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1. Herrlinger U, Steinbrecher A, Rieger J, Hau P, Kortmann RD, Meyermann R, Schabet M, Bamberg M, Dichgans J, Bogdahn U, Weller M: Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse. J Neurol; 2005 Mar;252(3):291-9
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  • [Title] Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse.
  • Adult medulloblastoma is a rare tumor with few retrospective studies published so far.
  • This study reports therapy and outcome in all adult (>or=16 years old) medulloblastoma (n=34) and supratentorial primitive neuroectodermal tumor (PNET) patients (n=2) treated in 2 neuro-oncological centers between 1976 and 2002.
  • In conclusion, adjuvant chemotherapy may prolong survival in adult medulloblastoma patients.
  • [MeSH-minor] Adolescent. Adult. Aged. Analysis of Variance. Combined Modality Therapy. Demography. Disease Progression. Disease-Free Survival. Dose-Response Relationship, Radiation. Drug Therapy / methods. Female. Humans. Male. Middle Aged. Radiotherapy, High-Energy / methods. Recurrence. Regression Analysis. Retrospective Studies. Risk Factors. Time Factors. Treatment Outcome

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  • (PMID = 16189725.001).
  • [ISSN] 0340-5354
  • [Journal-full-title] Journal of neurology
  • [ISO-abbreviation] J. Neurol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] Germany
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2. Chang Q, Ng HK: [Different hypermethylation status of RASSF1A in medulloblastoma and supratentorial primitive neuroectodermal tumor]. Zhonghua Bing Li Xue Za Zhi; 2007 Jan;36(1):24-8
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  • [Title] [Different hypermethylation status of RASSF1A in medulloblastoma and supratentorial primitive neuroectodermal tumor].
  • OBJECTIVE: To investigate the epigenetic involvement of RASSF1A in intracranial primitive neuroectodermal tumors (PNETs) and compare the methylation patterns between medulloblastoma (MBs) and supratentorial PNETs (SPNETs).
  • RASSF1A-deficient PNET cell lines were treated with 5-aza-2'deoxycytidine, a demethylating agent, to explore the relationship between hypermethylation and the gene expression.
  • These results demonstrated that such epigenetic alteration was tumor-specific.
  • [MeSH-major] Brain Neoplasms / genetics. Cerebellar Neoplasms / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Cell Line, Tumor. Child. Child, Preschool. DNA Methylation. Female. Gene Expression Regulation, Neoplastic. Gene Silencing. HeLa Cells. Humans. Infant. Male. Promoter Regions, Genetic / genetics. Reverse Transcriptase Polymerase Chain Reaction. Young Adult


3. Lewandowicz GM, Harding B, Harkness W, Hayward R, Thomas DG, Darling JL: Chemosensitivity in childhood brain tumours in vitro: evidence of differential sensitivity to lomustine (CCNU) and vincristine. Eur J Cancer; 2000 Oct;36(15):1955-64
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  • The aim of this study was to examine the range of sensitivity of a panel of short-term cultures derived from different types of malignant childhood brain tumours including medulloblastoma, ependymoma and glioblastoma multiforme to three cytotoxic drugs, lomustine (CCNU), vincristine (VCR) and procarbazine (PCB).
  • Short-term cell lines derived from ependymomas were considerably more resistant to VCR than other types of childhood brain tumours, while cultures derived from supratentorial primitive neuroectodermal tumour (PNET) displayed marked sensitivity to both lomustine and VCR.
  • Cultures from ependymomas, medulloblastoma and astrocytic gliomas had similar sensitivity to lomustine and PCB as cultures derived from adult malignant astrocytoma.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Brain Neoplasms / drug therapy
  • [MeSH-minor] Adult. Astrocytoma / drug therapy. Child. Dose-Response Relationship, Drug. Drug Screening Assays, Antitumor. Ependymoma / drug therapy. Female. Glioblastoma / drug therapy. Humans. Lomustine / therapeutic use. Male. Medulloblastoma / drug therapy. Procarbazine / therapeutic use. Tumor Cells, Cultured / drug effects. Vincristine / therapeutic use

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  • (PMID = 11000577.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 35S93Y190K / Procarbazine; 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine
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4. Didiano D, Shalaby T, Lang D, Grotzer MA: Telomere maintenance in childhood primitive neuroectodermal brain tumors. Neuro Oncol; 2004 Jan;6(1):1-8
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  • [Title] Telomere maintenance in childhood primitive neuroectodermal brain tumors.
  • Primitive neuroectodermal tumors (PNETs), including medulloblastoma (PNET/MB) and supratentorial PNET (sPNET), are the most common malignant brain tumors of childhood.
  • [MeSH-major] Brain Neoplasms / enzymology. Catechin / analogs & derivatives. Neuroectodermal Tumors, Primitive / enzymology. Telomere / enzymology
  • [MeSH-minor] Adolescent. Adult. Cell Line, Tumor. Cell Survival / drug effects. Cell Survival / physiology. Child. Child, Preschool. DNA-Binding Proteins. Dose-Response Relationship, Drug. Female. Fetus. Humans. Infant. Male. Middle Aged. Telomerase / antagonists & inhibitors. Telomerase / biosynthesis. Telomerase / genetics

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  • (PMID = 14769133.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 8R1V1STN48 / Catechin; BQM438CTEL / epigallocatechin gallate; EC 2.7.7.49 / Telomerase
  • [Other-IDs] NLM/ PMC1871965
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5. Lindsey JC, Lusher ME, Strathdee G, Brown R, Gilbertson RJ, Bailey S, Ellison DW, Clifford SC: Epigenetic inactivation of MCJ (DNAJD1) in malignant paediatric brain tumours. Int J Cancer; 2006 Jan 15;118(2):346-52
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  • Methylation-dependent transcriptional silencing of MCJ has been observed in ovarian cancers and associated with increased resistance to chemotherapeutic agents; however, its role in other cancer types has not been widely investigated.
  • We examined the status of MCJ in intracranial primitive neuroectodermal tumours [PNETs, comprising cerebellar PNETs (medulloblastomas) and supratentorial PNETs (stPNETs)] and ependymomas, together representing the most common malignant brain tumours of childhood.
  • [MeSH-major] Brain Neoplasms / genetics. Ependymoma / genetics. Epigenesis, Genetic. HSP40 Heat-Shock Proteins / biosynthesis. Membrane Proteins / biosynthesis. Neuroectodermal Tumors, Primitive / genetics
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor. Child. Child, Preschool. DNA Methylation. Female. Gene Expression Profiling. Gene Silencing. Humans. Male. Reverse Transcriptase Polymerase Chain Reaction

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  • [Copyright] Copyright 2005 Wiley-Liss, Inc.
  • (PMID = 16049974.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNAJC1 protein, human; 0 / HSP40 Heat-Shock Proteins; 0 / Membrane Proteins
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6. Chang Q, Pang JC, Li KK, Poon WS, Zhou L, Ng HK: Promoter hypermethylation profile of RASSF1A, FHIT, and sFRP1 in intracranial primitive neuroectodermal tumors. Hum Pathol; 2005 Dec;36(12):1265-72
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  • [Title] Promoter hypermethylation profile of RASSF1A, FHIT, and sFRP1 in intracranial primitive neuroectodermal tumors.
  • Medulloblastomas (MBs) and supratentorial primitive neuroectodermal tumors (SPNETs) are histologically alike intracranial PNETs found in different anatomical locations of the brain.
  • The aim of this study was to investigate whether promoter hypermethylation of putative tumor suppressor genes was involved in both types of intracranial PNETs.
  • Treatment of RASSF1A-deficient PNET cell lines with 5-aza-2'deoxycytidine, a demethylating agent, restored RASSF1A expression, providing evidence that promoter hypermethylation contributes to transcriptional silencing.
  • [MeSH-major] Acid Anhydride Hydrolases / genetics. Brain Neoplasms / genetics. Cell Cycle Proteins / genetics. DNA Methylation. Neoplasm Proteins / genetics. Neuroectodermal Tumors, Primitive / genetics. Protein-Serine-Threonine Kinases / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Ataxia Telangiectasia Mutated Proteins. Cell Line, Tumor. Child. Child, Preschool. DNA Primers / chemistry. DNA, Neoplasm / analysis. Female. Humans. Infant. Male. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 16311119.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cell Cycle Proteins; 0 / DNA Primers; 0 / DNA, Neoplasm; 0 / Neoplasm Proteins; 0 / RASSF1 protein, human; 0 / Tumor Suppressor Proteins; 0 / fragile histidine triad protein; EC 2.7.11.1 / ATR protein, human; EC 2.7.11.1 / Ataxia Telangiectasia Mutated Proteins; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 3.6.- / Acid Anhydride Hydrolases
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7. Nordfors K, Haapasalo J, Korja M, Niemelä A, Laine J, Parkkila AK, Pastorekova S, Pastorek J, Waheed A, Sly WS, Parkkila S, Haapasalo H: The tumour-associated carbonic anhydrases CA II, CA IX and CA XII in a group of medulloblastomas and supratentorial primitive neuroectodermal tumours: an association of CA IX with poor prognosis. BMC Cancer; 2010;10:148
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  • [Title] The tumour-associated carbonic anhydrases CA II, CA IX and CA XII in a group of medulloblastomas and supratentorial primitive neuroectodermal tumours: an association of CA IX with poor prognosis.
  • BACKGROUND: Medulloblastomas (MBs) and supratentorial primitive neuroectodermal tumours (PNETs) are the most common highly aggressive paediatric brain tumours.
  • In spite of extensive research on these tumours, there are only few known biomarkers or therapeutic target proteins, and the prognosis of patients with these tumours remains poor.
  • [MeSH-major] Antigens, Neoplasm / analysis. Biomarkers, Tumor / analysis. Carbonic Anhydrase II / analysis. Carbonic Anhydrases / analysis. Cerebellar Neoplasms / enzymology. Medulloblastoma / enzymology. Neuroectodermal Tumors, Primitive / enzymology. Supratentorial Neoplasms / enzymology
  • [MeSH-minor] Adolescent. Adult. Aged. Apoptosis. Chi-Square Distribution. Child. Child, Preschool. Cytoplasm / enzymology. Endothelial Cells / enzymology. Female. Finland. Humans. Immunohistochemistry. Infant. Infant, Newborn. Kaplan-Meier Estimate. Male. Middle Aged. Odds Ratio. Proportional Hazards Models. Time Factors. Treatment Outcome. Young Adult

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  • (PMID = 20398423.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; EC 4.2.1.- / Carbonic Anhydrase II; EC 4.2.1.1 / CA9 protein, human; EC 4.2.1.1 / Carbonic Anhydrases; EC 4.2.1.1 / carbonic anhydrase XII
  • [Other-IDs] NLM/ PMC2874782
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8. Strother D, Ashley D, Kellie SJ, Patel A, Jones-Wallace D, Thompson S, Heideman R, Benaim E, Krance R, Bowman L, Gajjar A: Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study. J Clin Oncol; 2001 May 15;19(10):2696-704
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study.
  • PURPOSE: This study was designed to determine the feasibility and safety of delivering four consecutive cycles of high-dose cyclophosphamide, cisplatin, and vincristine, each followed by stem-cell rescue, every 4 weeks, after completion of risk-adapted craniospinal irradiation to children with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor (PNET).
  • CONCLUSION: Administering four consecutive cycles of high-dose chemotherapy with stem-cell support after surgical resection and craniospinal irradiation is feasible in newly diagnosed patients with medulloblastoma/supratentorial PNET with aggressive supportive care.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy
  • [MeSH-minor] Adolescent. Adult. Blood Transfusion. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Feasibility Studies. Female. Humans. Male. Stem Cells / drug effects. Topotecan / administration & dosage. Vincristine / administration & dosage

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  • Hazardous Substances Data Bank. Topotecan .
  • Hazardous Substances Data Bank. CIS-DIAMINEDICHLOROPLATINUM .
  • Hazardous Substances Data Bank. CYCLOPHOSPHAMIDE .
  • Hazardous Substances Data Bank. VINCRISTINE .
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  • (PMID = 11352962.001).
  • [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 / R25 CA023944; United States / NCI NIH HHS / CA / CA 21765; United States / NCI NIH HHS / CA / P01 CA 23009
  • [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] 5J49Q6B70F / Vincristine; 7M7YKX2N15 / Topotecan; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
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9. Stewart CF, Iacono LC, Chintagumpala M, Kellie SJ, Ashley D, Zamboni WC, Kirstein MN, Fouladi M, Seele LG, Wallace D, Houghton PJ, Gajjar A: Results of a phase II upfront window of pharmacokinetically guided topotecan in high-risk medulloblastoma and supratentorial primitive neuroectodermal tumor. J Clin Oncol; 2004 Aug 15;22(16):3357-65
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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 phase II upfront window of pharmacokinetically guided topotecan in high-risk medulloblastoma and supratentorial primitive neuroectodermal tumor.
  • PURPOSE: To assess the antitumor efficacy of pharmacokinetically guided topotecan dosing in previously untreated patients with medulloblastoma and supratentorial primitive neuroectodermal tumors, and to evaluate plasma and CSF disposition of topotecan in these patients.
  • CONCLUSION: Topotecan is an effective agent against pediatric medulloblastoma in patients who have received no therapy other than surgery.
  • This drug warrants testing as part of standard postradiation chemotherapeutic regimens.
  • Furthermore, these results emphasize the importance of translational research in drug development, which in this case identified an effective drug.
  • [MeSH-major] Antineoplastic Agents / pharmacokinetics. Antineoplastic Agents / therapeutic use. Brain Neoplasms / drug therapy. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Topotecan / pharmacokinetics. Topotecan / therapeutic use
  • [MeSH-minor] Adolescent. Adult. Area Under Curve. Child. Child, Preschool. Female. Humans. Infusions, Intravenous. Male. Risk Factors. Treatment Outcome






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