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1. Wolff JE, Gnekow AK, Kortmann RD, Pietsch T, Urban C, Graf N, Kühl J: Preradiation chemotherapy for pediatric patients with high-grade glioma. Cancer; 2002 Jan 1;94(1):264-71
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  • [Title] Preradiation chemotherapy for pediatric patients with high-grade glioma.
  • BACKGROUND: To evaluate the feasibility and efficacy of intensive chemotherapy given prior to irradiation in pediatric patients with malignant glioma, the Society of Pediatric Oncology in Germany started a randomized trial in 1991.
  • The tumor locations were supratentorial in 42 patients, the cerebellum in 8 patients, and the spinal cord in 2 patients (the brainstem was excluded).
  • Tumor surgeries were biopsy in 10 patients, partial resection in 5 patients, subtotal resection in 10 patients, and macroscopic total resection in 21 patients.
  • Patients received either 54 grays of irradiation (n = 22 patients) followed by chemotherapy with lomustine, vincristine, and cisplatin (maintenance chemotherapy) or sandwich chemotherapy (n = 30 patients), which consisted of ifosfamide, etoposide, methotrexate, cisplatin, and cytosine arabinoside followed by irradiation.
  • The median survival was 5.2 years for patients who underwent tumor resection of > or = 90% compared with 1.3 years for patients who underwent less than complete resection (P < 0.0005).
  • After undergoing macroscopic total resection, sandwich chemotherapy (n = 15 patients) resulted in better overall survival (median, 5.2 years) compared with the maintenance protocol (n = 16 patients; median survival, 1.9 years; P = 0.015).
  • A Cox multivariate regression analysis showed better survival for female patients (P = 0.025), WHO Grade 3 disease (P = 0.016), tumor resection of > or = 90% (P = 0.003), irradiation with > or = 54 grays (P = 0.003), and sandwich chemotherapy (P = 0.006).
  • CONCLUSIONS: These data suggest that early, intensive chemotherapy increases survival rates in patients with malignant glioma who undergo complete resection.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Central Nervous System Neoplasms / drug therapy. Central Nervous System Neoplasms / radiotherapy. Glioma / drug therapy. Glioma / radiotherapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Male. Proportional Hazards Models. Retrospective Studies. Survival Analysis

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  • [Copyright] Copyright 2002 American Cancer Society.
  • (PMID = 11815986.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase III; Journal Article; Randomized Controlled Trial
  • [Publication-country] United States
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2. Otero-Rodríguez A, Hinojosa J, Esparza J, Muñoz MJ, Iglesias S, Rodríguez-Gil Y, Ricoy JR: Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature. Neurocirugia (Astur); 2009 Aug;20(4):381-6; discussion 386-7
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  • [Title] Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature.
  • INTRODUCTION: Primitive neuroectodermal tumors (PNETs) are malign neoplasms of the central nervous system which mainly locate in cerebellum (medulloblastoma).
  • In this report, we describe a new IPNET case and review the literature about these infrequent intramedullary tumors.
  • Subsequent chemotherapy was recommended.
  • CONCLUSION: IPNETs are uncommon tumors affecting children and young adults.
  • Outcome is dismal: most patients die within two years in spite of surgical resection followed by radiotherapy and chemotherapy.
  • [MeSH-major] Neuroectodermal Tumors, Primitive / pathology. Spinal Cord Neoplasms / pathology. Thoracic Vertebrae
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carboplatin / administration & dosage. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Disease Progression. Etoposide / administration & dosage. Humans. Infant. Magnetic Resonance Imaging. Male. Methotrexate / administration & dosage. Paraparesis / etiology. Prognosis

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  • (PMID = 19688140.001).
  • [ISSN] 1130-1473
  • [Journal-full-title] Neurocirugía (Asturias, Spain)
  • [ISO-abbreviation] Neurocirugia (Astur)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Spain
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin; Q20Q21Q62J / Cisplatin; YL5FZ2Y5U1 / Methotrexate
  • [Number-of-references] 19
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3. Merchant TE, Kun LE, Wu S, Xiong X, Sanford RA, Boop FA: Phase II trial of conformal radiation therapy for pediatric low-grade glioma. J Clin Oncol; 2009 Aug 1;27(22):3598-604
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  • [Title] Phase II trial of conformal radiation therapy for pediatric low-grade glioma.
  • PURPOSE: The use of radiotherapy in pediatric low-grade glioma (LGG) is controversial, especially for young patients.
  • We conducted a phase II trial of conformal radiation therapy (CRT) to estimate disease control by using a 10-mm clinical target volume (CTV) margin.
  • MATERIALS AND METHODS: Between August 1997 and August 2006, 78 pediatric patients with LGG and a median age of 8.9 years (range, 2.2 to 19.8 years) received 54 Gy CRT by using a 10-mm CTV and by targeting with systematic magnetic resonance imaging (MRI) registration.
  • Tumor locations were diencephalon (n = 58), cerebral hemisphere (n = 3), and cerebellum (n = 17).
  • Sixty-seven patients had documented or presumed WHO grade 1 tumors, 25 patients had prior chemotherapy, and 13 patients had neurofibromatosis type 1.
  • One patient experienced marginal treatment failure, eight experienced local failures, and four experienced metastatic failure.
  • The mean and standard error cumulative incidence of vasculopathy was 4.79% +/- 2.73% at 6 years, and it was higher for those younger than 5 years of age (P = .0105) at the time of CRT.
  • [MeSH-major] Brain Neoplasms / mortality. Brain Neoplasms / radiotherapy. Glioma / mortality. Glioma / radiotherapy. Neoplasm Invasiveness / pathology. Radiotherapy, Conformal / methods
  • [MeSH-minor] Adolescent. Age Factors. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biopsy, Needle. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Dose-Response Relationship, Radiation. Female. Humans. Immunohistochemistry. Kaplan-Meier Estimate. Magnetic Resonance Angiography. Male. Neoplasm Staging. Prognosis. Prospective Studies. Radiotherapy Dosage. Risk Assessment. Statistics, Nonparametric. Survival Analysis. Treatment Outcome. Young Adult

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  • (PMID = 19581536.001).
  • [ISSN] 1527-7755
  • [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, Phase II; Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3525947
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4. Hukin J, Siffert J, Cohen H, Velasquez L, Zagzag D, Allen J: Leptomeningeal dissemination at diagnosis of pediatric low-grade neuroepithelial tumors. Neuro Oncol; 2003 07;5(3):188-96
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  • [Title] Leptomeningeal dissemination at diagnosis of pediatric low-grade neuroepithelial tumors.
  • The goal of this study was to describe the demographic, histologic, and prognostic features of children with low-grade neuroepithelial tumors (LGN) of the CNS presenting with leptomeningeal metastases (LM) at diagnosis.
  • The charts were reviewed and patients contacted to validate the demographic data, treatment, and clinical status.
  • The distribution of LM patients by primary tumor site was diencephalon, 5; cerebrum, 2; spinal cord, 3; brainstem, 2; and cerebellum, 1.
  • Six of 8 patients with LM had durable objective responses to chemotherapy.
  • We suggest that staging be considered in the following circumstances: diencephalic primary site, unexplained hydrocephalus, clinical features suggestive of LM, and before adjuvant therapy is initiated.
  • The prognosis for children with LM at diagnosis is favorable, and its identification alters therapeutic strategies.
  • [MeSH-major] Central Nervous System Neoplasms / diagnosis. Meningeal Neoplasms / diagnosis. Neoplasms, Neuroepithelial / diagnosis

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  • (PMID = 12816725.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1920691
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5. Rodini CO, Suzuki DE, Nakahata AM, Pereira MC, Janjoppi L, Toledo SR, Okamoto OK: Aberrant signaling pathways in medulloblastomas: a stem cell connection. Arq Neuropsiquiatr; 2010 Dec;68(6):947-52

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Medulloblastoma is a highly malignant primary tumor of the central nervous system.
  • It represents the most frequent type of solid tumor and the leading cause of death related to cancer in early childhood.
  • Current treatment includes surgery, chemotherapy and radiotherapy which may lead to severe cognitive impairment and secondary brain tumors.
  • New perspectives for therapeutic development have emerged with the identification of stem-like cells displaying high tumorigenic potential and increased radio- and chemo-resistance in gliomas.
  • Under the cancer stem cell hypothesis, transformation of neural stem cells and/or granular neuron progenitors of the cerebellum are though to be involved in medulloblastoma development.
  • From the clinical viewpoint, modulation of signaling pathways such as TGFβ, regulating neural stem cell proliferation and tumor development, might be attempted as an alternative strategy for future drug development aiming at more efficient therapies and improved clinical outcome of patients with pediatric brain cancers.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Medulloblastoma / pathology. Neoplastic Stem Cells / pathology. Neural Stem Cells / pathology. Signal Transduction. Transforming Growth Factor beta

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  • (PMID = 21243257.001).
  • [ISSN] 1678-4227
  • [Journal-full-title] Arquivos de neuro-psiquiatria
  • [ISO-abbreviation] Arq Neuropsiquiatr
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Brazil
  • [Chemical-registry-number] 0 / Transforming Growth Factor beta
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6. Vibhakar R, Foltz G, Yoon JG, Field L, Lee H, Ryu GY, Pierson J, Davidson B, Madan A: Dickkopf-1 is an epigenetically silenced candidate tumor suppressor gene in medulloblastoma. Neuro Oncol; 2007 Apr;9(2):135-44
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  • [Title] Dickkopf-1 is an epigenetically silenced candidate tumor suppressor gene in medulloblastoma.
  • Medulloblastoma is a heterogeneous pediatric brain tumor with significant therapy-related morbidity, its five-year survival rates ranging from 30% to 70%.
  • Improvement in diagnosis and therapy requires better understanding of medulloblastoma pathology.
  • We used whole-genome microarray analysis to identify putative tumor suppressor genes silenced by epigenetic mechanisms in medulloblastoma.
  • This analysis yielded 714 up-regulated genes in immortalized medulloblastoma cell line D283 on treatment with histone deacetylase (HDAC) inhibitor trichostatin A (TSA).
  • We examined DKK1 expression in primary medulloblastoma cells and patient samples by reverse transcriptase PCR and found it to be significantly down-regulated relative to normal cerebellum.
  • Transfection of a DKK1 gene construct into D283 cell lines suppressed medulloblastoma tumor growth in colony focus assays by 60% (P < 0.001).
  • These data reveal that inappropriate histone modifications might deregulate DKK1 expression in medulloblastoma tumorigenesis and block its tumor-suppressive activity.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Gene Expression Regulation, Neoplastic / drug effects. Genes, Tumor Suppressor. Intercellular Signaling Peptides and Proteins / genetics. Medulloblastoma / genetics
  • [MeSH-minor] Cell Division / drug effects. Chromatin / genetics. Colony-Forming Units Assay. Enzyme Inhibitors / pharmacology. Gene Silencing. Histone Deacetylase Inhibitors. Humans. Hydroxamic Acids / pharmacology. Oligonucleotide Array Sequence Analysis. Reverse Transcriptase Polymerase Chain Reaction. Survival Analysis. Tumor Cells, Cultured

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  • (PMID = 17329407.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chromatin; 0 / DKK1 protein, human; 0 / Enzyme Inhibitors; 0 / Histone Deacetylase Inhibitors; 0 / Hydroxamic Acids; 0 / Intercellular Signaling Peptides and Proteins; 3X2S926L3Z / trichostatin A
  • [Other-IDs] NLM/ PMC1871668
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7. Samano AK, Ohshima-Hosoyama S, Whitney TG, Prajapati SI, Kilcoyne A, Taniguchi E, Morgan WW, Nelon LD, Lin AL, Togao O, Jung I, Rubin BP, Nowak BM, Duong TQ, Keller C: Functional evaluation of therapeutic response for a mouse model of medulloblastoma. Transgenic Res; 2010 Oct;19(5):829-40
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  • [Title] Functional evaluation of therapeutic response for a mouse model of medulloblastoma.
  • Medulloblastoma is an aggressive childhood cerebellar tumor.
  • Magnetic resonance imaging (MRI) was used to confirm and monitor tumor growth and as an anatomical biomarker for therapeutic response.
  • Wild type mice or medulloblastoma-prone, conditional Patched1 knockout mice were observed by behavioral assays and MRI from postnatal weeks 3-6.
  • Bortezomib treatment was administered during this period and therapeutic response was assessed using cerebellar volumes at the end of treatment.
  • Of the behavioral tests assessed in this study, stride length analysis was best able to detect differences between tumor-prone mice and wild type mice as early as postnatal day 37 (P=0.003).
  • Significant differences between stride lengths of bortezomib treated and control tumor-bearing mice could be detected as early as postnatal day 42 (P=0.020).
  • Cerebellar volumes measured by MRI at the end of treatment validated the therapeutic effects seen by behavioral tests (P=0.03).
  • These findings suggest that stride length analysis may serve as one of the more sensitive and cost-effective method for assessing new therapeutic compounds in this and other preclinical model of brain tumors.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Ataxia / etiology. Boronic Acids / therapeutic use. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy. Protease Inhibitors / therapeutic use. Psychomotor Performance. Pyrazines / therapeutic use. Receptors, Cell Surface / deficiency
  • [MeSH-minor] Animals. Bortezomib. Disease Models, Animal. Disease Progression. Drug Screening Assays, Antitumor / economics. Drug Screening Assays, Antitumor / methods. Gait Disorders, Neurologic / etiology. Lameness, Animal / etiology. Magnetic Resonance Imaging. Mice. Mice, Knockout

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  • (PMID = 20107895.001).
  • [ISSN] 1573-9368
  • [Journal-full-title] Transgenic research
  • [ISO-abbreviation] Transgenic Res.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / R01 NS045879
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Boronic Acids; 0 / Protease Inhibitors; 0 / Pyrazines; 0 / Receptors, Cell Surface; 0 / patched receptors; 69G8BD63PP / Bortezomib
  • [Other-IDs] NLM/ NIHMS759162; NLM/ PMC4770905
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8. Lee MJ, Ra YS, Park JB, Goo HW, Ahn SD, Khang SK, Song JS, Kim YJ, Ghim TT: Effectiveness of novel combination chemotherapy, consisting of 5-fluorouracil, vincristine, cyclophosphamide and etoposide, in the treatment of low-grade gliomas in children. J Neurooncol; 2006 Dec;80(3):277-84
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  • [Title] Effectiveness of novel combination chemotherapy, consisting of 5-fluorouracil, vincristine, cyclophosphamide and etoposide, in the treatment of low-grade gliomas in children.
  • Low-grade gliomas (LGG), which account for about 30% of brain tumors in children, are usually treated with surgical excision and/or radiotherapy.
  • For patients who have significant residual tumor after resection or relapse after radiation, the proper chemotherapy regimen has not yet been identified.
  • Thirteen children diagnosed with LGG outside the cerebellum between January 1999 and December 2004, all of whom had significant residual tumor after surgical resection, relapsed after radiation or showed visual deterioration, were treated for 18 months with a multi-drug regimen of vincristine, etoposide, cyclophosphamide and 5-fluorouracil.
  • Of the 7 patients who completed chemotherapy, 1 showed complete response (CR), 5 showed partial response (PR), and 1 had stable disease (SD).
  • In 5 patients, chemotherapy was prematurely discontinued; 4 of these patients showed tumor progression and 1 had SD.
  • One patient is still undergoing treatment.
  • The side effects of chemotherapy were manageable.
  • The median time to tumor response was 34 months (range, 2-82 months).
  • Pediatric LGG patients with residual tumor after surgery or who undergo relapse(s) may be successfully treated using our combination chemotherapy regimen.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Glioma / drug therapy. Neoplasm Recurrence, Local / drug therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Cyclophosphamide / administration & dosage. Disease-Free Survival. Etoposide / administration & dosage. Female. Fluorouracil / administration & dosage. Humans. Infant. Male. Neoplasm, Residual / drug therapy. Prospective Studies. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 16807782.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; U3P01618RT / Fluorouracil
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9. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • All of the patients died within a mean of 14 months due to tumor progression despite the chemotherapy.
  • In conclusion, AT/RTs are aggressive tumors.
  • [MeSH-major] Brain Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology

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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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10. Akay KM, Izci Y, Baysefer A, Atabey C, Kismet E, Timurkaynak E: Surgical outcomes of cerebellar tumors in children. Pediatr Neurosurg; 2004 Sep-Oct;40(5):220-5

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical outcomes of cerebellar tumors in children.
  • Cerebellar tumors in childhood are generally associated with a favorable outcome if they are managed appropriately.
  • 27 cases of pediatric cerebellar tumors, operated over a 7-year period, are presented.
  • The total removal of pediatric cerebellar tumors without neurological deficit is possible with appropriate microsurgical techniques excluding brain stem invasion.
  • Radiotherapy and chemotherapy are the adjuvant therapies according to the pathological diagnosis and the patient's age.
  • [MeSH-major] Astrocytoma / surgery. Cerebellar Neoplasms / surgery. Ependymoma / surgery. Medulloblastoma / surgery
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Humans. Male. Neoplasm Invasiveness. Prognosis. Radiotherapy, Adjuvant. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • [Copyright] Copyright (c) 2004 S. Karger AG, Basel.
  • (PMID = 15687736.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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11. Schoch B, Konczak J, Dimitrova A, Gizewski ER, Wieland R, Timmann D: Impact of surgery and adjuvant therapy on balance function in children and adolescents with cerebellar tumors. Neuropediatrics; 2006 Dec;37(6):350-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Impact of surgery and adjuvant therapy on balance function in children and adolescents with cerebellar tumors.
  • OBJECTIVES: This study examined the effects of posterior fossa tumor surgery and concomitant irradiation and/or chemotherapy on the long-term recovery of balance function in children and adolescent patients.
  • SUBJECTS AND METHODS: 22 patients, treated during childhood for a benign (n = 14) or malignant cerebellar tumor (n = 8), were examined in chronic state (mean latency between surgery and testing: 7.7 years, range 3 - 17 years).
  • All cerebellar lesions were documented by standardized and normalized MRI data.
  • RESULTS: Comparing the balance function of (i) children with or without affected cerebellar nuclei and (ii) children with and without adjuvant chemotherapy and/or radiotherapy revealed that damage to the cerebellar nuclei had more impact on neurological impairment than concomitant tumor therapy.
  • Chemotherapy with its neurological side effect was associated with enhanced postural sway in only two children with malignant tumors.
  • CONCLUSIONS: The study results indicate that the sparing of the deep cerebellar nuclei had the greatest impact on the recovery of balance function in pediatric patients treated for both a benign or malignant cerebellar tumor.
  • [MeSH-major] Cerebellar Neoplasms / surgery. Postural Balance
  • [MeSH-minor] Adolescent. Cerebellar Nuclei / drug effects. Cerebellar Nuclei / pathology. Cerebellar Nuclei / radiation effects. Cerebellar Nuclei / surgery. Cerebellum / drug effects. Cerebellum / radiation effects. Cerebellum / surgery. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Cranial Irradiation. Female. Follow-Up Studies. Humans. Image Processing, Computer-Assisted. Infant. Magnetic Resonance Imaging. Male. Neurologic Examination / drug effects. Postoperative Complications / diagnosis. Postoperative Complications / pathology. Radiotherapy, Adjuvant

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  • (PMID = 17357037.001).
  • [ISSN] 0174-304X
  • [Journal-full-title] Neuropediatrics
  • [ISO-abbreviation] Neuropediatrics
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
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12. Wolff JE, Wagner S, Reinert C, Gnekow A, Kortmann RD, Kühl J, Van Gool SW: Maintenance treatment with interferon-gamma and low-dose cyclophosphamide for pediatric high-grade glioma. J Neurooncol; 2006 Sep;79(3):315-21
Hazardous Substances Data Bank. CYCLOPHOSPHAMIDE .

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  • [Title] Maintenance treatment with interferon-gamma and low-dose cyclophosphamide for pediatric high-grade glioma.
  • METHODS: After induction treatment with simultaneous radiation and chemotherapy, patients were treated with individually increasing interferon-gamma (IFN-gamma) doses starting from 25 microg/m2/d s.c. increasing up to a maximum of 175 microg/m2/d within 7 weeks.
  • Forty pediatric glioma patients were enrolled (median age: 8.5 year, male: n = 22).
  • Tumor locations included cerebral cortex (n = 8), basal ganglia (n = 4), brainstem (n = 24), cerebellum (n = 3), spinal cord (n = 1).
  • The observation time of the six surviving patients was: 1.2, 1.9, 4.2, 4.4, 4.6 and 4.7 years respectively.
  • CONCLUSION: Maintenance treatment with IFN-gamma and low dose CPM has no sufficient beneficial effect for the treatment of high-grade glioma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Cyclophosphamide / administration & dosage. Glioma / drug therapy. Interferon-gamma / administration & dosage
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Male. Survival Analysis. Treatment Outcome

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  • (PMID = 16645718.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 82115-62-6 / Interferon-gamma; 8N3DW7272P / Cyclophosphamide
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13. Benesch M, Wagner S, Berthold F, Wolff JE: Primary dissemination of high-grade gliomas in children: experiences from four studies of the Pediatric Oncology and Hematology Society of the German Language Group (GPOH). J Neurooncol; 2005 Apr;72(2):179-83
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  • [Title] Primary dissemination of high-grade gliomas in children: experiences from four studies of the Pediatric Oncology and Hematology Society of the German Language Group (GPOH).
  • PATIENTS AND METHODS: We conducted a retrospective data analysis of all patients enrolled into four consecutive HGG protocols of the Pediatric Oncology and Hematology Society of the German Language Group (GPOH) to determine the incidence of primary CNS dissemination of HGG and to describe clinical characteristics and outcome of children with HGG who were diagnosed with CNS dissemination at initial presentation.
  • Data concerning tumor dissemination are available from 324 patients.
  • RESULTS: A total of 10 patients (3.1%) (anaplastic astrocytoma: n=3, glioblastoma multiforme: n=6, diffuse intrinsic pontine glioma: n=1) had primary tumor dissemination.
  • The most frequent primary tumor sites were the cortex (n=4), followed by the ventricles (n=2), cerebellum (n=1), spinal cord (n=1), and pons (n=1).
  • Following surgery eight patients received local radiotherapy and eight additional chemotherapy.
  • Median progression-free and overall survival was 0.8 years (95% CI 0.2-1.4) and 1.5 years (95% CI 0.67-2.29) for patients with primary tumor dissemination, respectively, with no statistically significant differences between the group with and the group without primary tumor dissemination.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Central Nervous System Neoplasms / pathology. Central Nervous System Neoplasms / therapy. Glioma / pathology. Glioma / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Cyclophosphamide / analogs & derivatives. Disease-Free Survival. Etoposide / administration & dosage. Female. Humans. Male. Neoplasm Invasiveness. Radiotherapy. Retrospective Studies. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 15925999.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; H64JRU6GJ0 / trofosfamide
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14. Wolff JE, Wagner S, Sindichakis M, Pietsch T, Gnekow A, Kortmann RD, Sträter R, Kuehl J: Simultaneous radiochemotherapy in pediatric patients with high-grade glioma: a phase I study. Anticancer Res; 2002 Nov-Dec;22(6B):3569-72
Hazardous Substances Data Bank. IFOSFAMIDE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Simultaneous radiochemotherapy in pediatric patients with high-grade glioma: a phase I study.
  • BACKGROUND: Sandwich chemotherapy as first postoperative treatment might cause resistance to ensuing irradiation.
  • Tumor locations included: cerebral cortex 12, deep cerebral locations 11, cerebellum 3, spinal cord 3.
  • Total (n = 9), subtotal (n = 3) and partial (n = 9) tumor resection or biopsy (n = 7) were followed by radiochemotherapy: fractionated radiation (1.8 Gyld up to 59.4 Gy) was given simultaneously with two cycles of chemotherapy (cisplatin 20 mg/m2/d x 5d, etoposide 100 mg/m2/d x 3d, and cisplatin, etoposide, ifosfamide: 1.5 g/m2/d).
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / radiotherapy. Glioma / drug therapy. Glioma / radiotherapy. Spinal Cord Neoplasms / drug therapy. Spinal Cord Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Astrocytoma / drug therapy. Astrocytoma / radiotherapy. Astrocytoma / surgery. Child. Child, Preschool. Cisplatin / administration & dosage. Cisplatin / adverse effects. Cohort Studies. Combined Modality Therapy / adverse effects. Dose Fractionation. Etoposide / administration & dosage. Etoposide / adverse effects. Female. Glioblastoma / drug therapy. Glioblastoma / radiotherapy. Glioblastoma / surgery. Humans. Ifosfamide / administration & dosage. Ifosfamide / adverse effects. Male. Prospective Studies

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  • (PMID = 12552957.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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15. Koeller KK, Rushing EJ: From the archives of the AFIP: pilocytic astrocytoma: radiologic-pathologic correlation. Radiographics; 2004 Nov-Dec;24(6):1693-708
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  • Pilocytic astrocytoma is the most common pediatric central nervous system glial neoplasm and the most common pediatric cerebellar tumor.
  • This tumor has a noteworthy benign biologic behavior that translates into an extremely high survival rate-94% at 10 years-that is by far the best of any glial tumor.
  • Most patients present in the first 2 decades, and clinical symptoms and signs are usually of several months duration and directly related to the specific location of the tumor.
  • The cerebellum, optic nerve and chiasm, and hypothalamic region are the most common locations, but the tumor can also be found in the cerebral hemisphere, ventricles, and spinal cord.
  • Surgical resection is the treatment of choice for all tumors, except for those involving the optic pathway and hypothalamic region, which may be treated with radiation therapy and chemotherapy.
  • Accurate interpretation of imaging studies plays an essential role in directing treatment of these tumors, particularly when they arise in the optic pathway of patients with neurofibromatosis type 1.
  • [MeSH-major] Astrocytoma / pathology. Astrocytoma / radiography. Tomography, X-Ray Computed

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  • (PMID = 15537977.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 98
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16. Viano JC, Herrera EJ, Suárez JC: Cerebellar astrocytomas: a 24-year experience. Childs Nerv Syst; 2001 Oct;17(10):607-10; discussion 611

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cerebellar astrocytomas: a 24-year experience.
  • INTRODUCTION: Cerebellar astrocytomas are the most benign tumors of the CNS.
  • METHODS AND RESULTS: We report on 38 children under 18 who had cerebellar astrocytoma in the posterior fossa and were treated by a multidisciplinary team in our Neurosurgical Department from January 1974 to December 1997.
  • Neither radiotherapy nor chemotherapy was indicated in patients with pilocytic or fibrillary astrocytomas.
  • These tumors were more frequently located in the right cerebellar hemisphere; increased intracranial pressure syndrome was the most frequent form of clinical presentation.
  • Total tumor resection was obtained in 29 (83%) cases and subtotal resection in 9 (17%).
  • In 6 (16%) cases, ventriculoperitoneal shunts were placed to control persistent hydrocephalus after tumor excision.
  • [MeSH-major] Astrocytoma. Cerebellar Neoplasms. Cerebellum / pathology. Neurosurgical Procedures
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Male. Prevalence. Retrospective Studies. Sex Distribution. Survival Analysis. Treatment Outcome

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  • (PMID = 11685523.001).
  • [ISSN] 0256-7040
  • [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] Journal Article
  • [Publication-country] Germany
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17. Rønning C, Sundet K, Due-Tønnessen B, Lundar T, Helseth E: Persistent cognitive dysfunction secondary to cerebellar injury in patients treated for posterior fossa tumors in childhood. Pediatr Neurosurg; 2005 Jan-Feb;41(1):15-21
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  • [Title] Persistent cognitive dysfunction secondary to cerebellar injury in patients treated for posterior fossa tumors in childhood.
  • Traditionally, the cerebral hemispheres have been regarded as the region of the brain responsible for cognitive functions, while the cerebellum has been considered to be primarily involved in motor functions.
  • Recent studies focus also on the possible involvement of the cerebellum in neurocognitive functions.
  • The aim of this study was to determine the neuropsychological profile of young adults treated for a posterior fossa tumor in childhood and look for possible support for the presence of the so-called 'cerebellar cognitive affective syndrome' in these patients.
  • The astrocytoma group (n = 12) had been treated for a low-grade cerebellar astrocytoma with surgery alone (mean age at surgery was 8.6 years and mean age at neuropsychological testing was 23.5 years).
  • The medulloblastoma group (n = 11) had been treated with surgery followed by radiotherapy and chemotherapy (mean age at surgery was 6.1 years and mean age at neuropsychological testing was 23.1 years).
  • No significant correlation between age at time of treatment and grade of neuropsychological impairment was found in the astrocytoma group, though there was a tendency that young age at time of treatment correlated with better outcome on IQ measures.
  • For this group, young age at time of treatment indicated a worse outcome.
  • CONCLUSIONS: Persistent cognitive dysfunction was detected in patients treated for posterior fossa medulloblastoma and cerebellar astrocytoma.
  • The astrocytoma group was treated with surgery alone, indicating that a cerebellar lesion can result in cognitive dysfunction.
  • Thus, this study gives support to the existence of the cerebellar cognitive affective syndrome.
  • Follow-up of all patients treated for posterior fossa tumor in childhood should include an extensive neuropsychological testing at regular intervals.
  • [MeSH-major] Astrocytoma / surgery. Cerebellar Neoplasms / surgery. Cerebellum / physiopathology. Cerebellum / surgery. Cognition Disorders / etiology. Medulloblastoma / surgery

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  • [Copyright] Copyright 2005 S. Karger AG, Basel.
  • (PMID = 15886508.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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18. Bowers DC, Krause TP, Aronson LJ, Barzi A, Burger PC, Carson BS, Weingart JD, Wharam MD, Melhem ER, Cohen KJ: Second surgery for recurrent pilocytic astrocytoma in children. Pediatr Neurosurg; 2001 May;34(5):229-34
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  • Pilocytic astrocytoma (PA) is the most common childhood brain tumor.
  • In cases where the tumor progresses or recurs following primary surgical resection, the appropriate treatment is unclear.
  • Options include chemotherapy, radiation therapy, surgical resection or a combination thereof.
  • To analyze the utility of further surgery, we performed a retrospective, single-institution review of pediatric patients with recurrent PAs from 1990 to 1999 who were treated with a second surgical resection.
  • Patients were excluded if they received adjuvant chemotherapy or radiation therapy.
  • Tumor locations included: cerebral hemisphere (3), cerebellum (7), optic pathway/hypothalamus (5), thalamus (1) and brainstem (4).
  • The indication for 4 surgeries included an enlarging tumor-associated cyst.
  • Two of 10 tumors after GTR, 0 of 2 tumors after NTR, and 7 of 8 tumors after STR had second recurrence/progression at a mean of 15 months (range 4-33 months) following second surgery.
  • Surgery for tumors or midline structures rarely resulted in a GTR (1 of 10 cases).
  • Surgery for tumors located in the cerebral hemispheres or cerebellum resulted in GTR or NTR in all cases and can result in long periods of progression-free survival without further adjuvant treatment.
  • [MeSH-major] Astrocytoma / surgery. Brain / surgery. Brain Neoplasms / surgery. Neoplasm Recurrence, Local / surgery
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Humans. Infant. Reoperation / adverse effects. Retrospective Studies. Treatment Outcome


19. Koeller KK, Rushing EJ: From the archives of the AFIP: medulloblastoma: a comprehensive review with radiologic-pathologic correlation. Radiographics; 2003 Nov-Dec;23(6):1613-37
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  • Medulloblastoma is the most common pediatric central nervous system malignancy and the most common primary tumor of the posterior fossa in children.
  • This highly malignant neoplasm occurs more frequently in males and usually before 10 years of age.
  • Clinical symptoms and signs are generally brief, typically less than 3 months in duration, and reflect the strong predilection of this tumor to arise within the cerebellum, most often in the vermis.
  • Surgical resection, radiation therapy, and chemotherapy have substantially lowered the mortality associated with this tumor, with 5-year survival rates now commonly well above 50%.
  • Still, both dissemination at the time of diagnosis and recurrence remain obstacles in achieving a cure.
  • The tumor has characteristic hyperattenuation on unenhanced computed tomographic scans that reflects the high nuclear-cytoplasmic ratio seen at histologic analysis.
  • The tumor typically appears heterogeneous on images, findings that are related to cyst formation, hemorrhage, and calcification and that are even more pronounced with magnetic resonance (MR) imaging.
  • Evidence of leptomeningeal metastatic spread is present in 33% of all cases at the time of diagnosis and is well evaluated with contrast-enhanced MR imaging of the brain and the spine.
  • With continued research, treatment of these common neoplasms should improve, perhaps even achieving a cure in the future.
  • [MeSH-major] Cerebellar Neoplasms / radiography. Magnetic Resonance Imaging. Medulloblastoma / radiography. Tomography, X-Ray Computed
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Female. Humans. Infant. Male. Meninges / pathology. Middle Aged. Neoplasm Invasiveness. Prognosis. Survival Rate

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  • (PMID = 14615567.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 102
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20. Shinwari Z, Manogaran PS, Alrokayan SA, Al-Hussein KA, Aboussekhra A: Vincristine and lomustine induce apoptosis and p21(WAF1) up-regulation in medulloblastoma and normal human epithelial and fibroblast cells. J Neurooncol; 2008 Apr;87(2):123-32
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  • Medulloblastomas arise in the cerebellum and are the most common pediatric primary malignant brain tumors.
  • Currently, medulloblastoma patients are best treated with surgical removal of the tumor, adjuvant radiation therapy and chemotherapy.
  • However, the effects of these drugs on medulloblastomas as well as on other cell types is still not well defined.
  • In the present report we present evidence that the cytotoxic effect of these drugs is not specific for medulloblastoma cells but includes also normal fibroblast and epithelial cells.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Apoptosis / drug effects. Cerebellar Neoplasms / drug therapy. Cyclin-Dependent Kinase Inhibitor p21 / drug effects. Lomustine / pharmacology. Medulloblastoma / drug therapy. Vincristine / pharmacology
  • [MeSH-minor] Cell Cycle / drug effects. Cell Line. Epithelial Cells / drug effects. Fibroblasts / drug effects. Flow Cytometry. Humans. Immunoblotting. Proto-Oncogene Proteins c-bcl-2 / drug effects. Up-Regulation. bcl-X Protein / drug effects

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  • (PMID = 18058069.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / BCL2L1 protein, human; 0 / CDKN1A protein, human; 0 / Cyclin-Dependent Kinase Inhibitor p21; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / bcl-X Protein; 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine
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21. Gulino A, Arcella A, Giangaspero F: Pathological and molecular heterogeneity of medulloblastoma. Curr Opin Oncol; 2008 Nov;20(6):668-75
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  • PURPOSE OF REVIEW: The outcome of medulloblastoma patients and the quality of life of survivors can be improved by both novel therapies and a better tumor classification.
  • This review will focus on the pathology and molecular biology of medulloblastoma and its potential to influence risk assignment and future therapy.
  • RECENT FINDINGS: A risk stratification system of pediatric medulloblastoma based on a combination of histopathological evaluation and targeted molecular analysis can be outlined.
  • Moreover, 17p loss, MYC amplification/expression, and 1q gain are associated with poor prognosis; in contrast, monosomy 6, mutation of CTNNB1, and trkC expression identify tumors with a favorable outcome.
  • Emerging evidence indicates that the different precursor cell populations that form the cerebellum are susceptible to mutations in signal pathways that regulate their functions; these mutations alter normal development programmes and may result in the formation of distinct variants of medulloblastoma.
  • SUMMARY: Better understanding of the growth control mechanisms involved in the development and progression of medulloblastoma will allow improved therapeutic stratification of patients using existing adjuvant therapy as well as the development of new therapeutic approaches.
  • [MeSH-major] Brain Neoplasms / drug therapy. Gene Expression Regulation, Neoplastic. Medulloblastoma / drug therapy
  • [MeSH-minor] Adolescent. Cerebellum / pathology. Child. Child, Preschool. Hedgehog Proteins / metabolism. Humans. Infant. Infant, Newborn. Quality of Life. Receptors, Notch / metabolism. Risk. Treatment Outcome. Wnt Proteins / metabolism

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  • (PMID = 18841049.001).
  • [ISSN] 1531-703X
  • [Journal-full-title] Current opinion in oncology
  • [ISO-abbreviation] Curr Opin Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Hedgehog Proteins; 0 / Receptors, Notch; 0 / Wnt Proteins
  • [Number-of-references] 31
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22. Shuper A, Stein J, Goshen J, Kornreich L, Yaniv I, Cohen IJ: Subacute central nervous system degeneration in a child: an unusual manifestation of ifosfamide intoxication. J Child Neurol; 2000 Jul;15(7):481-3
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  • A 5-year-old child with desmoplastic small round-cell tumor was treated with a protocol of very-high-dose, short-term chemotherapy, containing HD-CAV (cyclophosphamide, doxorubicin, vincristine, and mesna), ifosfamide, and etoposide.
  • Two days after the initiation of ifosfamide, he exhibited new-onset lethal encephalopathy manifested by subacutely progressive cerebellar and then temporal and frontocortical degeneration leading to a vegetative state and eventually to death.
  • The possibility of this complication should be considered when high-dose ifosfamide treatment is planned for children.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Cerebellum / drug effects. Cerebral Cortex / drug effects. Ifosfamide / adverse effects. Nerve Degeneration / chemically induced. Persistent Vegetative State / chemically induced
  • [MeSH-minor] Child, Preschool. Dose-Response Relationship, Drug. Fatal Outcome. Humans. Male

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  • (PMID = 10921521.001).
  • [ISSN] 0883-0738
  • [Journal-full-title] Journal of child neurology
  • [ISO-abbreviation] J. Child Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] UM20QQM95Y / Ifosfamide
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23. Gilmer-Hill HS, Ellis WG, Imbesi SG, Boggan JE: Spinal oligodendroglioma with gliomatosis in a child. Case report. J Neurosurg; 2000 Jan;92(1 Suppl):109-13
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  • The authors present a rare case of oligodendrogliomatosis in a child, which they believe originated from a primary spinal cord tumor.
  • At 2.5 years of age this boy developed poor balance, neck stiffness, and a regression in developmental milestones.
  • A computerized tomography (CT) scan of the head initially revealed ventriculomegaly and multiple cystic cerebellar lesions.
  • A CT scan of the head and an MR image obtained 3 years later demonstrated diffuse small cysts on the surface of the brainstem, cerebellum, medial temporal and inferior frontal cortices, subcortical white matter, and corpus callosum suggestive of leptomeningeal tumor spread.
  • The cells appeared to migrate along the subpial space but no tumor cells were present in the subarachnoid space.
  • Despite having a complicated course, chemotherapy with carboplatin has provided the patient with long-term palliation and a high quality of life.
  • This case may represent the fifth report in the literature of oligodendrogliomatosis occurring in a child but only the third occurring with a spinal primary tumor.
  • [MeSH-major] Brain Neoplasms / pathology. Glioma / pathology. Neoplasms, Multiple Primary / pathology. Oligodendroglioma / pathology. Spinal Cord Neoplasms / pathology
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Humans. Magnetic Resonance Imaging. Male. Palliative Care. Photomicrography. Tomography, X-Ray Computed


24. Chang HT, Latorre JG, Hahn S, Dubowy R, Schelper RL: Pediatric cerebellar pleomorphic xanthoastrocytoma with anaplastic features: a case of long-term survival after multimodality therapy. Childs Nerv Syst; 2006 Jun;22(6):609-13
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  • [Title] Pediatric cerebellar pleomorphic xanthoastrocytoma with anaplastic features: a case of long-term survival after multimodality therapy.
  • CASE REPORT: A 4-year-old girl had a large midline cerebellar solid and cystic mass partially attached to the meninges.
  • The original diagnosis was glioblastoma multiforme and she was treated by a gross-total surgical resection followed by chemotherapy and radiation therapy to the posterior fossa during the ensuing 14 months.
  • She has received no further therapy and appears to be doing well 12 years later.
  • METHODS: Additional special stains and immunocytochemistry were performed on the paraffin embedded tumor sections.
  • However, the additional stains revealed that the tumor is a relatively well-circumscribed meningeal-based astrocytic tumor (positive for GFAP) with extensive reticulin deposit and focal neuronal differentiation (positive for synaptophysin).
  • A Ki67 labeling index is generally very low, but is positive in up to 5-10% of tumor cells focally.
  • In the light of the favorable clinical outcome and the overall histological features, this tumor may be best reclassified as a rare example of cerebellar pleomorphic xanthoastrocytoma with foci of anaplasia.
  • [MeSH-major] Brain Neoplasms / therapy. Cerebellum / pathology. Combined Modality Therapy. Glioblastoma / therapy
  • [MeSH-minor] Child, Preschool. Female. Gene Expression Regulation, Neoplastic / physiology. Humans. Immunohistochemistry / methods. Longitudinal Studies. Magnetic Resonance Imaging / methods. Nerve Tissue Proteins / metabolism

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  • (PMID = 16570197.001).
  • [ISSN] 0256-7040
  • [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 / Nerve Tissue Proteins
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25. Szathmari A, Thiesse P, Galand-desmé S, Mottolese C, Bret P, Jouanneau E, Guyotat J, Lion-François L, Frappaz D: Correlation between pre- or postoperative MRI findings and cerebellar sequelae in patients with medulloblastomas. Pediatr Blood Cancer; 2010 Dec 15;55(7):1310-6
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  • [Title] Correlation between pre- or postoperative MRI findings and cerebellar sequelae in patients with medulloblastomas.
  • INTRODUCTION: Immediate and delayed cerebellar dysfunction may be expected after surgical resection of a medulloblastoma.
  • MATERIAL AND METHODS: The data of 31 patients in continuous complete remission after removal of medulloblastoma, irradiation and chemotherapy, were retrospectively reviewed.
  • Magnetic Resonance Imaging (MRI) was analyzed for the following items: preoperative MRI (ratio of the surface of the tumor/posterior fossa, presence of ventricular dilatation or tonsilar hernia, involvement of the dentate nucleus) and delayed post-operative MRI (amount of cerebellar parenchyma removed, degree of cerebellar atrophy, presence of T1 hypointense regions in remaining cerebellar area and removal of region containing dentate nucleus).
  • These data were correlated with immediate and long-term cerebellar syndrome and daily life repercussions.
  • RESULTS: On preoperative MRI, the ratio of the surface of the tumor/posterior fossa and the presence of tonsilar hernia were significantly correlated with long-term sequelae on speech (respectively P = 0.027 and P = 0.05).
  • On delayed MRI, cerebellar atrophy was inversely correlated with ability to sustain daily tasks (P = 0.002).
  • Hypointense T1 territory in remaining cerebellar parenchyma significantly correlated with immediate post-operative cerebellar syndrome (P = 0.01) and showed a tendency for post-operative mutism (P = 0.087) but was not correlated with any long-term sequelae.
  • CONCLUSION: Increased cranial pressure on initial MRI and cerebellar atrophy detected on subsequent MRI studies correlated with immediate and long-term cerebellar sequelae.
  • [MeSH-major] Cerebellar Neoplasms / surgery. Magnetic Resonance Imaging. Medulloblastoma / surgery. Postoperative Complications
  • [MeSH-minor] Adolescent. Adult. Atrophy. Cerebellum / pathology. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Intracranial Hypertension / etiology. Intracranial Hypertension / pathology. Male. Young Adult

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  • (PMID = 20981689.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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26. Benesch M, Lackner H, Sovinz P, Suppan E, Schwinger W, Eder HG, Dornbusch HJ, Moser A, Triebl-Roth K, Urban C: Late sequela after treatment of childhood low-grade gliomas: a retrospective analysis of 69 long-term survivors treated between 1983 and 2003. J Neurooncol; 2006 Jun;78(2):199-205
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  • [Title] Late sequela after treatment of childhood low-grade gliomas: a retrospective analysis of 69 long-term survivors treated between 1983 and 2003.
  • The aim of the present study was to evaluate the spectrum of late effects in a large cohort of pediatric patients with low-grade gliomas (WHO grade I and II) during an observation period of 20 years.
  • Eighty-seven patients with low-grade gliomas grouped according to tumor location (cerebellum: n=28; cerebral hemispheres: n=21; central midline: n=15; brainstem: n=12; tectum: n=5; other locations: n=6) were evaluated for tumor- and/or treatment-related late effects by analysis of medical and computer records, and personal interviews.
  • Seventy patients underwent neurosurgery, 29 patients received additional radiotherapy and 20 additional chemotherapy.
  • Median follow-up of survivors is 96 months with an overall survival of 79% (cerebellum: 89%; cerebral hemispheres: 95%; central midline: 80%; brainstem: 25%; tectum: 100%; other locations: 66%).
  • Chronic medical problems (mild ataxia to multiple severe neuroendocrine deficits) are observed in 100% of patients with brainstem/central midline tumors and in 40-50% of patients with low-grade gliomas of other locations.
  • Tumor- and treatment-related late effects are common in patients with low-grade gliomas with the most severe occurring in patients with brainstem or central midline tumors.
  • As long-term survival is excellent in patients with low-grade gliomas except for tumors located in the brainstem, future treatment studies should focus on avoiding long-term late effects.
  • [MeSH-major] Brain Neoplasms / therapy. Endocrine System Diseases / epidemiology. Glioma / therapy. Nervous System Diseases / epidemiology
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / adverse effects. Austria / epidemiology. Child. Child, Preschool. Cohort Studies. Combined Modality Therapy / adverse effects. Disease-Free Survival. Female. Follow-Up Studies. Hearing Disorders / epidemiology. Hearing Disorders / etiology. Humans. Infant. Male. Radiation Injuries / epidemiology. Retrospective Studies. Survivors / statistics & numerical data. Vision Disorders / epidemiology. Vision Disorders / etiology

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  • (PMID = 16739030.001).
  • [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 / Antineoplastic Agents
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27. Chen KS, Hung PC, Wang HS, Jung SM, Ng SH: Medulloblastoma or cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease)? Pediatr Neurol; 2002 Nov;27(5):404-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Medulloblastoma or cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease)?
  • Dysplastic cerebellar gangliocytoma is a rare benign tumor associated with specific neuroimaging findings of abnormal laminated or folial pattern in the posterior fossa.
  • In patients with a posterior fossa tumor suggestive of a dysplastic gangliocytoma on neuroimaging studies, a pathologic confirmation is necessary.
  • [MeSH-major] Cerebellum / pathology. Ganglioneuroma / diagnosis. Medulloblastoma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Drug Therapy. Gadolinium. Humans. Infant. Magnetic Resonance Imaging. Male. Neoplasm, Residual / surgery. Reoperation. Tomography, X-Ray Computed


28. Slampa P, Pavelka Z, Dusek L, Hynkova L, Sterba J, Ondrova B, Princ D, Novotny T, Kostakova S: Longterm treatment results of childhood medulloblastoma by craniospinal irradiation in supine position. Neoplasma; 2007;54(1):62-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Longterm treatment results of childhood medulloblastoma by craniospinal irradiation in supine position.
  • Medulloblastoma, a primitive neuroectodermal tumor growing in cerebellum, is one of the most sensitive to radiation therapy childhood brain tumors.
  • The radiotherapy is an essential method of treatment for these tumours, but the surgery is the primary treatment of choice in medulloblastoma.
  • I this study between January 1997 and March 2005 were post-operative irradiated a total number of 33 pediatric patients aged under 15 years (median age 8.7 years) with medulloblastoma.
  • All tumors were histologically proved and were localizated infratentorially in the posterior fossa.
  • All of the patients were irradiated with a dose of 24-36 Gy to the whole craniospinal axis and boost with conformal therapy restricted to the tumor bed to the total dose of 50-54 Gy (30-36 Gy "high risk", 24-30 Gy "standard risk" group).
  • Chemotherapy received 26 patients (78%).
  • Irradiation was performed using standard fractionation (5 fractions per week) with a single dose of 1.5-1.8 Gy for craniospinal axis by photon beam (6 MV) of the linear accelerator.
  • No relationship was found between survival and age, sex or tumor size.
  • Endocrine deficits occurred in 45% (8 patients of the group were hypothyroid, 6 patients needed growth hormone replacement therapy, 1 patient had early puberty).
  • Further evaluation of the effectiveness of our therapy is not feasible due to the small number of patients.
  • [MeSH-major] Cerebellar Neoplasms / radiotherapy. Medulloblastoma / radiotherapy. Radiotherapy / methods. Supine Position
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Kaplan-Meier Estimate. Male. Neoplasm Recurrence, Local. Radiotherapy Dosage. Time Factors. Treatment Outcome






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