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1. Noll EN, Lin J, Nakatsuji Y, Miller RH, Black PM: GM3 as a novel growth regulator for human gliomas. Exp Neurol; 2001 Apr;168(2):300-9
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  • [Title] GM3 as a novel growth regulator for human gliomas.
  • To determine whether GM3 influenced the expansion of human neural tumors the effects of GM3 treatment on primary human brain tumors were assayed.
  • Here we demonstrate that GM3 treatment dramatically reduces cell numbers in primary cultures of high-grade human glioblastoma multiforme (GBM) tumors and the rat 9L cell gliosarcoma cell line.
  • By contrast, GM3 treatment had little effect on cell number in cultures of normal human brain.
  • A single injection of GM3 3 days after intracranial implantation of 9L tumor cells in a murine xenograft model system resulted in a significant increase in the symptom-free survival period of host animals.
  • Cultures of high-grade ependymomas, mixed gliomas, astrocytomas, oligodendrogliomas, and gangliogliomas were all susceptible to GM3 treatment.
  • These results suggest that GM3 may have considerable value as a selectively toxic chemotherapeutic agent for human high-grade gliomas.
  • [MeSH-major] Brain Neoplasms / drug therapy. G(M3) Ganglioside / therapeutic use. Glioma / drug therapy
  • [MeSH-minor] Animals. Cell Death / drug effects. Cell Death / physiology. Cell Division / drug effects. Cell Division / physiology. Cell Survival / drug effects. Cell Survival / physiology. Child, Preschool. Drug Screening Assays, Antitumor. Glioblastoma / drug therapy. Glioblastoma / pathology. Humans. Male. Mice. Rats. Transplantation, Heterologous. Tumor Cells, Cultured / drug effects. Tumor Cells, Cultured / physiology

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  • (PMID = 11259118.001).
  • [ISSN] 0014-4886
  • [Journal-full-title] Experimental neurology
  • [ISO-abbreviation] Exp. Neurol.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / NS 36674
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / G(M3) Ganglioside
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2. Zhang JX, Kang CS, Shi L, Zhao P, Liu N, You YP: Use of thymidine kinase gene-modified endothelial progenitor cells as a vector targeting angiogenesis in glioma gene therapy. Oncology; 2010;78(2):94-102
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  • [Title] Use of thymidine kinase gene-modified endothelial progenitor cells as a vector targeting angiogenesis in glioma gene therapy.
  • OBJECTIVE: The poor prognosis of patients with glioma is due to infiltrative growth of glioma cells, which correlates with their ability to induce angiogenesis.
  • Tumor angiogenesis is supported by the mobilization and functional incorporation of endothelial progenitor cells (EPCs).
  • The aim of this study was to propose the use of gene-modified EPCs as a vector system that allowed systemic gene delivery into multiple areas of tumor angiogenesis for glioma therapy.
  • METHODS: Thymidine kinase (TK) gene-modified EPCs were mixed with glioma cells or human umbilical vein endothelial cells (HUVECs) at varying ratios for ganciclovir in vitro.
  • EPCs were injected via tail vein into nude mice bearing glioma, and EPC incorporation into the tumor was determined immunohistochemically.
  • RESULTS: TK gene-modified EPCs exerted a potent bystander effect on glioma cells and HUVECs by induction of apoptosis via caspase activation in vitro.
  • EPCs incorporated preferentially into glioma vasculatures.
  • CONCLUSION: The results indicate the feasibility of EPC-based gene delivery into disseminated areas of tumor angiogenesis as a rational strategy for glioma gene therapy.
  • [MeSH-major] Apoptosis / physiology. Genetic Therapy / methods. Glioma / therapy. Neovascularization, Pathologic / prevention & control. Stem Cell Transplantation. Stem Cells / enzymology. Thymidine Kinase / genetics
  • [MeSH-minor] Animals. Antigens, CD / analysis. Cell Division / drug effects. Endothelium, Vascular / cytology. Endothelium, Vascular / physiology. Fetal Blood / cytology. Ganciclovir / pharmacology. Glycoproteins / analysis. Humans. Leukocytes, Mononuclear / cytology. Mice. Mice, Inbred BALB C. Mice, Nude. Peptides / analysis. Transplantation, Heterologous. Umbilical Veins

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  • [Copyright] Copyright 2010 S. Karger AG, Basel.
  • (PMID = 20357517.001).
  • [ISSN] 1423-0232
  • [Journal-full-title] Oncology
  • [ISO-abbreviation] Oncology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / AC133 antigen; 0 / Antigens, CD; 0 / Glycoproteins; 0 / Peptides; EC 2.7.1.21 / Thymidine Kinase; P9G3CKZ4P5 / Ganciclovir
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3. Kish PE, Blaivas M, Strawderman M, Muraszko KM, Ross DA, Ross BD, McMahon G: Magnetic resonance imaging of ethyl-nitrosourea-induced rat gliomas: a model for experimental therapeutics of low-grade gliomas. J Neurooncol; 2001 Jul;53(3):243-57
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  • [Title] Magnetic resonance imaging of ethyl-nitrosourea-induced rat gliomas: a model for experimental therapeutics of low-grade gliomas.
  • Human low-grade gliomas represent a population of brain tumors that remain a therapeutic challenge.
  • Preclinical evaluation of agents, to test their preventive or therapeutic efficacy in these tumors, requires the use of animal models.
  • Spontaneous gliomas develop in models of chemically induced carcinogenesis, such as in the transplacental N-ethyl-N-nitrosourea (ENU) rat model.
  • However, without the ability to detect initial tumor formation, multiplicity or to measure growth rates, it is difficult to test compounds for their interventional or preventional capabilities.
  • In this study Fisher-334 rats, treated transplacentally with ENU, underwent magnetic resonance imaging (MRI) examination in order to evaluate this approach for detection of tumor formation and growth.
  • ENU-induced intracranial cerebral tumors were first observable in T2-weighted images beginning at 4 months of age and grew with a mean doubling time of 0.487 +/- 0.112 months.
  • These tumors were found histologically to be predominately mixed gliomas.
  • Two therapeutic interventions were evaluated using MRI, vitamin A (all-trans retinol palmitate, RP), as a chemopreventative agent and the anti-angiogenic drug SU-5416.
  • RP was found to significantly delay the time to first tumor observation by one month (P = 0.05).
  • No differences in rates of tumor formation or growth rates were observed between control and RP-treated groups.
  • MRI studies of rats treated with SU-5416 resulted in reduction in tumor growth rates compared to matched controls.
  • These results show that MRI can be used to provide novel information relating to the therapeutic efficacy of agents against the ENU-induced tumor model.
  • [MeSH-major] Brain Neoplasms / chemically induced. Brain Neoplasms / diagnosis. Carcinogens. Ethylnitrosourea. Glioma / chemically induced. Glioma / diagnosis. Magnetic Resonance Imaging. Vitamin A / analogs & derivatives
  • [MeSH-minor] Angiogenesis Inhibitors / therapeutic use. Animals. Diet. Disease Models, Animal. Indoles / therapeutic use. Neoplasms, Multiple Primary. Neoplasms, Second Primary / chemically induced. Neoplasms, Second Primary / diagnosis. Neoplasms, Second Primary / drug therapy. Pyrroles / therapeutic use. Rats. Rats, Inbred F344. Receptor Protein-Tyrosine Kinases / antagonists & inhibitors. Receptors, Growth Factor / antagonists & inhibitors. Receptors, Vascular Endothelial Growth Factor. Survival Analysis. Time Factors

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  • (PMID = 11718257.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Carcinogens; 0 / Indoles; 0 / Pyrroles; 0 / Receptors, Growth Factor; 11103-57-4 / Vitamin A; 1D1K0N0VVC / retinol palmitate; 71IA9S35AJ / Semaxinib; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptors, Vascular Endothelial Growth Factor; P8M1T4190R / Ethylnitrosourea
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4. Hyder DJ, Sung L, Pollack IF, Gilles FH, Yates AJ, Davis RL, Boyett JM, Finlay JL: Anaplastic mixed gliomas and anaplastic oligodendroglioma in children: results from the CCG 945 experience. J Neurooncol; 2007 May;83(1):1-8
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  • [Title] Anaplastic mixed gliomas and anaplastic oligodendroglioma in children: results from the CCG 945 experience.
  • PURPOSE: To review interpathologist diagnosis variability and survival of children treated for either anaplastic mixed glioma (AMG) or anaplastic oligodendroglioma (AO) with surgery, irradiation and chemotherapy.
  • PATIENTS AND METHODS: Two hundred and fifty patients with an institutional diagnosis of malignant glioma were enrolled on Children's Cancer Group CCG-945 between 1985 and 1991, and administered vincristine during involved field radiotherapy, then six cycles of prednisone, lomustine and, vincristine; or two cycles of "eight-drugs-in-one-day" (8-in-1) chemotherapy then involved-field radiotherapy followed by six cycles of 8-in-1 chemotherapy.
  • Complete resection and cerebral tumor location was associated with better overall survival (OS) in patients with institutional diagnoses of AMG.
  • However, central review established that only nine of 26 children had AMG: either mixed oligoastrocytoma (MOA) or anaplastic mixed oligoastrocytoma (AOA) and only one had AO.
  • CONCLUSION: Diagnosis of these tumors is challenging, with only 35% of institutional diagnoses confirmed for AMG and 25% for AO, and survival among children with these tumors is poor, despite intensive therapy.
  • This suggests reliable diagnostic markers and new therapeutic approaches are needed.
  • [MeSH-major] Central Nervous System Neoplasms / diagnosis. Central Nervous System Neoplasms / therapy. Glioma / diagnosis. Glioma / therapy. Oligodendroglioma / diagnosis. Oligodendroglioma / therapy
  • [MeSH-minor] Adolescent. Astrocytoma / diagnosis. Astrocytoma / therapy. Brain Neoplasms / diagnosis. Brain Neoplasms / therapy. Child. Child, Preschool. Cohort Studies. Drug Therapy. Female. Humans. Infant. Male. Neurosurgical Procedures. Radiotherapy. Spinal Cord Neoplasms / diagnosis. Spinal Cord Neoplasms / therapy. Survival Analysis

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  • (PMID = 17252186.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Randomized Controlled Trial
  • [Publication-country] United States
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5. Buhl R, Stark AM, Hugo HH, Rohr A, Mehdorn HM: Gliosarcoma: clinical experiences and additional information with MR spectroscopy. Neurol Res; 2009 Oct;31(8):873-7
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  • They have mixed features of glial and sarcomatous components.
  • Six tumors were in the frontal lobe, four in the temporal lobe, three parietal, two in the thalamic area and one in the occipital lobe.
  • Median survival time in our patient group was 7 months (2-11 months).
  • On histological examination, we found glial fibrillary acid protein positive cells surrounded by sarcomatous tissue and reticular fibers.
  • MR spectroscopy and location of the tumor adjacent to the dura with inhomogeneous contrast enhancement might give hints pre-operatively for the differential diagnosis of gliosarcoma.
  • Further works with adjuvant chemotherapy are necessary.
  • [MeSH-minor] Aged. Cell Proliferation. Diagnosis, Differential. Female. Glial Fibrillary Acidic Protein / metabolism. Humans. Magnetic Resonance Spectroscopy. Male. Middle Aged. Time Factors. Treatment Outcome

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  • (PMID = 19215667.001).
  • [ISSN] 0161-6412
  • [Journal-full-title] Neurological research
  • [ISO-abbreviation] Neurol. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Glial Fibrillary Acidic Protein
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6. Hukin J, Siffert J, Velasquez L, Zagzag D, Allen J: Leptomeningeal dissemination in children with progressive low-grade neuroepithelial tumors. Neuro Oncol; 2002 10;4(4):253-60
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  • [Title] Leptomeningeal dissemination in children with progressive low-grade neuroepithelial tumors.
  • Our purpose is to describe the incidence and clinical features of leptomeningeal dissemination (LM) in children with progressive low-grade neuroepithelial tumor (LGN).
  • We have continuously tracked all patients with primary CNS tumors since 1986.
  • Satisfactorily followed data were obtained on 427 of the 588 patients with localized LGN at diagnosis between 1986 and 1998, 177 (42%) of whom developed progressive or recurrent disease.
  • The median age at initial diagnosis was 5 years and at LM diagnosis was 8.5 years.
  • The primary tumor sites were diencephalon (6), brainstem (3), cerebellum (2), cerebrum (1), and spinal cord (1).
  • The histologies were pilocytic astrocytoma (4), ganglioglioma (4), fibrillary astrocytoma (3), mixed glioma (1), and glioneurofibroma (1).
  • Management included chemotherapy (2) or radiotherapy (3) or both (7); 1 patient received only radical resections of symptomatic lesions.
  • Tumors arising from the diencephalon appeared to predispose to LM; no other predisposing features were identified.
  • We strongly urge that for optimum treatment planning all patients with recurrent LGN be staged with an enhanced spine and brain MRI before adjuvant therapy is initiated.
  • The good survival of patients with LGN and LM reflects a more indolent disease than malignant CNS tumors with LM.
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Disease Progression. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Neoplasm Recurrence, Local / epidemiology. Neoplasm Recurrence, Local / therapy. Survival Analysis. Treatment Outcome

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  • (PMID = 12356355.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1920666
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7. Fisher BJ, Leighton CC, Vujovic O, Macdonald DR, Stitt L: Results of a policy of surveillance alone after surgical management of pediatric low grade gliomas. Int J Radiat Oncol Biol Phys; 2001 Nov 1;51(3):704-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Results of a policy of surveillance alone after surgical management of pediatric low grade gliomas.
  • PURPOSE: To document the incidence of tumor progression in pediatric patients with low-grade gliomas (LGGs), with particular emphasis on those patients who did not receive postoperative chemotherapy or radiotherapy (RT).
  • Ninety-one percent (n = 117) of the tumors were astrocytomas, of which 22 were pilocytic, 3 were oligodendrogliomas, 7 were mixed gliomas, and 1 was a ganglioglioma.
  • Of the 103 subtotally resected patients, 48 received postoperative RT (median dose 59 Gy in 25 fractions) and 10 patients were irradiated at the time of tumor progression.
  • The results of the univariate analysis of the overall survival by the Wilcoxon model were statistically significant for Karnofsky performance status (p = 0.03), RT timing (i.e., postoperative vs. deferred; p = 0.05), and tumor location (p = 0.02).
  • None of the patients who underwent gross complete resections received postoperative RT and none developed tumor recurrence.
  • Of the 103 patients who had subtotal resections, 33 had progression, with a median postprogression survival of 39 months.
  • The rate of tumor progression among the subtotally resected LGG patients who did not receive immediate postoperative RT was 42%.
  • The timing of RT and tumor location lost statistical significance for overall survival when the completely resected patients were excluded from the analysis.
  • At a median survival of 7.3 years, 42% of the subtotally resected LGG patients who did not receive immediate postoperative RT had tumor progression.
  • No statistically significant difference in survival was seen between the postoperative and deferred RT groups, even though the postoperative RT group was a group with poorer prognostic features (bulky residual tumor postoperatively, Karnofsky performance status <70, and nonhemispheric, noncerebellar tumors), indicating that RT may be beneficial for this particular subset of patients.


8. Xie J, Luo SQ, Ma ZY, Zhang YQ, Zeng HY, Qiu XG: [The clinical features and treatment of intracranial tumors in infants]. Zhonghua Yi Xue Za Zhi; 2004 Aug 2;84(15):1270-5
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  • [Title] [The clinical features and treatment of intracranial tumors in infants].
  • OBJECTIVE: To explore the treatment plan of intracranial tumors in infants.
  • METHODS: The clinical features and curative effect of 114 patients of intracranial tumors in infants who were admitted and performed microsurgery were analyzed retrospectively.
  • The total removal of tumor was 72.0%, subtotal removal 17.5%, partial removal 10.5%.
  • RESULTS: The tumor located at posterior fossa (37.7%), sella region (31.6%), hemisphere (19.3%) including in basal ganglia.
  • But 6 patients developed worse than the children of the same age.
  • CONCLUSION: Good prognosis can not be obtained for embryonal tumors and ependymoma through operation only.
  • Although radiotherapy is useful of inhibiting the growth of tumor, the dosage should be decreased for infants and the complications in long term also should be considered simultaneously.
  • It is a trend towards performing chemotherapy after operation.
  • It is not necessary to perform auxiliary therapy for astrocytoma, oligodendroglioma, and mixed glioma, because the prognosis is good after total removal of the tumor.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / surgery. Microsurgery
  • [MeSH-minor] Astrocytoma / diagnosis. Astrocytoma / surgery. Cerebellar Neoplasms / diagnosis. Cerebellar Neoplasms / surgery. Child, Preschool. Cranial Fossa, Posterior. Female. Follow-Up Studies. Humans. Infant. Male. Medulloblastoma / diagnosis. Medulloblastoma / surgery. Retrospective Studies. Sella Turcica

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  • (PMID = 15387964.001).
  • [ISSN] 0376-2491
  • [Journal-full-title] Zhonghua yi xue za zhi
  • [ISO-abbreviation] Zhonghua Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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9. van den Bent MJ: Advances in the biology and treatment of oligodendrogliomas. Curr Opin Neurol; 2004 Dec;17(6):675-80
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  • [Title] Advances in the biology and treatment of oligodendrogliomas.
  • PURPOSE OF REVIEW: The sensitivity of oligodendroglioma to chemotherapy and the prognostic significance of combined loss of 1p/19q in these tumors are now well established.
  • This review discusses recent molecular, genetic and clinical advances made in studies on oligodendroglioma and mixed oligoastrocytoma.
  • In contrast to earlier reports, the expression of the basic helix-loop-helix transcription factors is not specific for OD, but may occur in glial tumors of all lineages.
  • Gene expression profiling using gene arrays allows the separation of glial tumors according to histology, tumor grade and prognosis.
  • On MRI imaging, OD with combined 1p/19q loss has typical characteristics, including indistinct borders and a mixed signal intensity on T2-weighted images.
  • Despite the large increase in knowledge on the molecular abnormalities in OD, the therapeutic options for these tumors have not improved significantly since the introduction of temozolomide.
  • The increased survival after chemotherapy has been clearly established, but the timing of chemotherapy seems less critical.
  • It is clear that temozolomide is a good alternative to procarbazine, CCNU and vincristine (PCV) chemotherapy, in particular, because it is better tolerated.
  • No randomized trials, however, have compared PCV-chemotherapy to temozolomide.
  • New agents--and probably more targeted therapies--are needed to further improve treatment.
  • SUMMARY: The progress in the understanding of genetic and molecular abnormalities of OD has improved the recognition of treatment-sensitive OD, although this has not yet been mirrored in improved therapies or new treatment options.
  • While chemotherapy improves the outcome of OD, further improvements will likely require new drugs or new treatment concepts.
  • [MeSH-major] Brain Neoplasms / drug therapy. Brain Neoplasms / genetics. Oligodendroglioma / drug therapy. Oligodendroglioma / genetics
  • [MeSH-minor] Antineoplastic Agents / pharmacology. Antineoplastic Agents / therapeutic use. Chromosome Aberrations. Chromosomes, Human, Pair 1 / genetics. Chromosomes, Human, Pair 19 / genetics. Gene Expression Regulation, Neoplastic / genetics. Humans. Radiotherapy / methods. Radiotherapy / trends

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  • (PMID = 15542975.001).
  • [ISSN] 1350-7540
  • [Journal-full-title] Current opinion in neurology
  • [ISO-abbreviation] Curr. Opin. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 47
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10. Chansriwong P, Sirisinha T: Clinical features, management and outcomes of high-grade glioma patients in Ramathibodi Hospital. J Med Assoc Thai; 2010 Feb;93 Suppl 2:S68-73
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  • [Title] Clinical features, management and outcomes of high-grade glioma patients in Ramathibodi Hospital.
  • OBJECTIVE: To identify prognostic factors for survival and evaluate the effect of treatment on survival of patients with high-grade glioma treated at Ramathibodi Hospital.
  • MATERIAL AND METHOD: Medical records of patients with diagnosis of high-grade glioma registered in Ramathibodi cancer registry were reviewed.
  • Mean age of diagnosis was 41.86 years (range 18-71 years).
  • Histological findings were anaplastic glioma (22.20%), glioblastoma multiforme (63.90%) and mixed glioma (13.90%).
  • Of fifteen patients underwent total tumor removal, 17 patients had partial resection and in 4 cases biopsy alone was done.
  • Nine patients also received chemotherapy (6 temozolomide and 3 BCNU).
  • Median follow-up time was 413.2 days.
  • An overall survival time was 604.04 days and median disease free survival time was 402.45 days.
  • In univariated analysis, the following favorable prognostic factors were identified: histological findings of glioblastoma multiforme (GBM) and mixed glioma, received radiotherapy.
  • CONCLUSION: Adult high-grade glioma had poor prognosis despite aggressive treatment.
  • Radiotherapy significantly improved survival while surgical tumor removal and chemotherapy did not.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Brain Neoplasms / therapy. Carmustine / therapeutic use. Dacarbazine / analogs & derivatives. Glioma / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Hospitals. Humans. Incidence. Male. Middle Aged. Prognosis. Proportional Hazards Models. Retrospective Studies. Survival Analysis. Treatment Outcome. Young Adult

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  • (PMID = 21299082.001).
  • [ISSN] 0125-2208
  • [Journal-full-title] Journal of the Medical Association of Thailand = Chotmaihet thangphaet
  • [ISO-abbreviation] J Med Assoc Thai
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] Thailand
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide; U68WG3173Y / Carmustine
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11. Ertan Y, Sezak M, Turhan T, Kantar M, Erşahin Y, Mutluer S, Vergin C, Oniz H, Akalin T: Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases. Childs Nerv Syst; 2009 Jun;25(6):707-11
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  • [Title] Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases.
  • BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare aggressive infantile neoplasm of uncertain origin.
  • Tumors were located in the cerebellum (two cases), frontoparietal lobe (one case), and third ventricle (one case).
  • Histopathologically, the tumors were composed of rhabdoid cells and undifferentiated small cells mixed with epithelial or mesenchymal components.
  • However, one of the tumors was composed predominantly of a mesenchymal component mimicking a sarcoma.
  • Immunohistochemically, vimentin (4/4), epithelial membrane antigen (4/4), cytokeratin (3/4), smooth muscle actin (4/4), glial fibrillary acidic protein (4/4), S-100 (4/4), and synaptophysin (1/4) were positive in varying proportions, while desmin and INI-1 were negative in all the cases.
  • All of the patients died within a mean of 14 months due to tumor progression despite the chemotherapy.
  • Only one of our patients lived for 40 months after the diagnosis.
  • In conclusion, AT/RTs are aggressive tumors.
  • Morphologically, a large spectrum can be seen, like predominantly sarcoma in appearance, but immunohistochemistry is helpful in the correct diagnosis.
  • [MeSH-major] Brain Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology
  • [MeSH-minor] Actins / analysis. Brain / pathology. Brain Chemistry. Child. Child, Preschool. Chromosomal Proteins, Non-Histone / analysis. DNA-Binding Proteins / analysis. Desmin / analysis. Diagnosis, Differential. Female. Glial Fibrillary Acidic Protein / analysis. Humans. Immunohistochemistry. Infant. Keratins / analysis. Male. Mucin-1 / analysis. S100 Proteins / analysis. SMARCB1 Protein. Synaptophysin / analysis. Transcription Factors / analysis. Vimentin / analysis

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  • [CommentIn] Childs Nerv Syst. 2009 Nov;25(11):1387; author reply 1389 [19636570.001]
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  • (PMID = 19212771.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Actins; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Desmin; 0 / Glial Fibrillary Acidic Protein; 0 / Mucin-1; 0 / S100 Proteins; 0 / SMARCB1 Protein; 0 / SMARCB1 protein, human; 0 / Synaptophysin; 0 / Transcription Factors; 0 / Vimentin; 68238-35-7 / Keratins
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12. Sunyach MP, Pommier P, Martel Lafay I, Guyotat J, Ginestet G, Jouanneau E, Jouvet A, Sindou M, Bret P, Carrie C, Frappaz D: Conformal irradiation for pure and mixed oligodendroglioma: the experience of Centre Leon Berard Lyon. Int J Radiat Oncol Biol Phys; 2003 May 1;56(1):296-303
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  • [Title] Conformal irradiation for pure and mixed oligodendroglioma: the experience of Centre Leon Berard Lyon.
  • PURPOSE: To assess whether conformal radiotherapy (CRT) after incomplete surgery or biopsy for pure oligodendrogliomas and mixed gliomas results in decreased long-term sequelae without impairing local control and while reducing irradiated volume.
  • MATERIALS AND METHODS: Twenty-six consecutive patients who presented with pure (21) or mixed (5) oligodendrogliomas and who were given incomplete resections were treated according 3 different strategies: CRT alone (12), chemotherapy followed by CRT (4), and chemotherapy and delayed CRT at the time of tumor progression (10).
  • Median dose was 60 Gy.
  • Quality of CRT was assessed using tumor and normal tissue conformal indexes.
  • Among 11 nonevolutive patients, 6 have a full-time or part-time job.
  • CONCLUSIONS: Despite CRT, infield recurrence was a common feature in patients with oligodendrogliomas and mixed tumors.
  • Further research, including molecular biology typing of tumors and type of treatment, is warranted to improve survival and quality of life.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Glioma / radiotherapy. Oligodendroglioma / radiotherapy. Radiotherapy, Conformal
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biopsy. Brain Damage, Chronic / etiology. Brain Injuries / etiology. Chemotherapy, Adjuvant. Combined Modality Therapy. Disease Progression. Disease-Free Survival. Employment. Female. France / epidemiology. Humans. Life Tables. Lomustine. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local. Procarbazine. Psychological Tests. Quality of Life. Radiotherapy Dosage. Radiotherapy, Adjuvant. Social Adjustment. Surveys and Questionnaires. Survival Analysis. Survival Rate. Treatment Outcome. Vincristine

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  • (PMID = 12694851.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 35S93Y190K / Procarbazine; 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine; PCV protocol
  • [Number-of-references] 44
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13. Dumont AS, Farace E, Schiff D, Shaffrey ME: Intraventricular gliomas. Neurosurg Clin N Am; 2003 Oct;14(4):571-91
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  • [Title] Intraventricular gliomas.
  • Gliomas are the most common primary brain tumor in adults, and those within or relating to the ventricular surface represent a less common but important subcategory.
  • The most common intraventricular gliomas include ependymomas, SEs, and SEGAs.
  • Other less common varieties have been reported, including chordoid gliomas, glioblastoma multiforme, and mixed glial-neuronal tumors.
  • Each type of intraventricular glioma is associated with its own unique constellation of epidemiologic, clinical, radiologic, and pathologic defining characteristics.
  • Each tumor type has its own management considerations and nuances with unique prognostic indicators and outcomes.
  • The outcome for certain intraventricular gliomas (especially ependymomas) remains relatively poor.
  • Translational research aiming to advance the knowledge of tumor biology into new targeted cellular and molecular therapies holds tremendous promise to improve the overall outcome.
  • Additionally, more thorough delineation of prognostic factors as well as modifications and refinements to radiation and chemotherapy may help to improve the still significantly poor outcomes for patients harboring these lesions.
  • Future cooperative intra- and interinstitutional efforts between scientists and clinicians will hopefully culminate in an improved outlook and eventual cure for patients with gliomas.
  • [MeSH-major] Cerebral Ventricle Neoplasms / pathology. Glioma / pathology. Neurosurgical Procedures / methods
  • [MeSH-minor] Age Factors. Combined Modality Therapy. Diagnosis, Differential. Humans. Incidence. Magnetic Resonance Imaging. Prevalence

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  • (PMID = 15024802.001).
  • [ISSN] 1042-3680
  • [Journal-full-title] Neurosurgery clinics of North America
  • [ISO-abbreviation] Neurosurg. Clin. N. Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 159
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14. Chinot OL, Honore S, Dufour H, Barrie M, Figarella-Branger D, Muracciole X, Braguer D, Martin PM, Grisoli F: Safety and efficacy of temozolomide in patients with recurrent anaplastic oligodendrogliomas after standard radiotherapy and chemotherapy. J Clin Oncol; 2001 May 01;19(9):2449-55
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  • [Title] Safety and efficacy of temozolomide in patients with recurrent anaplastic oligodendrogliomas after standard radiotherapy and chemotherapy.
  • PURPOSE: Most primary oligodendrogliomas and mixed gliomas (oligoastrocytoma) respond to treatment with procarbazine, lomustine, and vincristine (PCV), with response rates of approximately 80%.
  • However, limited data on second-line treatments are available in patients with recurrent tumors.
  • This study describes the effects of temozolomide in patients with recurrent anaplastic oligodendroglioma (AO) and anaplastic mixed oligoastrocytoma (AOA).
  • PATIENTS AND METHODS: Forty-eight patients with histologically confirmed AO or AOA who had received previous PCV chemotherapy were treated with temozolomide (150 to 200 mg/m2/d for 5 days per 28-day cycle).
  • Secondary end points included progression-free survival (PFS), time to progression, overall survival (OS), safety, and tolerability.
  • For the entire treatment group, median PFS was 6.7 months and median OS was 10 months.
  • Twelve patients developed grade 1/2 thrombocytopenia and three patients developed grade 3/4 thrombocytopenia.
  • CONCLUSION: Temozolomide is safe and effective in the treatment of recurrent AO and AOA.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Astrocytoma / drug therapy. Brain Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Dacarbazine / therapeutic use. Neoplasm Recurrence, Local / drug therapy. Oligodendroglioma / drug therapy

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  • (PMID = 11331324.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; YF1K15M17Y / temozolomide
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15. Bauman GS, Cairncross JG: Multidisciplinary management of adult anaplastic oligodendrogliomas and anaplastic mixed oligo-astrocytomas. Semin Radiat Oncol; 2001 Apr;11(2):170-80
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  • [Title] Multidisciplinary management of adult anaplastic oligodendrogliomas and anaplastic mixed oligo-astrocytomas.
  • Once thought to be rare, oligodendroglial tumors might actually represent up to 25% of primary glial neoplasms.
  • In recent years, the histologic criteria for the diagnosis of oligodendroglioma have been broadened to include most small cell, monomorphic glial neoplasms.
  • These refinements have led to an increased recognition of oligodendroglial neoplasms, but uniform definitions of pure versus mixed oligodendroglioma as well as the criteria for high-grade (anaplastic) versus low-grade tumors remain elusive.
  • From a prognostic standpoint, the presence of an oligodendroglial component in a malignant glioma predicts longer survivals times for patients treated with surgery, and radiation therapy with or without chemotherapy.
  • High rates of response to PCV (procarbazine, CCNU and Vincristine) chemotherapy also have been noted among patients with anaplastic oligodendroglial neoplasms.
  • Ongoing prospective trials seek to clarify the role of PCV chemotherapy when added to radiation therapy and surgery.
  • In addition, the role of molecular markers as diagnostic aides and guides to therapy and prognosis are being explored for patients with pure and mixed anaplastic oligodendroglial tumors.
  • [MeSH-major] Brain Neoplasms / therapy. Glioma / therapy. Oligodendroglioma / therapy
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols. Combined Modality Therapy. Humans. Lomustine. Neoplasm Staging. Procarbazine. Prognosis. Treatment Outcome. Vincristine

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  • [Copyright] Copyright 2001 by W.B. Saunders Company
  • (PMID = 11285555.001).
  • [ISSN] 1053-4296
  • [Journal-full-title] Seminars in radiation oncology
  • [ISO-abbreviation] Semin Radiat Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 35S93Y190K / Procarbazine; 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine; PCV protocol
  • [Number-of-references] 56
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16. Newton HB, Dalton J, Ray-Chaudhury A, Gahbauer R, McGregor J: Aggressive papillary glioneuronal tumor: case report and literature review. Clin Neuropathol; 2008 Sep-Oct;27(5):317-24
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  • [Title] Aggressive papillary glioneuronal tumor: case report and literature review.
  • Papillary glioneuronal tumors (PGNT) are a rare, recently described form of mixed neoplasm composed of glial and neuronal components.
  • PGNT usually occur in children and young adults, and typically demonstrate low-grade pathology, with a low proliferative index of 1-3%.
  • Here we describe a newly diagnosed case of PGNT with a more aggressive phenotype that required irradiation and chemotherapy.
  • The patient was a 19-year-old female who developed progressive headaches and visual seizures.
  • Postoperative MRI scans showed patchy enhancement and residual T2 and FLAIR abnormality.
  • Pathology revealed a highly cellular neoplasm with papillary-like structures, containing cells with glial and neuronal differentiation.
  • Regions of mitoses and focal necrosis were noted, along with a Ki-67 labeling index of 26%.
  • The diagnosis was aggressive PGNT, and treatment consisted of conformal irradiation and concomitant temozolomide over 6 weeks.
  • Postirradiation follow-up MRI scans demonstrated a reduction of residual enhancement and FLAIR abnormality.
  • This patient demonstrates that PGNT may, in rare cases, display an aggressive clinicopathologic phenotype that requires a therapeutic approach more consistent with a high-grade glioma.
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Dacarbazine / analogs & derivatives. Dacarbazine / therapeutic use. Female. Humans. Magnetic Resonance Imaging. Radiotherapy, Conformal

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  • (PMID = 18808063.001).
  • [ISSN] 0722-5091
  • [Journal-full-title] Clinical neuropathology
  • [ISO-abbreviation] Clin. Neuropathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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17. Lee JR, Georgi DE, Wang BY: Malignant myoepithelial tumor of soft tissue: a report of two cases of the lower extremity and a review of the literature. Ann Diagn Pathol; 2007 Jun;11(3):190-8
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  • [Title] Malignant myoepithelial tumor of soft tissue: a report of two cases of the lower extremity and a review of the literature.
  • Myoepithelial tumors of the soft tissues have only recently been described.
  • Two cases of lower extremity malignant myoepithelial tumors are reported.
  • One case of malignant mixed tumor overlying the gastrocnemius muscle was treated with wide local excision, but metastasized to regional lymph nodes 14 months after surgical excision.
  • One patient with malignant myoepithelioma of the right lower leg was treated with limb amputation and is alive without disease at 46 months.
  • A review of the literature discloses 120 additional cases of soft tissue myoepithelial tumors, 102 of which are myoepitheliomas and 18 are mixed tumors.
  • Thirty-seven percent of the myoepitheliomas met the criteria for malignancy, and 33% of the mixed tumors were malignant.
  • Of these, 30% had locally recurrent disease and 32% developed metastatic disease.
  • Treatment benefit from chemotherapy and radiation therapy is unclear.
  • [MeSH-major] Myoepithelioma / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Calcium-Binding Proteins / metabolism. Cell Proliferation. Glial Fibrillary Acidic Protein / metabolism. Humans. Leg / pathology. Male. Microfilament Proteins / metabolism. Middle Aged. Mixed Tumor, Malignant / diagnosis. Mixed Tumor, Malignant / pathology. Necrosis / pathology. Phosphopyruvate Hydratase / metabolism. Vimentin / metabolism

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  • (PMID = 17498593.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Calcium-Binding Proteins; 0 / Glial Fibrillary Acidic Protein; 0 / Microfilament Proteins; 0 / Vimentin; 0 / calponin; EC 4.2.1.11 / Phosphopyruvate Hydratase
  • [Number-of-references] 35
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18. Olson JD, Riedel E, DeAngelis LM: Long-term outcome of low-grade oligodendroglioma and mixed glioma. Neurology; 2000 Apr 11;54(7):1442-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Long-term outcome of low-grade oligodendroglioma and mixed glioma.
  • BACKGROUND: Low-grade oligodendrogliomas and mixed gliomas can be indolent and remain unchanged for years.
  • Optimal timing and effectiveness of initial treatment is uncertain and therapy can be associated with toxicity.
  • METHODS: Retrospective review of patients diagnosed between 1979 and 1997 with low-grade oligodendroglioma or mixed glioma.
  • Time to progression, survival, prognostic factors, and treatment toxicities were evaluated.
  • RESULTS: A total of 106 patients (77 oligodendroglioma, 29 mixed glioma) were identified; median age was 36.7 years.
  • Initial presenting symptoms were seizures in 76 (72%) and headache in 11 (10%); tumor was diagnosed as an incidental finding in five patients.
  • Tumor progression was diagnosed in 72 patients (68%).
  • Overall median time to progression (MTTP) was 5.0 years (range 0.5 to 14.2).
  • MTTP and OS were not significantly affected by treatment.
  • Of 62 patients who received radiation therapy, 9 (15%) developed radiation necrosis and 13 developed radiation therapy-related cognitive changes, requiring ventriculoperitoneal shunting in six.
  • Significant myelosuppression was seen in 35 of 76 (46%) patients treated with chemotherapy.
  • CONCLUSIONS: Low-grade oligodendroglioma and mixed glioma have a long median overall survival.
  • There were no apparent differences in either immediate versus deferred treatment or choice of initial therapy on disease-free or overall survival.
  • Chemotherapy was associated with significant acute toxicity in almost one half of patients; radiation therapy produced late neurotoxicity in one third, justifying deferred treatment until clinically necessary.
  • [MeSH-major] Brain Neoplasms / therapy. Glioma / therapy. Neoplasm Recurrence, Local / diagnosis. Oligodendroglioma / therapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cisplatin / adverse effects. Cisplatin / therapeutic use. Cyclophosphamide / adverse effects. Cyclophosphamide / therapeutic use. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasms, Second Primary / diagnosis. Neoplasms, Second Primary / therapy. Postoperative Complications. Radiotherapy, Adjuvant / adverse effects. Retrospective Studies. Survival Rate. Treatment Outcome. Vindesine / adverse effects. Vindesine / therapeutic use


19. Markowska-Woyciechowska A, Zub L, Jarus-Dziedzic K, Rabczyński J, Paradowski B, Budrewicz S, Jabłoński P: [Dysembryoplastic neuroepithelial tumor (DNT)--case report and literature review]. Neurol Neurochir Pol; 2000 Sep-Oct;34(5):1031-8
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  • [Title] [Dysembryoplastic neuroepithelial tumor (DNT)--case report and literature review].
  • Neuroepithelial dysembryoplastic tumour was first described by Daumas-Duport in 1988 and in WHO classification was included into the group of neuronal and mixed neuroglial tumours.
  • This is a benign and very rare tumor with a good prognosis occurring in children and young adults.
  • Oligodendrocyte-like cells, glial and neuronal elements are usually found.
  • The tumour has no tendency for recurrence even in case of incomplete removal and does not require chemotherapy nor radiotherapy which is significantly important for accurate diagnosis, in order to avoid an aggressive therapy in young patients.
  • [MeSH-major] Brain Neoplasms / diagnosis. Neuroectodermal Tumors, Primitive / diagnosis. Parietal Lobe
  • [MeSH-minor] Adult. Epilepsy / etiology. Female. Humans. Tomography, X-Ray Computed

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  • (PMID = 11253470.001).
  • [ISSN] 0028-3843
  • [Journal-full-title] Neurologia i neurochirurgia polska
  • [ISO-abbreviation] Neurol. Neurochir. Pol.
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Poland
  • [Number-of-references] 20
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20. Jaeckle KA, Hess KR, Yung WK, Greenberg H, Fine H, Schiff D, Pollack IF, Kuhn J, Fink K, Mehta M, Cloughesy T, Nicholas MK, Chang S, Prados M, North American Brain Tumor Consortium: Phase II evaluation of temozolomide and 13-cis-retinoic acid for the treatment of recurrent and progressive malignant glioma: a North American Brain Tumor Consortium study. J Clin Oncol; 2003 Jun 15;21(12):2305-11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phase II evaluation of temozolomide and 13-cis-retinoic acid for the treatment of recurrent and progressive malignant glioma: a North American Brain Tumor Consortium study.
  • PURPOSE: Temozolomide (TMZ) and 13-cis-retinoic acid (cRA) have shown activity in prior single-agent trials of recurrent malignant gliomas (MG).
  • This phase II trial evaluated efficacy and toxicity of combination temozolomide and cRA treatment in recurrent MG.
  • PATIENTS AND METHODS: Adults with recurrent supratentorial MG for whom surgery, radiation, and/or chemotherapy failed were eligible.
  • Treatment included oral TMZ 150 or 200 mg/m2/d, days 1 through 5, and cRA 100 mg/m2/d, days 1 to 21, every 28 days.
  • RESULTS: Eighty-eight eligible patients (glioblastoma multiforme [n = 40]; anaplastic gliomas [n = 48; astrocytoma, 28; oligodendroglioma, 14; mixed glioma, six]) received treatment.
  • Among 499 treatment cycles, the most common grade 3/4 events included granulocytopenia (1.8%), thrombocytopenia (1.4%), and hypertriglyceridemia (1.2%).
  • CONCLUSION: TMZ and cRA were active, exceeding our 20% thresholds for PFS 6 success, assuming 20% improvement over our previously reported database (glioblastoma multiforme: expected, 30%; observed, 32%; anaplastic glioma: expected, 40%; observed, 50%).
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Dacarbazine / analogs & derivatives. Glioma / drug therapy. Supratentorial Neoplasms / drug therapy
  • [MeSH-minor] Adult. Antineoplastic Agents, Alkylating / administration & dosage. Disease Progression. Female. Humans. Isotretinoin / administration & dosage. Male. Middle Aged. Neoplasm Recurrence, Local. Survival Analysis. Treatment Outcome

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  • (PMID = 12805331.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA16672; United States / NCI NIH HHS / CA / CA62399; United States / NCI NIH HHS / CA / CA62412; United States / NCI NIH HHS / CA / CA62422; United States / NCI NIH HHS / CA / CA62426; United States / NCI NIH HHS / CA / CA62455; United States / NCRR NIH HHS / RR / M01-RR00042; United States / NCRR NIH HHS / RR / M01-RR00633; United States / NCRR NIH HHS / RR / M01-RR03186; United States / NCRR NIH HHS / RR / M01-RR0865; United States / NCRR NIH HHS / RR / MO1-RR00056; United States / NCI NIH HHS / CA / UO1CA62399; United States / NCI NIH HHS / CA / UO1CA62405; United States / NCI NIH HHS / CA / UO1CA62407-08; United States / NCI NIH HHS / CA / UO1CA62421
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide; EH28UP18IF / Isotretinoin
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21. Psarros TG, Swift D, Mulne AF, Burns DK: Neurocytoma-like neoplasm of the thoracic spine in a 15-month-old child presenting with diffuse leptomeningeal dissemination and communicating hydrocephalus. Case report. J Neurosurg; 2005 Aug;103(2 Suppl):184-90
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  • [Title] Neurocytoma-like neoplasm of the thoracic spine in a 15-month-old child presenting with diffuse leptomeningeal dissemination and communicating hydrocephalus. Case report.
  • This unusual mixed glioneuronal neoplasm of the spine resembling central neurocytoma is only the second reported example of a neoplasm of this type involving the spinal cord and is, seemingly, the first to present with diffuse leptomeningeal dissemination and communicating hydrocephalus.
  • This 15-month-old boy presented with somnolence, bilateral sixth nerve palsy, and lower-extremity weakness and was found to harbor a primary neoplasm within the thoracic spine and associated syrinx, widespread leptomeningeal dissemination, and communicating hydrocephalus.
  • The patient underwent cerebrospinal fluid shunt placement, thoracic laminectomy for tumor debulking and biopsy, chemotherapy, and radiation therapy to the neuraxis.
  • Immunohistochemically, the tumor was marked by evidence of mixed glial (glial fibrillary acidic protein-positive) and neuronal (synaptophysin-positive) differentiation.
  • Mixed glioneuronal neoplasms are a heterogeneous group of tumors whose biological potential remains incompletely defined.
  • The authors' goal is to acquaint neurosurgeons with the expanding spectrum of mixed glioneuronal neoplasms and with the potential of some of these lesions to pursue an aggressive clinical course.
  • [MeSH-major] Arachnoid. Hydrocephalus / etiology. Neurocytoma / diagnosis. Pia Mater. Spinal Cord Neoplasms / diagnosis
  • [MeSH-minor] Cerebrospinal Fluid Shunts. Combined Modality Therapy. Humans. Immunohistochemistry. Infant. Laminectomy. Magnetic Resonance Imaging. Male. Neoplasm Invasiveness. Reoperation. Thoracic Vertebrae. Tomography, X-Ray Computed

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  • (PMID = 16370289.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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22. Halperin EC: Neonatal neoplasms. Int J Radiat Oncol Biol Phys; 2000 Apr 1;47(1):171-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PURPOSE: To describe neoplasms diagnosed in children </= 28 days of age along with their treatment, associated congenital anomalies, and the long-term consequences of the diagnoses and treatments.
  • METHODS AND MATERIALS: Utilizing autopsy records, a computerized tumor registry, and medical records, we identified patients and stillborns at Duke University Medical Center (DUMC) diagnosed with neoplasms at </= 28 days of age between 1930 and 1998.
  • The 20 patients identified via the computerized registry system for 1980-1998 constitute 2% (20/925) of all neoplasms seen in patients </= 16 years of age over this same time period at DUMC.
  • The histologic diagnoses were teratoma/germ cell tumor (n = 8, 35%), neuroblastoma (n = 5, 22%), retinoblastoma (n = 4, 17%), primary central nervous system (CNS) tumor (n = 3, 13%), and one case each of rhabdomyosarcoma, glossal glial choristoma, and hemangioma in the setting of Kasabach-Merritt Syndrome.
  • Of the eight teratoma/germ cell tumor patients, 6 were female (75%) and 2 male (25%).
  • There was one malignant germ cell tumor, 2 immature teratomas, and 5 teratomas.
  • The one patient with malignant germ cell tumor, treated with surgery and chemotherapy, died.
  • Two were treated with surgery + chemotherapy + radiotherapy; two with surgery + chemotherapy; and one with surgery alone.
  • Associated congenital anomalies and medical problems were ventricular septal defect, seizure disorder, and Fanconi's anemia.
  • A child with a dumbbell neuroblastoma, treated with surgery and chemotherapy, is paraplegic.
  • The two children with trilateral retinoblastoma died after therapy with surgery, craniospinal and orbital irradiation, and chemotherapy.
  • Two children with bilateral disease are long-term survivors: one treated with radiotherapy + chemotherapy and one with radiotherapy alone.
  • The three patients with CNS tumors were female.
  • The histologies were glioblastoma multiforme, anaplastic astrocytoma, and malignant mixed oligodendroglioma.
  • Two of the patients are long-term survivors after surgery + chemotherapy.
  • Six children received eight courses of radiation therapy: 2 for Stage 4S neuroblastoma with respiratory compromise from an enlarging liver and 4 for retinoblastoma.
  • The two infants with trilateral retinoblastoma received two courses of irradiation each: one of the treatment of intraocular tumor and a second, at an older age, for the pineal tumor.
  • CONCLUSION: The most common neonatal neoplasm histologic diagnoses are teratoma/germ cell tumor, neuroblastoma, and retinoblastoma.
  • Radiation therapy is administered infrequently in a population highly susceptible to late ill effects.
  • When radiotherapy is required, anesthesia may be repetitively administered to aid in reproducible treatment.
  • [MeSH-minor] Anesthesia. Brain Neoplasms / epidemiology. Brain Neoplasms / pathology. Brain Neoplasms / therapy. Female. Follow-Up Studies. Hemangioma / epidemiology. Hemangioma / pathology. Hemangioma / therapy. Humans. Infant, Newborn. Male. Neuroblastoma / epidemiology. Neuroblastoma / pathology. Neuroblastoma / therapy. Registries. Retinoblastoma / epidemiology. Retinoblastoma / pathology. Retinoblastoma / therapy. Survivors. Teratoma / epidemiology. Teratoma / pathology. Teratoma / therapy

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  • (PMID = 10758320.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
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23. Marti A, Almostarchid B, Maher M, Saidi A: Desmoplastic non-infantile ganglioglioma. Case report. J Neurosurg Sci; 2000 Sep;44(3):150-4
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  • Desmoplastic gangliogliomas are rare mixed glial and neuronal cerebral tumors, especially described in infants below 4 years of age but exceptional cases have been reported in young adults.
  • These tumors are generally localised in parietal or temporal lobes, present as a large cystic lesion with peripheral contrast enhancement.
  • They also have characteristic histological features: extensive desmoplasia and tumoral cells of variable size exhibiting immunohistochemical and ultrastructural features of glial and neuronal differentiation.
  • Total surgical removal is sufficient for the treatment of these tumors and no radiotherapy or chemotherapy are indicated if complete resection is achieved.
  • This tumor presented as a large parieto- temporal cystic lesion with rimmed contrast enhancement.
  • At histological examination, this tumor exhibited extensive desmoplasia and comprised 2 types of tumoral cells: small cells with round nuclei, positive for NSE, neurofilaments and synaptophysin and sometimes presenting typical morphological features of neuronal differentiation, and large cells with abundant eosinophilic strongly staining for GFAP.
  • This observation emphazises on the fact that desmoplastic ganglioglioma can no more be considered as a specific entity of infancy and must be well recognised even in young adults because it may be misdiagnosed as malignant glioma.
  • [MeSH-major] Brain Neoplasms / diagnosis. Ganglioglioma / diagnosis. Parietal Lobe. Temporal Lobe
  • [MeSH-minor] Adult. Astrocytes / pathology. Cysts / diagnosis. Cysts / pathology. Humans. Male. Neurosurgical Procedures. Reoperation. Tomography, X-Ray Computed


24. Herholz K, Kracht LW, Heiss WD: Monitoring the effect of chemotherapy in a mixed glioma by C-11-methionine PET. J Neuroimaging; 2003 Jul;13(3):269-71
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  • [Title] Monitoring the effect of chemotherapy in a mixed glioma by C-11-methionine PET.
  • The effect of chemotherapy with procarbazine, CCNU, and vincristine in an anaplastic oligoastrocytoma was monitored by repeated positron emission tomography (PET) with C-11-methionine (C-11-MET).
  • Chemotherapy caused a continuous decline of active tumor volume at a rate of approximately 2.4% per day, resulting in complete remission that persisted until the end of follow-up at 3 years.
  • Thus, the authors conclude that C-11-MET PET may be useful for monitoring chemotherapy in gliomas and deserves further study.
  • [MeSH-major] Astrocytoma / radionuclide imaging. Brain Neoplasms / radionuclide imaging. Cerebral Cortex / radionuclide imaging. Drug Monitoring / methods. Tomography, Emission-Computed / methods
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Female. Humans. Methionine. Radiopharmaceuticals. Treatment Outcome

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  • (PMID = 12889176.001).
  • [ISSN] 1051-2284
  • [Journal-full-title] Journal of neuroimaging : official journal of the American Society of Neuroimaging
  • [ISO-abbreviation] J Neuroimaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 58576-49-1 / carbon-11 methionine; AE28F7PNPL / Methionine
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25. Beauchesne PD, Taillandier L, Bernier V, Carnin C: Concurrent radiotherapy: fotemustine combination for newly diagnosed malignant glioma patients, a phase II study. Cancer Chemother Pharmacol; 2009 Jun;64(1):171-5
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  • [Title] Concurrent radiotherapy: fotemustine combination for newly diagnosed malignant glioma patients, a phase II study.
  • PURPOSE: Fotemustine is a nitrosourea compound used for the treatment of malignant gliomas, especially in France.
  • Recently, an EORTC-NCIC study has shown that a concomitant combination of radiotherapy plus temozolomide (an oral cytotoxic drug) improved survival in glioblastoma patients.
  • We set out to test a concurrent combination of radiotherapy and fotemustine for newly malignant gliomas.
  • Patients over 18 years of age able to give informed consent and with histologically proven, newly diagnosed supratentorial malignant gliomas were eligible.
  • All patients were treated by a standard cranial irradiation (conformal irradiation, tumor bulk plus a margin of 2.5 cm) and concomitant daily administration of 10 mg/m(2) of fotemustine (5 days per week, 6 weeks, 1 h 30 min before radiation therapy).
  • Adjuvant chemotherapy, fotemustine, was administered at tumor progression as standard and classic regimen.
  • Histology included 16 glioblastomas, 3 anaplastic astrocytomas, 2 anaplastic oligodendrogliomas and 1 mixed glioma.
  • Median survival from the initial diagnosis was 9.9 months, two patients are currently alive.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Glioma / drug therapy. Nitrosourea Compounds / therapeutic use. Organophosphorus Compounds / therapeutic use. Supratentorial Neoplasms / drug therapy
  • [MeSH-minor] Adult. Aged. Chemotherapy, Adjuvant / methods. Combined Modality Therapy. Disease Progression. Female. Hematologic Diseases / etiology. Humans. Male. Middle Aged. Prospective Studies. Stereotaxic Techniques. Survival Rate

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  • (PMID = 19352662.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Nitrosourea Compounds; 0 / Organophosphorus Compounds; GQ7JL9P5I2 / fotemustine
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26. Prayson RA, Abramovich CM: Glioneuronal tumor with neuropil-like islands. Hum Pathol; 2000 Nov;31(11):1435-8
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  • [Title] Glioneuronal tumor with neuropil-like islands.
  • Mixed glioneuronal neoplasms are relatively uncommon tumors in the central nervous system.
  • Recently, an unusual glioneuronal tumor arising in adults marked histologically by neuropil-like islands was described.
  • The patient underwent partial resection of the tumor, which histologically resembled anaplastic astrocytoma, and received a course of radiation therapy and chemotherapy.
  • Increasing seizure frequency and expanding size on neuroimaging prompted a re-excision of the tumor.
  • The second resection was marked by islands of tissue resembling gray matter with slightly atypical neuronal and glial cells situated in the white matter.
  • These islands stained positively with synaptophysin and did not stain with glial fibrillary acid protein.
  • Mild vascular proliferation and moderate nuclear pleomorphism also characterized the tumor.
  • P53 immunoreactivity was observed in approximately 40% of tumor cell nuclei.
  • This lesion is felt to represent a clinically aggressive glioneuronal neoplasm with an unusual and distinctive histologic phenotype.
  • [MeSH-minor] Adult. Antigens, Nuclear. Cell Nucleus / chemistry. Cell Nucleus / pathology. Female. Humans. Immunoenzyme Techniques. Ki-67 Antigen. Neovascularization, Pathologic / pathology. Nuclear Proteins / analysis. Reoperation. Synaptophysin / analysis. Treatment Outcome. Tumor Suppressor Protein p53 / analysis

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  • [Copyright] Copyright 2000 by W.B. Saunders Company
  • (PMID = 11112223.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Antigens, Nuclear; 0 / Ki-67 Antigen; 0 / Nuclear Proteins; 0 / Synaptophysin; 0 / Tumor Suppressor Protein p53
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27. Abrey LE, Childs BH, Paleologos N, Kaminer L, Rosenfeld S, Salzman D, Finlay JL, Gardner S, Peterson K, Hu W, Swinnen L, Bayer R, Forsyth P, Stewart D, Smith AM, Macdonald DR, Weaver S, Ramsey DA, Nimer SD, DeAngelis LM, Cairncross JG: High-dose chemotherapy with stem cell rescue as initial therapy for anaplastic oligodendroglioma. J Neurooncol; 2003 Nov;65(2):127-34
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] High-dose chemotherapy with stem cell rescue as initial therapy for anaplastic oligodendroglioma.
  • PURPOSE: Anaplastic oligodendroglioma is a chemosensitive glial neoplasm.
  • PATIENTS AND METHODS: Sixty-nine patients with a median age of 42 (range: 18-67) and a median Karnofsky Performance Score of 90 (range: 70-100) were enrolled.
  • Sixteen patients had a prior diagnosis of low-grade oligodendroglioma and 16 had mixed oligoastrocytoma pathology.
  • Only patients with demonstrably chemosensitive enhancing tumors or those free of enhancing tumor after surgery and induction therapy were eligible to receive high-dose thiotepa.
  • Neither histology nor prior low-grade oligodendroglioma correlated with relapse; however, persistent non-enhancing tumor at transplant conferred an increased risk of relapse (p = 0.028).
  • The transplant regimen was well-tolerated; median hospital stay was 20 days (range: 7-43) with a median time to ANC and platelet engraftment of 10 days.
  • Thirty patients (43%) did not receive high-dose thiotepa because of stable or progressive disease (n = 21), excessive toxicity (n = 4), refusal of further therapy (n = 2), failure to obtain insurance coverage (n = 2), or other (n = 1).
  • No treatment-related or long-term neurotoxicity was seen in the transplanted patients.
  • CONCLUSIONS: High-dose chemotherapy with stem cell rescue as initial treatment for anaplastic oligodendroglioma is feasible and associated with prolonged tumor control in some patients.
  • [MeSH-major] Antineoplastic Agents, Alkylating / administration & dosage. Brain Neoplasms / drug therapy. Hematopoietic Stem Cell Transplantation. Oligodendroglioma / drug therapy. Thiotepa / administration & dosage
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Combined Modality Therapy. Female. Humans. Lomustine / administration & dosage. Male. Middle Aged. Procarbazine / administration & dosage. Prognosis. Prospective Studies. Survival Rate. Vincristine / administration & dosage

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  • (PMID = 14686732.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 35S93Y190K / Procarbazine; 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine; 905Z5W3GKH / Thiotepa; PCV protocol
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