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)

Progressive or recurrent high-grade gliomas are characterized by a very poor prognosis, and the relevance of second-line chemotherapy is still unassessed. Although it has been reported that liposomal anthracyclines and carboplatin show some activity in these patients, their association has never been investigated. We have treated six children with recurrent high-grade glioma after surgery, radiotherapy and chemotherapy, and one child with progressive teratoid/rhabdoid tumor with the combination of liposomal daunorubicin and carboplatin plus etoposide. Five out of seven children showed a major response and the 29 month progression-free survival was 38%. The above regimen was feasible and children showed only little and transient hematological toxicity. In our opinion, these results justify further investigation of the above combination chemotherapy for recurrent or progressive malignant brain tumors in children.
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PMID:Second-line chemotherapy with the association of liposomal daunorubicin, carboplatin and etoposide in children with recurrent malignant brain tumors. 1501 84

Results of surgical treatment of 253 patients with glial tumors and 47 patients with metastatic tumors of the brain are analyzed. An analysis with CT and MRT was used in the preoperative diagnosis for the calculation of the tumor volume and the dislocation degree. Microsurgical technologies used in the treatment allowed to shorten the period of postoperative rehabilitation, to reduce the development of neurological deficit that created conditions for early postoperative X-ray and chemotherapy.
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PMID:[Clinical aspects of glial and metastatic neoplasms of brain]. 1519 64

The identification of molecular genetic abnormalities in specific types of pediatric brain tumors is beginning to play a role in the stratification of patients into treatment groups. The finding of an INI1 alteration in an atypical teratoid/rhabdoid tumor or malignant neoplasm with overlapping histologic features will be required for entry onto disease-specific protocols within the Children's Oncology Group. Refinement in the classification of medulloblastoma and malignant glioma patients will likely depend on the genetic and signaling pathways that characterize these tumors. Advances in this area will depend on the ability to identify new disease genes, validate prognostic markers, and develop biologically based therapeutic strategies to tailor treatment.
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PMID:Molecular analysis of pediatric brain tumors. 1548 13

Brain tumors are a heterogeneous group of neoplasms with different origins, pathobiologies, treatments and prognoses. The collective contributions from the fields of neuro-oncology, neurosurgery, radiation oncology, neurology, neuropathology, neuroradiology and molecular biology have all led to significant advances in the treatment of certain brain tumors. Ideas from these fields, under the cooperative umbrella of clinical cancer trial consortia, have been tested in large-scale studies. As a result, patient survivals have increased markedly for these tumor types. Unfortunately, there are certain brain tumors in childhood, such as the diffuse intrinsic pontine glioma and atypical teratoid rhabdoid tumor, for which survival advantages have not been found. This review will discuss the current and possible future therapies of the most common pediatric brain tumors and highlight some of the novel imaging modalities that are used pre- and intraoperatively.
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PMID:Advances in the treatment of pediatric brain tumors. 1585 14

In recent years, numerous new entities or variants of recognized central nervous system tumors have been described in the literature, and the morphologic spectrum of these neoplasms is delineated incompletely. The accurate diagnosis and classification of these lesions is important to ensure that patients receive adequate therapy and prognostic information. The clinicopathologic features and differential diagnosis of 4 new entities, including the chordoid glioma of the third ventricle, cerebellar liponeurocytoma, atypical teratoid/rhabdoid tumor, and papillary glioneuronal tumor, are discussed in this review.
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PMID:Newly defined central nervous system neoplasms. 1610 Aug 66

Pilocytic astrocytoma (PA) is the most common glioma of childhood. Despite their relatively high incidence, the molecular mechanisms responsible for tumorigenesis and growth of PA are poorly understood. Previous in vitro studies in our laboratory showed that despite the absence of ErbB1, PA was sensitive to ErbB1 tyrosine kinase inhibitor gefitinib. To identify alternative targets of gefitinib in PA, we studied other members of the ErbB receptor tyrosine kinase family that have been identified in brain tumors. Using gene expression microarray and Western blot analyses, we found that ErbB3 is highly overexpressed in PA compared with other pediatric brain tumors (glioblastoma, ependymoma, medulloblastoma, atypical teratoid/rhabdoid tumor, and choroid plexus papilloma). Developmental biology studies have identified Sox10 as a regulator of ErbB3 expression during development of the neural crest. Investigation of Sox10 in PA revealed that it is highly overexpressed relative to other pediatric brain tumors, lending support to the theory that Sox10-regulated overexpression of ErbB3 may be driving growth in PA. Sox10-regulated ErbB3 overexpression is a novel insight into the biology of PA, suggests possible recapitulation of developmental pathways in tumorigenesis, and presents possible targets for therapeutic intervention that might be used for hypothalamic variants not amenable to surgical cure.
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PMID:Paired overexpression of ErbB3 and Sox10 in pilocytic astrocytoma. 1689 10

We report a rare case of atypical teratoid/rhabdoid tumor that presented with imaging findings similar to those of optic pathway glioma. The diagnosis of atypical teratoid/rhabdoid tumor was determined following surgical resection of the tumor by collective histologic and immunohistochemical staining, and cytogenetic analysis. We describe the clinical presentation, neuroimaging findings and pathology, and compare them to those of optic gliomas.
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PMID:Atypical teratoid/rhabdoid tumor of the optic nerve. 1869 60

This 32-year-old woman, 27 weeks pregnant, harbored a cystic mass with a solid component in the left frontal lobe. Histologically, the lesion was hypercellular and contained a diffuse sheet of eosinophilic cells of various sizes. The cells were almost round and had a few prominent, eccentrically placed, hyperchromatic nuclei of various sizes. Immunohistochemically, the tumor was reactive for vimentin, epithelial membrane antigen, cytokeratin AE1/AE3, smooth muscle actin, and BAF47/INI-1, and negative for glial fibrillary acidic protein, neurofilament protein, S100 protein, CK7, CK20, HMB-45, MIC2, and Bcl-2. The Ki 67 labeling index was 4.2%. Comparative genomic hybridization analysis revealed aberrations of the chromosomal copy number of +7 and -10. This tumor could not be categorized according to the present World Health Organization classification. Results of staining with glial fibrillary acidic protein were not consistent with a glioma, and staining with INI-1 was inconsistent with atypical teratoid/rhabdoid tumor. The tumor was therefore designated as a "cerebral tumor with extensive rhabdoid features."
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PMID:Cerebral tumor with extensive rhabdoid features and a favorable prognosis. 1923 29

Rhabdoid tumor cells are typically observed in atypical teratoid/rhabdoid tumor (AT/RT) but may also be seen in meningioma,glioma, melanoma, rhabdomyosarcoma and metastatic carcinoma.We present an astroblastoma with unusual rhabdoid features which is rarely described in the English literature. Apart from the rhabdoid tumor cells, all the histopathological features typical for astroblastoma are present in this case. These features include pseudopapillary arrangement, astroblastic pseudorosettes, perivascular hyalinization and calcifications, absence of fibrillary background and a pushing tumor border. The tumor cells display a multilineage immunohistochemical profile. In addition, diffuse and strong membranous and cytoplasmic dot-like pattern is appreciated with epithelial membrane antigen (EMA). The diagnosis of astroblastoma is also well supported by the age of presentation, anatomical location and radiological features of the tumor.We believe that on top of the above-mentioned unusual tumors with rhabdoid cells, astroblastoma should also be considered in the list of differential diagnosis.
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PMID:A 33-year-old Chinese woman with a left frontal tumor. 1929 Oct 1

The management of brain tumors in very young children remains a challenge for neuro-oncologists in large part because of the greater vulnerability of the developing brain to treatment-related toxicity. Nearly three decades of infant brain tumor clinical trials have led to significant progress in the delineation of prognostic factors and improvements in outcome. Innovative strategies that employ high-dose chemotherapy, intrathecal chemotherapy, modified focal irradiation, or combinations of these have been used to delay or avoid the use of conventional craniospinal irradiation in order to minimize the risk for deleterious neurocognitive impairment in survivors. However, it is difficult to evaluate the impact of such approaches on intellectual and functional outcome, and results to date are limited. This review covers the most recent therapeutic advances for the most common histological subtypes of malignant infant brain tumors: medulloblastoma, supratentorial primitive neuroectodermal tumor, ependymoma, atypical teratoid rhabdoid tumor, choroid plexus carcinoma, and high-grade glioma. Survival and neurocognitive outcome are emphasized.
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PMID:Current treatment approaches for infants with malignant central nervous system tumors. 1934 75


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