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

The clinical, surgical, and pathological data from 35 published cases of oligodendroglioma and of one personal case are analysed and compared with those from other tumours of the cord and from cerebral oligodendrogliomas. Oligodendroglioma of the cord has a slightly lower average age than other gliomas and is closer to that of glioblastoma. In oligodendroglioma of the cord, as of the brain, acute onset or aggravation of the symptoms and an oscillating course are frequent. Two correlated data are particularly worth noting: a) the mean CSF protein content in oligodendroglioma of the cord is higher than in any other glioma; b) intracranial hypertension, in the form of papilloedema or hydrocephalus, or both, was present in 31% of cases. This signifies cerebral oligodendrogliomatosis, which was found in 6 out of 10 necropsied cases. At operation most oligodendrogliomas of the cord appear as infiltrating "gelatinous" tumours, though a minority have a firm consistency and apparently clearcut contours, which seem to be associated with a better prognosis. Postoperative radiotherapy seems to be useful.
 ...
PMID:Oligodendrogliomas of the spinal cord. 699 45

Oligodendroglioma represents a distinct type of diffuse glioma with a relatively favorable prognosis. Although an O2A-like glial progenitor cell of origin has been suggested, a neuronal-oligodendroglial progenitor cell is also of interest, particularly because variable degrees of neuronal marker expression have been reported in typical oligodendrogliomas. We present 2 female and 2 male patients (ages 34-54) with frontal lobe oligodendrogliomas containing a) morphologically distinct collections of small round cells with hyperchromatic nuclei, b) well-formed Homer Wright-like and perivascular rosettes, and c) demonstrable neuronal differentiation by immunohistochemistry and/or electron microscopy in the rosette-associated regions. Unlike extraventricular neurocytomas, these cases featured an infiltrative growth pattern and a classic oligodendroglioma immunophenotype in non-rosette bearing portions of each tumor. FISH analysis demonstrated chromosome 1p and 19q codeletions in 3 (75%) cases, both in regions with and without rosettes. Recurrences were common, although all patients are currently alive 4 months to 13 yr from initial diagnosis. Based on clinicopathologic and genetic features, we diagnosed these tumors as oligodendrogliomas with neurocytic differentiation. However, it is unclear whether they represent a) gliomas with divergent neuronal differentiation, b) a distinctive form of glioneuronal neoplasm, or c) a reflection of glioneuronal histogenesis in oligodendrogliomas in general. In any case, their occurrence suggests a histogenetic overlap between oligodendroglioma and extraventricular neurocytoma not previously recognized.
 ...
PMID:Oligodendrogliomas with neurocytic differentiation. A report of 4 cases with diagnostic and histogenetic implications. 1243 Jul 11

Treatment of malignant glioma requires a multidisciplinary team. Treatment includes surgery, radiotherapy, and chemotherapy. Recently developed agents have demonstrated activity against recurrent malignant glioma and efficacy if given concurrently with radiotherapy in the upfront setting. Oligodendroglioma with 1p/19q deletions has been recognized as a distinct pathologic entity with particular sensitivity to radiotherapy and chemotherapy. Randomized trials have shown that early neoadjuvant or adjuvant administration of procarbazine, lomustine, and vincristine chemotherapy prolongs disease-free survival; however, it has no impact on overall survival. Temozolomide, a novel alkylating agent, has shown modest activity against recurrent glioma. In combination with radiotherapy in newly diagnosed patients with glioblastoma, temozolomide significantly prolongs survival. Molecular studies have demonstrated that the benefit is mainly observed in patients whose tumors have a methylated methylguanine methyltransferase gene promoter and are thus unable to repair some of the chemotherapy-induced DNA damage. For lower-grade glioma, the use of chemotherapy remains limited to recurrent disease, and first-line administration is the subject of ongoing clinical trials. Irinotecan and agents like gefitinib, erlotinib, and imatinib targeting the epidermal growth factor receptor and platelet-derived growth factor receptor have shown some promise in recurrent malignant glioma. This review summarizes recent developments, focusing on the clinical management of patients in daily neuro-oncology practice.
 ...
PMID:Changing paradigms--an update on the multidisciplinary management of malignant glioma. 1647 37

Human gliomas including astrocytomas and oligodendrogliomas are defined as being composed of neoplastic astrocytes and oligodendrocytes respectively. Here, on the basis of in vitro functional assays, we show that gliomas contain a mixture of glial progenitor cells and their progeny. We have set up explant cultures from pilocytic astrocytomas, glioblastomas and oligodendrogliomas and studied antigens that characterize glial lineage, from the precursor cells (glial restricted precursors and oligodendrocyte-type2-astrocyte/oligodendrocyte precursor cells expressing the A2B5 ganglioside) to the differentiated cells (oligodendrocyte and type-1 and type-2 astrocytes). All tumoral explants contain A2B5+ cells and can generate migrating cells with distinctive functional properties according to glioma subtypes. In pilocytic astrocytomas, very few migrating cells are dividing and can differentiate in type-2 astrocytes or towards the oligodendrocyte lineage. In glioblastomas, most migrating cells are dividing, express A2B5 or glial fibrillary acid protein (GFAP) and can generate oligodendrocytes and type-1 and type-2 astrocytes in appropriate medium. Oligodendroglioma explants are made by actively dividing glial precursor cells expressing A2B5 or PSA-NCAM. Only few cells can migrate and differentiation towards oligodendrocyte lineage does not occur. Isolated A2B5+ cells from both glioblastomas and oligodendrogliomas showed similar genetic alterations as the whole tumour. Therefore, pilocytic astrocytomas contain slowly dividing oligodendrocyte-type2-astrocyte/oligodendrocyte precursor cells in keeping with their benign behaviour whereas both glioblastomas and oligodendrogliomas contain neoplastic glial restricted precursor cells. In oligodendrogliomas, these cells are trapped in undifferentiated and proliferating state. The precursor cells properties present in gliomas give new insight into their histogenesis and open up new avenues for research in the field of gliomagenesis.
 ...
PMID:In vitro identification and functional characterization of glial precursor cells in human gliomas. 1659 47

Survivors of central nervous system (CNS) tumours are particularly vulnerable to tumour- and treatment-related disability. We present the incidence of specific and overall functional and health-related late effects in a national adult survivor cohort. Diagnostic subgroups at particular risk for persistent sequels are identified. Data collection targeted 708 eligible >18 years old survivors, 708 parent proxies and 1000 general population controls. Functional disability including sensory and cognitive impairment, emotional status and pain was assessed using the Health Utilities Index Mark 2/3 (HUI2/3). Survivors and controls, and diagnostic subgroups were contrasted to identify the general and relative risk for late effects by sub-diagnosis. Survivors had persistent late effects in sensation, mobility, self-care and cognition. Deficits in these domains indicated clinically important disability in overall health, although indices of emotion and pain were unaffected compared to controls. Late effects tended to aggravate with time, and female survivors had poorer health. Oligodendroglioma, mixed/unspecified glioma, intracranial germ cell tumour and medulloblastoma survivors had poorest overall health. Least late effects were found for other specified/unspecified CNS tumours (including meningeoma and nerve sheath tumours), and for astrocytoma. An impact on educational, vocational and family-related outcomes, and higher utilisation of social insurance or government subsidies validated health-related sequelae in adulthood. Comparisons with controls confirm persistent disability in multiple functional domains in adult CNS tumour survivors. The heightened proportion of survivors presenting severe disability is a factor that specifically differentiates survivors from controls, although diagnostic subgroups differ significantly regarding the amount and severity of late effects.
 ...
PMID:Health and persistent functional late effects in adult survivors of childhood CNS tumours: a population-based cohort study. 1961 28

Currently, the use of microscopical examinations alone is insufficient for the histological classification and grading of gliomas. In recognition of the emerging role of molecular diagnostic approaches to glioma classification, the role of genetic profiles has been emphasized on, as in the distinct subtypes of glioma. Glioblastomas (WHO grade IV) may develop de novo (primary glioblastomas) or through progression from lower-grade astrocytomas (secondary glioblastomas) both glioblastomas show similar histological features. However, these subtypes of glioblastoma involve distinct disease entities that evolve through different genetic pathways and are likely to differ in prognosis and response to therapy. Oligodendroglioma is recognized as a particular subtype of gliomas that shows remarkable response to chemotherapy [procarbazine+CCNU+vincristine (PCV)], making their correct diagnosis important. However, the histological differentiation of oligodendrogliomas from diffuse astrocytoma could be highly subjective in cases without typical morphological features. Loss of heterozygosity (LOH) on chromosomes 1p and 19q is correlated with sensitivity to PCV chemotherapy with increased survival in anaplastic oligodendroglioma cases (WHO grade III). Similarly, combined LOH on 1p and 19q has been identified as a predictor of favorable overall survival in oligodendrogliomas. Additionally, the status of the O6-methylguanine-methyltransferase gene (O6-MGMT) is associated with resistance to temozolomide (TMZ). We have previously found methylation or expression mosaicism of O6-MGMT in gliomas, resulting in problems on tumor sampling and response to TMZ. An assessment of the O6-MGMT methylation mosaicism in heterogeneous glioma may provide a more accurate assessment for the response to TMZ. This article suggests that more biological and molecular approaches to brain tumor classification will provide improved means to treat these tumors.
 ...
PMID:[Clinicopathological diagnosis of gliomas by genotype analysis]. 1961 54

Oligodendrogliomas are the third most common type of glioma. Oligodendrogliomas are typically indolent tumours, yet the majority of them are incurable and most patients will ultimately die. Anaplastic oligodendrogliomas are important to recognise from other primary brain tumours, since they have unique molecular, histological and clinical features. Oligodendroglioma, especially the anaplastic type, has a tendency to intracranial and extracranial metastasis. In this paper, we present an unusual manifestation of anaplastic oligodendroglioma.
 ...
PMID:Unusual intracranial spread of anaplastic oligodendroglioma. 2289 Oct 21

Oligodendroglioma poses a biological conundrum for malignant adult human gliomas: it is a tumor type that is universally incurable for patients, and yet, only a few of the human tumors have been established as cell populations in vitro or as intracranial xenografts in vivo. Their survival, thus, may emerge only within a specific environmental context. To determine the fate of human oligodendroglioma in an experimental model, we studied the development of an anaplastic tumor after intracranial implantation into enhanced green fluorescent protein (eGFP) positive NOD/SCID mice. Remarkably after nearly nine months, the tumor not only engrafted, but it also retained classic histological and genetic features of human oligodendroglioma, in particular cells with a clear cytoplasm, showing an infiltrative growth pattern, and harboring mutations of IDH1 (R132H) and of the tumor suppressor genes, FUBP1 and CIC. The xenografts were highly invasive, exhibiting a distinct migration and growth pattern around neurons, especially in the hippocampus, and following white matter tracts of the corpus callosum with tumor cells accumulating around established vasculature. Although tumors exhibited a high growth fraction in vivo, neither cells from the original patient tumor nor the xenograft exhibited significant growth in vitro over a six-month period. This glioma xenograft is the first to display a pure oligodendroglioma histology and expression of R132H. The unexpected property, that the cells fail to grow in vitro even after passage through the mouse, allows us to uniquely investigate the relationship of this oligodendroglioma with the in vivo microenvironment.
 ...
PMID:A novel, diffusely infiltrative xenograft model of human anaplastic oligodendroglioma with mutations in FUBP1, CIC, and IDH1. 2352 65

Oligodendroglioma (WHO Grade 2) and anaplastic oligodendroglioma (WHO Grade 3) are glial tumors composed of neoplastic cellular elements that resemble oligodendrocytes. The treatment of recurrent, alkylator refractory oligodendroglial tumors is challenging given the paucity of effective treatment and lack of randomized controlled trials on which to base therapy. Notwithstanding the lack of prospective, randomized data, treatment of oligodendroglial tumors with bevacizumab can be recommended tentatively recognizing that preliminary studies suggest efficacy. Somatic mutations of the isocitrate dehydrogenase enzymes (IDH1 and IDH2) appear to play a critical role in the pathogenesis of most oligodendroglial tumors and agents that target these mutations are a potential therapeutic option. Additionally, reversal of CpG island hypermethylated phenotype status through inhibition of DNA methyltransferase with an inhibitor such as decitabine may provide a target for future studies.
...
PMID:Bevacizumab and other novel therapies for recurrent oligodendroglial tumors. 2650 17

It has become increasingly evident that morphologically similar gliomas may have distinct clinical phenotypes arising from diverse genetic signatures. To date, glial tumours from the Tunisian population have not been investigated. To address this, we correlated the clinico-pathology with molecular data of 110 gliomas by a combination of HM450K array, MLPA and TMA-IHC. PTEN loss and EGFR amplification were distributed in different glioma histological groups. However, 1p19q co-deletion and KIAA1549:BRAF fusion were, respectively, restricted to Oligodendroglioma and Pilocytic Astrocytoma. CDKN2A loss and EGFR overexpression were more common within high-grade gliomas. Furthermore, survival statistical correlations led us to identify Glioblastoma (GB) prognosis subtypes. In fact, significant lower overall survival (OS) was detected within GB that overexpressed EGFR and Cox2. In addition, IDH1R132H mutation seemed to provide a markedly survival advantage. Interestingly, the association of IDHR132H mutation and EGFR normal status, as well as the association of differentiation markers, defined GB subtypes with good prognosis. By contrast, poor survival GB subtypes were defined by the combination of PTEN loss with PDGFRa expression and/or EGFR amplification. Additionally, GB presenting p53-negative staining associated with CDKN2A loss or p21 positivity represented a subtype with short survival. Thus, distinct molecular subtypes with individualised prognosis were identified. Interestingly, we found a unique histone mutation in a poor survival young adult GB case. This tumour exceptionally associated the H3F3A G34R mutation and MYCN amplification as well as 1p36 loss and 10q loss. Furthermore, by exhibiting a remarkable methylation profile, it emphasised the oncogenic power of G34R mutation connecting gliomagenesis and chromatin regulation.
 ...
PMID:Molecular Diagnostic and Prognostic Subtyping of Gliomas in Tunisian Population. 2695 5


1 2 Next >>