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1. Timmermann B, Kortmann RD, Kühl J, Meisner C, Dieckmann K, Pietsch T, Bamberg M: Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91. J Clin Oncol; 2002 Feb 01;20(3):842-9
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  • [Title] Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91.
  • PURPOSE: To evaluate the outcome of children with supratentorial primitive neuroectodermal tumors after surgery, irradiation, and chemotherapy and to identify factors predictive for survival.
  • Irradiation volume was recommended to encompass the neuraxis with 35.2-Gy total dose followed by a boost (20.0 Gy) to the primary tumor site (n = 54).
  • Seven patients were irradiated to the tumor region only with a total dose of 54.0 Gy.
  • There was a positive trend in outcome for nonmetastatic and pineal tumors.
  • Volume of irradiation should encompass the whole CNS with additional boost to the tumor region.
  • [MeSH-major] Neuroectodermal Tumors / radiotherapy. Supratentorial Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cytarabine / administration & dosage. Drug Administration Schedule. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Lomustine / administration & dosage. Male. Methotrexate / administration & dosage. Neoplasm Recurrence, Local. Radiotherapy Dosage. Survival Rate. Treatment Outcome. Vincristine / administration & dosage


2. Massimino M, Gandola L, Giangaspero F, Sandri A, Valagussa P, Perilongo G, Garrè ML, Ricardi U, Forni M, Genitori L, Scarzello G, Spreafico F, Barra S, Mascarin M, Pollo B, Gardiman M, Cama A, Navarria P, Brisigotti M, Collini P, Balter R, Fidani P, Stefanelli M, Burnelli R, Potepan P, Podda M, Sotti G, Madon E, AIEOP Pediatric Neuro-Oncology Group: Hyperfractionated radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (Associazione Italiana di Ematologia-Oncologia Pediatrica) study. Int J Radiat Oncol Biol Phys; 2004 Apr 1;58(5):1336-45
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  • [Title] Hyperfractionated radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (Associazione Italiana di Ematologia-Oncologia Pediatrica) study.
  • RESULTS: Sixty-three consecutive children were enrolled: 46 NED, 17 ED; the tumor was infratentorial in 47 and supratentorial in 16, with spinal metastasis in 1.
  • CONCLUSIONS: HFRT, despite the high total dose adopted, did not change the prognosis of childhood ependymoma as compared to historical series: New radiotherapeutic approaches are needed to improve local control.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / radiotherapy. Ependymoma / drug therapy. Ependymoma / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Child. Child, Preschool. Cyclophosphamide / administration & dosage. Dose Fractionation. Etoposide / administration & dosage. Feasibility Studies. Humans. Infratentorial Neoplasms / drug therapy. Infratentorial Neoplasms / radiotherapy. Infratentorial Neoplasms / surgery. Patient Compliance. Prospective Studies. Radiotherapy, Adjuvant. Supratentorial Neoplasms / drug therapy. Supratentorial Neoplasms / radiotherapy. Supratentorial Neoplasms / surgery. Survival Analysis. Vincristine / administration & dosage

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  • (PMID = 15050308.001).
  • [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; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide
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3. Yanofsky RA, Seshia SS, Dawson AJ, Stobart K, Greenberg CR, Booth FA, Prasad C, Del Bigio MR, Wrogemann JJ, Fike F, Gatti RA: Ataxia-telangiectasia: atypical presentation and toxicity of cancer treatment. Can J Neurol Sci; 2009 Jul;36(4):462-7
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  • BACKGROUND: The onset of progressive cerebellar ataxia in early childhood is considered a key feature of ataxia-telangiectasia (A-T), accompanied by ocular apraxia, telangiectasias, immunodeficiency, cancer susceptibility and hypersensitivity to ionizing radiation.
  • The two children who received cranial irradiation developed supratentorial primitive neuroectodermal tumors of the brain, an association not previously described, with fatal outcomes.
  • Radiation therapy and radiomimetic drugs should be avoided in individuals with A-T.
  • [MeSH-major] Antineoplastic Agents / adverse effects. Ataxia Telangiectasia / chemically induced. Ataxia Telangiectasia / diagnosis. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy
  • [MeSH-minor] Adolescent. Ataxia Telangiectasia Mutated Proteins. Cell Cycle Proteins / metabolism. Child. Child, Preschool. DNA-Binding Proteins / metabolism. Humans. Magnetic Resonance Imaging. Male. Nerve Tissue Proteins / metabolism. Protein-Serine-Threonine Kinases / metabolism. Retrospective Studies. Tumor Suppressor Proteins / metabolism. alpha-Fetoproteins / metabolism

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  • (PMID = 19650357.001).
  • [ISSN] 0317-1671
  • [Journal-full-title] The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
  • [ISO-abbreviation] Can J Neurol Sci
  • [Language] eng
  • [Grant] United States / NIAID NIH HHS / AI / AI 067769; United States / NINDS NIH HHS / NS / NS 052528
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Cell Cycle Proteins; 0 / DNA-Binding Proteins; 0 / Nerve Tissue Proteins; 0 / Tumor Suppressor Proteins; 0 / alpha-Fetoproteins; EC 2.7.11.1 / ATM protein, human; EC 2.7.11.1 / Ataxia Telangiectasia Mutated Proteins; EC 2.7.11.1 / Protein-Serine-Threonine Kinases
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4. 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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5. 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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  • [Title] Chemosensitivity in childhood brain tumours in vitro: evidence of differential sensitivity to lomustine (CCNU) and vincristine.
  • 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.
  • [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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6. 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.
  • Inhibition of telomerase function represents a novel experimental therapeutic strategy in childhood PNETs that warrants further investigation.
  • [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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7. Keene DL, Johnston DL, Grimard L, Michaud J, Vassilyadi M, Ventureyra E: Vascular complications of cranial radiation. Childs Nerv Syst; 2006 Jun;22(6):547-55
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  • One hundred and 13 cases involved tumors of the central nervous system.
  • Post radiation cerebral vascular disease occurred in 11 (5%) patients, and all but one patient had a tumor involving the central nervous system (mainly in the posterior fossa and supratentorial midline).
  • CONCLUSION: There is an increased risk of cerebral vascular disease after radiation therapy in childhood, especially in children who received high dose radiation at the posterior fossa and supratentorial axial region.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Cranial Irradiation / adverse effects. Drug-Related Side Effects and Adverse Reactions. Vascular Diseases / etiology






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