma in adults from the four cases we encountered. All four cases (two men and two women, aged 52-80 years; mean age, 67 years) had a floating sensation or vertigo at the onset of their disease. Initially, these patients were given a diagnosis of cerebellar infarction or cavernous angioma, or had faint abnormalities in the cerebellum that were overlooked. These patients were followed up for 2-14 months (mean, 6 months), and the tumor was detected when their clinical symptoms deteriorated. The tumor was located in the hemisphere in one patient and in the vermis in three patients. MRI revealed ring-like enhancement in two patients and nodular enhancement in two patients. All patients underwent subtotal tumor resection, followed by postoperative radiochemotherapy. However, three patients died at 16 to 44 months (mean, 28 months), and cerebrospinal fluid (CSF) dissemination was observed in three patients. Two cases were classified as WHO grade III and two as WHO grade IV. The pathological features were typical of malignant glioma, partially presenting the features of low-grade glioma, such as pilocytic, astrocytic, or oligodendroglial components. Nuclear pleomorphism and vascular endothelial proliferation were prominent, and micronecrosis was relatively less evident. The MIB-1 index was 12%-40%, and most of the patients were p53 protein positive. At the onset of cerebellar malignant glioma, diagnostic imaging shows few signs of brain tumor. Thereafter, tumors grow in a short period of time, following a rapid clinical course. Because cerebellar malignant glioma is a rare disease, it is difficult to differentiate it from metastatic brain tumors and primary central nervous system lymphoma by preoperative imaging. Some patho logical features suggesting malignant transformation from low-grade glioma were detected.
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PMID:Radiopathological characteristics of cerebellar malignant glioma in adults. 1985 16

Although there are many reports on the clinical use of the MIB-1 labeling index (LI), which is a measure of proliferative activity in astrocytomas; its significance varies between studies. There are no known molecules that are directly linked to the MIB-1 LI in astrocytomas. We evaluated the clinical value of the MIB-1 LI in our human glioblastoma cases and determined the molecules that possibly influenced the MIB-1 LI. An immunohistochemical study of the MIB-1 protein was performed and MIB-1 LIs of 38 glioblastomas were determined. In the same cases, epidermal growth factor receptor (EGFR), platelet-derived growth factor receptor-alpha (PDGFRA), and sphingosine-1-phosphate receptor type 1 (S1P(1)), which are known regulators of glioma cell proliferation, were detected and quantified by quantitative real-time-PCR or western blotting. Kaplan-Meier survival curves for 38 patients with glioblastomas showed that a high MIB-1 LI correlated with poor survival (P < 0.05). Among the molecules tested, only the low expression of S1P(1) was significantly correlated with the high MIB-1 LI in glioblastomas (P < 0.05). Multivariate analysis revealed that the S1P(1) expression level was a significant prognostic factor. Our results indicate that the MIB-1 LI is an important prognostic factor in human glioblastomas. Furthermore, downregulation of S1P(1) expression increases proliferative activity, and thus enhances the malignancy of glioblastomas, resulting in a poor survival.
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PMID:The expression level of sphingosine-1-phosphate receptor type 1 is related to MIB-1 labeling index and predicts survival of glioblastoma patients. 1993 66

We report a rare case of epileptogenic glioma composed of glial progenitor cells that differentiated into an astrocytic and oligodendrocytic tumor. This 4-year-old girl presented with a 1-year history of complex partial seizure. MR scan showed a mass in the left temporal lobe with a cyst and a contrast-enhanced component. Subtotal resection of the tumor was achieved. Histological examination revealed that the tumor exhibited low cellularity composed of astrocytic and oligodendrocytic components, as well as low mitotic activity (MIB-1 = 1%). Immunohistochemical examination revealed GFAP positivity within the astrocytic cells, olig2 positivity within the oligodendrocytic cells, and S100 positivity in both cell types. MAP2 and CD34 were negative, and neurofilament was only positive in preexisting neurons. The pathological diagnosis was epileptogenic glioma (grade I) composed of glial progenitor cells. The postoperative course has been uneventful with good seizure control for 3 years.
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PMID:Epileptogenic glioma in a 4-year-old child: a case report. 2104 16


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