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1. Rodriguez FJ, Eberhart C, O'Neill BP, Slezak J, Burger PC, Goldthwaite P, Wu W, Giannini C: Histopathologic grading of adult medulloblastomas. Cancer; 2007 Jun 15;109(12):2557-65
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  • [Title] Histopathologic grading of adult medulloblastomas.
  • BACKGROUND: Histopathologic evaluation of the degree and extent of anaplasia is a useful prognostic parameter in pediatric medulloblastomas.
  • Whether the same applies to adult medulloblastomas is not known.
  • METHODS: The study included 74 adult patients with histologically confirmed medulloblastomas and retrospectively reassessed 67 cases with available slides for the presence of nodularity, collagen deposition (desmoplasia without nodules), and degree and extent of anaplasia.
  • CONCLUSIONS: The incidence of severe anaplasia in adult medulloblastomas is lower than in the pediatric population.
  • [MeSH-major] Cerebellar Neoplasms / classification. Medulloblastoma / classification
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Aged. Female. Humans. Immunoenzyme Techniques. Incidence. Male. Middle Aged. Neoplasm Staging. Prognosis. Retrospective Studies. Sex Distribution. Survival Rate

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  • [Copyright] Copyright 2007 American Cancer Society.
  • (PMID = 17487854.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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2. Badruddoja MA, Keir ST, King I, Zeidner J, Vredenburgh JJ, Muhlbaier LH, Bigner DD, Friedman HS: Activity of VNP40101M (Cloretazine) in the treatment of CNS tumor xenografts in athymic mice. Neuro Oncol; 2007 Jul;9(3):240-4
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  • [Title] Activity of VNP40101M (Cloretazine) in the treatment of CNS tumor xenografts in athymic mice.
  • The current study was designed to assess the activity of VNP40101M administered at a dose of 18 mg/kg daily for five days against a panel of human adult and pediatric CNS tumors growing subcutaneously or intracranially in athymic nude mice.
  • The results demonstrated statistically significant (p < 0.05) growth delays of 15.0, 8.3, 51.0, 60+, 60+, and 60+ days in subcutaneous xenografts derived from childhood glioblastoma multiforme (D-456 MG), childhood ependymoma (D-528 EP and D-612 EP), childhood medulloblastoma (D-425 MED), and adult malignant glioma (D-245 MG and D-54 MG), respectively, with corresponding tumor regressions in 10 of 10, 4 of 10, 8 of 10, 9 of 10, 9 of 10, and 10 of 10 treated mice, respectively.
  • Additional experiments conducted against subcutaneous D-245 MG xenografts by using reduced doses of 13.5 or 9.0 mg/kg daily for five days demonstrated tumor growth delays of 82.2 and 53.5 days, with corresponding tumor regressions in 8 of 9 and 9 of 10 treated mice, respectively (all values, p < 0.001), with one toxic death.
  • These findings suggest that VNP40101M is active in the treatment of a wide range of human central nervous system tumors and warrants translation to the clinic.

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  • (PMID = 17522334.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / P50 NS020023; United States / NINDS NIH HHS / NS / 5P50-NS20023-23
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Hydrazines; 0 / Prodrugs; 0 / Sulfonamides; 14J2G0U3NQ / laromustine
  • [Other-IDs] NLM/ PMC1907418
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3. Secondino S, Citterio A, Pedrazzoli P, Funaioli C, Scialfa GG, Siena S: Neuroimaging abnormalities in adult medulloblastoma undergoing intensified therapy. Anticancer Res; 2008 Nov-Dec;28(6B):3991-2
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  • [Title] Neuroimaging abnormalities in adult medulloblastoma undergoing intensified therapy.
  • We report on radiological abnormalities resembling recurrent tumor in adult medulloblastoma receiving intensified chemotherapy and radiotherapy.
  • Evidence provided in this paper confirms previous reports in the pediatric population and suggests that neuroradiologist and medical oncologists should be aware of new possible radiological findings related to aggressive treatments for brain tumors.
  • [MeSH-major] Brain Neoplasms / diagnosis. Medulloblastoma / diagnosis
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging

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  • (PMID = 19192661.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
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4. Giordana MT, D'Agostino C, Pollo B, Silvani A, Ferracini R, Paiolo A, Ghiglione P, Chiò A: Anaplasia is rare and does not influence prognosis in adult medulloblastoma. J Neuropathol Exp Neurol; 2005 Oct;64(10):869-74
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  • [Title] Anaplasia is rare and does not influence prognosis in adult medulloblastoma.
  • Histopathologic grading based on increasing anaplasia predicts clinical behavior of pediatric medulloblastomas.
  • The present study was aimed at grading 86 medulloblastomas of adult patients (aged 18 and older) by anaplasia and analyzing the predictive power.
  • Severe nuclear pleomorphism was found in 4 of 86 cases; the only large-cell medulloblastoma was from an 18-year-old patient.
  • The histologic spectrum of medulloblastoma in adults is different from that in children.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Medulloblastoma / pathology
  • [MeSH-minor] Adult. Aged. Anaplasia. Female. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Survival Analysis

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  • (PMID = 16215458.001).
  • [ISSN] 0022-3069
  • [Journal-full-title] Journal of neuropathology and experimental neurology
  • [ISO-abbreviation] J. Neuropathol. Exp. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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5. Spiller SE, Ravanpay AC, Hahn AW, Olson JM: Suberoylanilide hydroxamic acid is effective in preclinical studies of medulloblastoma. J Neurooncol; 2006 Sep;79(3):259-70
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  • [Title] Suberoylanilide hydroxamic acid is effective in preclinical studies of medulloblastoma.
  • PURPOSE: Suberoylanilide hydroxamic acid (SAHA) has been studied in adult solid and hematologic malignancies.
  • However, little information has been reported on the effects of SAHA on central nervous system (CNS) tumors including medulloblastoma, the most common malignant brain tumor in children.
  • We investigated SAHA in preclinical medulloblastoma models to determine its anti-cancer efficacy as well as its ability to affect intracranial lesions when administered systemically.
  • EXPERIMENTAL DESIGN AND RESULTS: Tissue culture studies were performed treating primary human fibroblasts, established medulloblastoma cell lines, and primary human medulloblastoma tumors with SAHA.
  • At 10 microM concentration, SAHA had little effect on normal fibroblasts but caused >90% apoptosis in cultured medulloblastoma cells.
  • Primary medulloblastomas from patients were sensitive to SAHA compared to vehicle alone in ex vivo studies.
  • In athymic mice with medulloblastoma xenograft tumors, oral SAHA resulted in apoptosis of tumor tissue and significantly slowed tumor growth.
  • In the ND2:Smo transgenic mouse medulloblastoma model, SAHA treatment caused significant apoptosis in these cerebellar tumors.
  • CONCLUSIONS: SAHA effectively induces cell death in established medulloblastoma cell lines, human patient primary tumor cultures, medulloblastoma xenografts and intracranial spontaneous medulloblastomas.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Cerebellar Neoplasms / drug therapy. Hydroxamic Acids / pharmacology. Medulloblastoma / drug therapy

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  • (PMID = 16645722.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA112350-01
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Hydroxamic Acids; 58IFB293JI / vorinostat
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6. Mittal P, Gupta K, Saggar K, Kaur S: Adult medulloblastoma mimicking Lhermitte-Duclos disease: can diffusion weighted imaging help? Neurol India; 2009 Mar-Apr;57(2):203-5
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  • [Title] Adult medulloblastoma mimicking Lhermitte-Duclos disease: can diffusion weighted imaging help?
  • Lhermitte-Duclos disease, also known as dysplastic cerebellar gangliocytoma, is a rare cerebellar benign tumor with characteristic appearance of thickened cerebellar folia giving a laminated or striated appearance, quite diagnostic of the condition.
  • We had seen a patient with medulloblastoma with imaging findings suspicious for thickened cerebellar folia reminiscent of Lhermitte-Duclos disease.
  • [MeSH-major] Cerebellar Neoplasms / diagnosis. Diffusion Magnetic Resonance Imaging. Hamartoma Syndrome, Multiple / diagnosis. Medulloblastoma / diagnosis
  • [MeSH-minor] Contrast Media. Humans. Magnetic Resonance Imaging. Male. Young Adult

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  • (PMID = 19439857.001).
  • [ISSN] 0028-3886
  • [Journal-full-title] Neurology India
  • [ISO-abbreviation] Neurol India
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Contrast Media
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7. Lai R: Survival of patients with adult medulloblastoma: a population-based study. Cancer; 2008 Apr 1;112(7):1568-74
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  • [Title] Survival of patients with adult medulloblastoma: a population-based study.
  • BACKGROUND: Adult medulloblastoma accounts for less than 1% of adult intracranial tumors.
  • RESULTS: Four hundred fifty-four patients with adult medulloblastoma were diagnosed from 1973-2004 in the 17 regions covered by SEER.
  • [MeSH-major] Cerebellar Neoplasms / mortality. Medulloblastoma / mortality
  • [MeSH-minor] Adult. Cohort Studies. Female. Follow-Up Studies. Humans. Male. Prognosis. Registries. SEER Program. Survival Rate

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  • (PMID = 18278809.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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8. Privitera G, Acquaviva G, Ettorre GC, Spatola C: Antiangiogenic therapy in the treatment of recurrent medulloblastoma in the adult: case report and review of the literature. J Oncol; 2009;2009:247873

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  • [Title] Antiangiogenic therapy in the treatment of recurrent medulloblastoma in the adult: case report and review of the literature.
  • Medulloblastoma is a rare tumor in central nervous system, with an even rarer occurrence in adulthood.
  • We report the case of a 51-year-old man with recurrent medulloblastoma.
  • The aim of this report is to show that recurrent medulloblastoma in adults can be approached with a multimodality treatment and that antiangiogenic therapy should have a role in the management of this disease.

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  • (PMID = 20111585.001).
  • [ISSN] 1687-8469
  • [Journal-full-title] Journal of oncology
  • [ISO-abbreviation] J Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2804042
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9. Germanwala AV, Mai JC, Tomycz ND, Niranjan A, Flickinger JC, Kondziolka D, Lunsford LD: Boost Gamma Knife surgery during multimodality management of adult medulloblastoma. J Neurosurg; 2008 Feb;108(2):204-9
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  • [Title] Boost Gamma Knife surgery during multimodality management of adult medulloblastoma.
  • OBJECT: The aim of this paper was to determine prognostic factors for adult medulloblastoma treated with boost Gamma Knife surgery (GKS) following resection and craniospinal irradiation.
  • METHODS: The authors performed a retrospective analysis of 12 adult patients with histologically proven medulloblastoma or supratentorial primitive neuroectodermal tumor who between February 1991 and December 2004 underwent >or=1 sessions of GKS for posttreatment residual or recurrent tumors (6 tumors in each group).
  • Stereotactic radiosurgery was applied to residual and recurrent posterior fossa tumor as well as to foci of intracranial medulloblastoma metastases.
  • The mean GKS-treated tumor volume was 9.4 cm3 (range 0.5-39 cm3).
  • RESULTS: Following adjunctive radiosurgery, 5 patients had no evidence of tumor on magnetic resonance (MR) imaging, 3 patients had stable tumor burden on MR imaging, and 4 patients had evidence of tumor progression locally with or without intracranial metastases.
  • All patients with tumor progression died.
  • The majority of patients who achieved tumor eradication (80%) and tumor stabilization (67%) after GKS had residual tumor as the reason for their referral for GKS.
  • The best outcomes were attained in patients with residual disease who were younger, had smaller tumor volumes, had no evidence of metastatic disease, and had received higher cumulative GKS doses.
  • CONCLUSIONS: Single or multiple GKS sessions were a well-tolerated, feasible, and effective adjunctive treatment for posterior fossa residual or recurrent medulloblastoma as well as intracranial metastatic medulloblastoma in adult patients.
  • [MeSH-major] Cerebellar Neoplasms / surgery. Medulloblastoma / surgery. Neoadjuvant Therapy. Radiosurgery / methods
  • [MeSH-minor] Adult. Age Factors. Chemotherapy, Adjuvant. Cranial Irradiation. Disease Progression. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local / radiotherapy. Neoplasm Recurrence, Local / surgery. Neoplasm, Residual. Neuroectodermal Tumors / radiotherapy. Neuroectodermal Tumors / surgery. Remission Induction. Retrospective Studies. Spine / radiation effects. Supratentorial Neoplasms / radiotherapy. Supratentorial Neoplasms / surgery. Survival Rate. Time Factors. Treatment Outcome

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  • (PMID = 18240913.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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10. Mühlisch J, Bajanowski T, Rickert CH, Roggendorf W, Würthwein G, Jürgens H, Frühwald MC: Frequent but borderline methylation of p16 (INK4a) and TIMP3 in medulloblastoma and sPNET revealed by quantitative analyses. J Neurooncol; 2007 May;83(1):17-29
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  • [Title] Frequent but borderline methylation of p16 (INK4a) and TIMP3 in medulloblastoma and sPNET revealed by quantitative analyses.
  • Certain risk groups among tumors of the central nervous system (CNS) in children take an almost inevitably fatal course.
  • Aberrant methylation is common in malignant brain tumors of childhood and may have implications for stratification and therapy.
  • Methylation of p16 (INK4A), p14 (ARF), TIMP3, CDH1, p15 (INK4B )and DAPK1 in medulloblastoma (MB) and ependymoma has been discussed controversially in the literature.
  • Only p16 (INK4A )and TIMP3 were methylated consistently in medulloblastomas (p16 (INK4A ) 14%, TIMP3 11%) and p16 (INK4A) also in anaplastic ependymomas (1/4 tumors).
  • Therapeutic and diagnostic implications urge into depth analyses of methylation as a mechanism, which might fill some of the gaps of our understanding of brain tumor origin.
  • [MeSH-major] Brain Neoplasms / genetics. Cerebellar Neoplasms / genetics. DNA Methylation. Genes, p16. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Tissue Inhibitor of Metalloproteinase-3 / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Apoptosis Regulatory Proteins / genetics. Calcium-Calmodulin-Dependent Protein Kinases / genetics. Child. Child, Preschool. Death-Associated Protein Kinases. Female. Gene Silencing. Humans. Infant. Male. Middle Aged. Nerve Tissue Proteins / genetics. Receptors, Immunologic / genetics

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  • (PMID = 17206475.001).
  • [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 / Apoptosis Regulatory Proteins; 0 / Nerve Tissue Proteins; 0 / Receptors, Immunologic; 0 / TIMP3 protein, human; 0 / Tissue Inhibitor of Metalloproteinase-3; 0 / roundabout protein; EC 2.7.11.1 / DAPK1 protein, human; EC 2.7.11.1 / Death-Associated Protein Kinases; EC 2.7.11.17 / Calcium-Calmodulin-Dependent Protein Kinases
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11. Ang C, Hauerstock D, Guiot MC, Kasymjanova G, Roberge D, Kavan P, Muanza T: Characteristics and outcomes of medulloblastoma in adults. Pediatr Blood Cancer; 2008 Nov;51(5):603-7
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  • [Title] Characteristics and outcomes of medulloblastoma in adults.
  • BACKGROUND: Adult medulloblastoma is a rare disease for which there is no internationally accepted standard of care.
  • We sought to review the presentation, management, and outcome of patients with adult medulloblastoma treated at the McGill University teaching hospitals over the past 18 years.
  • METHODS: Medical records were reviewed to gather demographic and clinical data including presenting symptoms, tumor characteristics, management, survival, and treatment toxicity.
  • CONCLUSION: Adult medulloblastoma has distinct characteristics from the pediatric population including presentation in the lateral cerebellar hemispheres.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Cerebellar Neoplasms / therapy. Medulloblastoma / pathology. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Chemotherapy, Adjuvant / adverse effects. Combined Modality Therapy. Cranial Irradiation. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / therapy. Neurosurgical Procedures. Radiotherapy, Adjuvant / adverse effects. Salvage Therapy / methods. Treatment Outcome

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18649371.001).
  • [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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12. Padovani L, André N, Carrie C, Muracciole X: [Childhood and adult medulloblastoma: what difference?]. Cancer Radiother; 2009 Oct;13(6-7):530-5
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  • [Title] [Childhood and adult medulloblastoma: what difference?].
  • [Transliterated title] Le médulloblastome de l'enfant et de l'adulte: quelle différence?
  • Medulloblastoma is the most frequent childhood brain tumor (30%) but account only for less than 1% of adult brain tumor.
  • Due to the rarety in adult population, no prospective studies and few data about late effects are available.
  • Adult medulloblastoma is a therapeutic challenge and their therapeutic strategies are similar to pediatric protocols.
  • In order to improve the understanding of adult disease and to homogenize the treatment, National Cancer Institute (INCa) stimulated the creation of web conference to discuss each case prospectively and to propose a protocol of treatment.
  • A better comprehension of biological processes and abnormal cellular signalling pathways involved in medulloblastoma pathogenesis had led toward a new prognostic classification to adapt the therapeutic strategy and gives hope of new therapeutic tools.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Medulloblastoma / pathology
  • [MeSH-minor] Adult. Age Factors. Antineoplastic Agents / adverse effects. Antineoplastic Agents / therapeutic use. Brain Neoplasms / epidemiology. Child. Cognition Disorders / epidemiology. Cognition Disorders / etiology. Combined Modality Therapy. France / epidemiology. Humans. Incidence. Molecular Biology / methods. Radiotherapy / adverse effects. Radiotherapy / methods. Surgical Procedures, Operative

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  • (PMID = 19713143.001).
  • [ISSN] 1769-6658
  • [Journal-full-title] Cancer radiothérapie : journal de la Société française de radiothérapie oncologique
  • [ISO-abbreviation] Cancer Radiother
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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13. Hsieh PC, Wu CT, Lin KL, Jaing TH, Tseng CK, Lui TN, Jung SM: The clinical experience of medulloblastoma treatment and the significance of time sequence for development of leptomeningeal metastasis. Childs Nerv Syst; 2008 Dec;24(12):1463-7
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  • [Title] The clinical experience of medulloblastoma treatment and the significance of time sequence for development of leptomeningeal metastasis.
  • OBJECTS: Among patients with medulloblastoma, an unrare aggressive central nervous system (CNS) tumor found mostly in the posterior fossa, drop metastasis from primary location to anatomically lower sites including whole spinal cord is known as a potential way for tumor to spread.
  • There were already lots of extensive discussions regarding diagnosis and treatment for such common neurologic complication in systemic cancer; however, fewer were found about medulloblastoma.
  • MATERIALS AND METHODS: We retrospectively review a series of 36 patients who suffered from posterior fossa medulloblastoma and found leptomeningeal metastasis in 12 of them.
  • CONCLUSIONS: By survival analysis, patients with late drop metastasis, defined as new radiologic evidence of leptomeningeal involvement after primary CNS procedures, were found to have significant survival difference to those with early metastasis defined as drop metastasis found at the time of diagnosis (log-rank test, p = 0.0047).
  • [MeSH-major] Infratentorial Neoplasms / pathology. Medulloblastoma / pathology. Meningeal Neoplasms / secondary
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging / methods. Male. Prognosis. Retrospective Studies. Survival Analysis. Time Factors. Young Adult

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  • (PMID = 18802711.001).
  • [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] Journal Article
  • [Publication-country] Germany
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14. Pfister SM, Remke M, Benner A, Werft W, Mendrzyk F, Scheurlen W, Kulozik A, Lichter P, Korshunov A: Use of CDK6 oncogene amplification and 17q gain to predict poor clinical risk groups in adult medulloblastoma. J Clin Oncol; 2009 May 20;27(15_suppl):2030

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Use of CDK6 oncogene amplification and 17q gain to predict poor clinical risk groups in adult medulloblastoma.
  • : 2030 Background: While in children medulloblastoma comprises the most common malignant brain tumor, it accounts for only 1% of intracranial malignancies in adults.
  • The infrequent appearance of MB in adults poses the question, whether these tumors are the same in adults and children in terms of biological and clinical peculiarities.
  • METHODS: Array-CGH was performed for a total 34 adult medulloblastoma samples (>18 years) and results were compared with data from 101 pediatric patients.
  • Selected genomic regions were further investigated by FISH analysis in an independent cohort of 415 samples (112 adult and 303 pediatric).
  • All 146 adult patients received a standard treatment regimen consisting of tumor resection, irradiation of the neuroaxis with 36 Gy, a boost of 20-23 Gy to the posterior fossa, and eight cycles of vincristin, lomustine, and cisplatin.
  • RESULTS: Copy-number gains of chromosome 17q as well as high-level amplifications of CDK6 were identified as significant adverse prognostic markers in adult medulloblastoma.
  • Apart from one exception, CDK6 amplifications were only observed in adult patients (9% in adults versus 0.2 % in children), whereas amplifications of MYC or MYCN were significantly overrepresented in the pediatric cohort, but when present were also associated with dismal prognosis in adults.
  • Based on these results, we propose a molecular staging system for adult medulloblastoma: i) cases with oncogene amplification (10% of cases, 5-year OS = 0%);.
  • CONCLUSIONS: We report on the largest cohort of adult medulloblastoma investigated for genomic imbalances to date.
  • We propose a model for the molecular risk stratification of adult medulloblastoma comprising three distinct genomic risk groups with significantly different survival and tumor biology.

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  • (PMID = 27964634.001).
  • [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] Journal Article
  • [Publication-country] United States
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15. Selek U, Zorlu F, Hurmuz P, Cengiz M, Turker A, Soylemezoglu F, Gurkaynak M: Craniospinal radiotherapy in adult medulloblastoma. Strahlenther Onkol; 2007 May;183(5):236-40
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  • [Title] Craniospinal radiotherapy in adult medulloblastoma.
  • PURPOSE: To evaluate the outcome and prognostic factors of adult patients with medulloblastoma.
  • PATIENTS AND METHODS: 26 adult medulloblastoma patients with a median age of 27 were subjected to craniospinal radiotherapy.
  • Patient characteristics, treatment factors and tumor characteristics failed to show any significance in univariate analysis.
  • [MeSH-major] Cerebellar Neoplasms / radiotherapy. Cranial Irradiation. Medulloblastoma / radiotherapy. Spine / radiation effects
  • [MeSH-minor] Adolescent. Adult. Cohort Studies. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Male. Prognosis. Radiotherapy Dosage. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 17497094.001).
  • [ISSN] 0179-7158
  • [Journal-full-title] Strahlentherapie und Onkologie : Organ der Deutschen Röntgengesellschaft ... [et al]
  • [ISO-abbreviation] Strahlenther Onkol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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16. Ongürü O, Karslioglu Y, Ozcan A, Celik E: Anti-apoptotic and growth-promoting markers in adult medulloblastomas. Clin Neuropathol; 2010 Nov-Dec;29(6):384-9
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  • [Title] Anti-apoptotic and growth-promoting markers in adult medulloblastomas.
  • AIM: the aim of this study was to investigate the pathologic features, proliferation potential and expression of some anti-apoptotic and growth-promoting markers in adult medulloblastomas.
  • METHOD: we analyzed the immunohistochemical expression of survivin, c-KIT, Bcl-2, fascin, p-53 and Ki-67 in 18 adult medulloblastomas (> 16 years of age).
  • 14 cases were classical, 2 desmoplastic/nodular and 2 large cell medulloblastomas.
  • Moderate-to-high nuclear survivin expression was observed with high percentages (55 - 100%) in all medulloblastomas while Bcl-2 was mildly positive in only 1 case.
  • Fascin expression was observed in 13 medulloblastomas (72%), 9 of which showing moderate to high immunoreactivity.
  • CONCLUSION: frequent nuclear survivin expression implies the predominance of anti-apoptotic factors in pathogenesis of adult medulloblastomas.
  • It may also be a potential therapeutic target for adult medulloblastomas.
  • Although Blc-2 immunoreactivity was previously reported in approximately 30% in medulloblastomas, we have observed that it is rarely expressed in the present series of adult medulloblastomas.
  • To our knowledge, this is the first study evaluating fascin expression in medulloblastomas.
  • Mild-to-moderate cytoplasmic c-KIT immunoreactivity without membranous staining in adult medulloblastomas may support the previous studies reporting low level of c-KIT protein expression with lack of activating mutations in medulloblastomas.
  • It seems p53 is rarely involved in the course of develepment of adult medulloblastomas.
  • [MeSH-major] Apoptosis Regulatory Proteins / metabolism. Cerebellar Neoplasms / metabolism. Growth Substances / metabolism. Medulloblastoma / metabolism
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / metabolism. Carrier Proteins / metabolism. Female. Humans. Inhibitor of Apoptosis Proteins. Male. Microfilament Proteins / metabolism. Microtubule-Associated Proteins / metabolism. Middle Aged. Proto-Oncogene Proteins c-bcl-2 / metabolism. Proto-Oncogene Proteins c-kit / metabolism. Retrospective Studies. Tumor Suppressor Protein p53 / metabolism. Young Adult

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  • (PMID = 21073843.001).
  • [ISSN] 0722-5091
  • [Journal-full-title] Clinical neuropathology
  • [ISO-abbreviation] Clin. Neuropathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Apoptosis Regulatory Proteins; 0 / BIRC5 protein, human; 0 / Biomarkers, Tumor; 0 / Carrier Proteins; 0 / Growth Substances; 0 / Inhibitor of Apoptosis Proteins; 0 / Microfilament Proteins; 0 / Microtubule-Associated Proteins; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / Tumor Suppressor Protein p53; 146808-54-0 / fascin; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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17. Engelhard HH, Corsten LA: Leptomeningeal metastasis of primary central nervous system (CNS) neoplasms. Cancer Treat Res; 2005;125:71-85
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  • [Title] Leptomeningeal metastasis of primary central nervous system (CNS) neoplasms.
  • Leptomeningeal dissemination of primary CNS tumors varies widely by histologic subtype.
  • In certain tumors including medulloblastoma, ependymoma, germ cell tumors, and primary CNS lymphoma, seeding of the cerebrospinal fluid space is a critical factor in determining stage, prognosis and appropriate therapy.
  • Other tumor types, such as glioma, may have radiographic evidence of leptomeningeal metastases without clear impact on prognosis or therapy.
  • [MeSH-major] Brain Neoplasms / pathology. Central Nervous System Neoplasms / pathology. Meningeal Neoplasms / secondary
  • [MeSH-minor] Adult. Child. Humans. Incidence

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  • (PMID = 16211884.001).
  • [ISSN] 0927-3042
  • [Journal-full-title] Cancer treatment and research
  • [ISO-abbreviation] Cancer Treat. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 86
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18. Santagata S, Hornick JL, Ligon KL: Comparative analysis of germ cell transcription factors in CNS germinoma reveals diagnostic utility of NANOG. Am J Surg Pathol; 2006 Dec;30(12):1613-8
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  • [Title] Comparative analysis of germ cell transcription factors in CNS germinoma reveals diagnostic utility of NANOG.
  • To assess the diagnostic utility of NANOG in central nervous system (CNS) germ cell tumors, we analyzed its expression by immunohistochemistry in a series of 12 CNS germinomas and compared its expression with other stem cell markers.
  • Strong nuclear expression of NANOG was demonstrated in >90% of the tumor cells in all cases.
  • NANOG was not detected in tumor types frequently considered in the differential diagnosis of CNS germinoma: pineoblastoma, primitive neuroectodermal tumors, medulloblastoma, lymphoma, pituitary adenoma, atypical teratoid/rhabdoid tumor, Langerhans cell histiocytosis, and gliomas.
  • These findings demonstrate that NANOG is a sensitive and specific marker of CNS germinoma.
  • These findings also suggest a potential biologic role for NANOG in maintenance of CNS germinoma.
  • [MeSH-major] Brain Neoplasms / metabolism. DNA-Binding Proteins / metabolism. Germinoma / metabolism. Homeodomain Proteins / metabolism
  • [MeSH-minor] Adolescent. Adult. Alkaline Phosphatase / metabolism. Biomarkers, Tumor / metabolism. Cell Nucleus / metabolism. Child. HMGB Proteins / metabolism. Humans. Isoenzymes / metabolism. Octamer Transcription Factor-3 / metabolism. SOXB1 Transcription Factors. Transcription Factors / metabolism

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  • (PMID = 17122519.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / NS047213
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA-Binding Proteins; 0 / HMGB Proteins; 0 / Homeodomain Proteins; 0 / Isoenzymes; 0 / NANOG protein, human; 0 / Octamer Transcription Factor-3; 0 / POU5F1 protein, human; 0 / SOX2 protein, human; 0 / SOXB1 Transcription Factors; 0 / Transcription Factors; 0 / germ-cell AP isoenzyme; EC 3.1.3.1 / Alkaline Phosphatase
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19. Holland H, Koschny R, Krupp W, Meixensberger J, Bauer M, Schober R, Kirsten H, Ganten TM, Ahnert P: Cytogenetic and molecular biological characterization of an adult medulloblastoma. Cancer Genet Cytogenet; 2007 Oct 15;178(2):104-13
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  • [Title] Cytogenetic and molecular biological characterization of an adult medulloblastoma.
  • Medulloblastoma is a malignant invasive embryonal tumor, occurring in children mainly.
  • It is rare in adults (<1% of adult brain tumors), and so comprehensive cytogenetic and molecular biological data on adult medulloblastomas are very limited.
  • We performed comprehensive cytogenetic analyses of an adult medulloblastoma, WHO grade IV, using trypsin-Giemsa staining (GTG-banding), multicolor fluorescence in situ hybridization (M-FISH), and locus-specific FISH, complemented by molecular karyotyping using high-density single nucleotide polymorphism (SNP) arrays.
  • Molecular karyotyping by SNP array confirmed chromosomal changes -2p, -10q, -16q, and -Xq and revealed de novo partial uniparental disomy 1q and 9q.
  • Applying an upcoming therapeutic approach, we found that primary medulloblastoma cells were resistant to TRAIL, a novel anticancer cytokine, but could be efficiently sensitized by cotreatment with the proteasome inhibitor bortezomib.
  • Bortezomib-TRAIL cotreatment may serve as a powerful therapeutic option for medulloblastoma patients.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Medulloblastoma / genetics
  • [MeSH-minor] Adult. Child. Chromosome Aberrations. Chromosome Banding. Female. Flow Cytometry. Gene Expression Profiling. Gene Expression Regulation, Neoplastic. Humans. In Situ Hybridization, Fluorescence. Karyotyping. Matrix Metalloproteinase 2 / genetics. Phosphopyruvate Hydratase / genetics. Polymorphism, Single Nucleotide. Synaptophysin / genetics

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  • (PMID = 17954265.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Synaptophysin; EC 3.4.24.24 / Matrix Metalloproteinase 2; EC 4.2.1.11 / Phosphopyruvate Hydratase
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20. Chargari C, Feuvret L, Levy A, Lamproglou I, Assouline A, Hemery C, Ghorbal L, Lopez S, Tep B, G GB, Lang P, Laigle-Donadey F, Cornu P, Mazeron JJ, Simon JM: Reappraisal of clinical outcome in adult medulloblastomas with emphasis on patterns of relapse. Br J Neurosurg; 2010 Aug;24(4):460-7
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  • [Title] Reappraisal of clinical outcome in adult medulloblastomas with emphasis on patterns of relapse.
  • BACKGROUND: Clinical outcome and prognostic factors were assessed in adult medulloblastoma patients, with emphasis on patterns of relapse.
  • PATIENTS AND METHODS: Records of 36 consecutive adult patients with medulloblastoma were reviewed.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Medulloblastoma / pathology. Neoplasm Recurrence, Local / pathology
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant / methods. Disease-Free Survival. Female. Humans. Magnetic Resonance Angiography. Male. Middle Aged. Outcome Assessment (Health Care). Prognosis. Radiotherapy, Adjuvant / methods. Young Adult

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  • (PMID = 20726753.001).
  • [ISSN] 1360-046X
  • [Journal-full-title] British journal of neurosurgery
  • [ISO-abbreviation] Br J Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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21. Howes TL, Buatti JM, Kirby PA, Carlisle TL, Ryken TC: Radiation induced adult medulloblastoma: a case report. J Neurooncol; 2006 Nov;80(2):191-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Radiation induced adult medulloblastoma: a case report.
  • Adult medulloblastoma is a rare intracranial tumor.
  • Our patient is a 61 year old woman treated with cranial irradiation 15 years previously for a low grade astrocytoma in the left posterior temporal lobe that was recently diagnosed with medulloblastoma in the right cerebellum.
  • This is the first reported case of radiation induced adult medulloblastoma.
  • [MeSH-major] Cerebellar Neoplasms / etiology. Medulloblastoma / etiology. Neoplasms, Radiation-Induced / pathology
  • [MeSH-minor] Astrocytoma / radiotherapy. Brain Neoplasms / radiotherapy. Craniotomy. Female. Humans. Magnetic Resonance Imaging. Middle Aged. Neurosurgical Procedures. Temporal Lobe / pathology

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  • (PMID = 16710747.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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22. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • As in pediatric medulloblastoma patients, primary infiltration of the floor of the 4(th) ventricle indicates a poor prognosis.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Medulloblastoma / therapy
  • [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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23. Kao CL, Chiou SH, Ho DM, Chen YJ, Liu RS, Lo CW, Tsai FT, Lin CH, Ku HH, Yu SM, Wong TT: Elevation of plasma and cerebrospinal fluid osteopontin levels in patients with atypical teratoid/rhabdoid tumor. Am J Clin Pathol; 2005 Feb;123(2):297-304
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  • [Title] Elevation of plasma and cerebrospinal fluid osteopontin levels in patients with atypical teratoid/rhabdoid tumor.
  • Osteopontin, a cancer metastasis-associated gene, is specifically up-regulated in central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT), but its biological behavior in the progression of CNS AT/RT has never been studied.
  • We obtained plasma, cerebrospinal fluid (CSF), and brain tissue specimens from lobectomy or hemispherectomy samples from 39 patients (medulloblastoma, 16; AT/RT, 8; epilepsy, 6; hydrocephalus, 9).
  • By enzyme-linked immunosorbent assay, the median osteopontin levels in plasma and CSF in AT/RT (852.0 and 1,175.0 ng/mL, respectively) were significantly higher than in medulloblastoma (492.5 and 524.5 ng/mL, respectively) and hydrocephalus and epilepsy (208.0 and 168.0 ng/mL, respectively) (P < .05).
  • The results of real-time reverse transcriptase-polymerase chain reaction and immunohistochemical analysis demonstrated that osteopontin expression in AT/RT (n = 5) was significantly higher than in medulloblastoma (n = 8) samples.
  • The differences in osteopontin expression in plasma, CSF, and tumor samples in AT/RT and medulloblastoma correlated with survival differences.
  • [MeSH-major] Brain Neoplasms / metabolism. Rhabdoid Tumor / metabolism. Sialoglycoproteins. Teratoma / metabolism
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Enzyme-Linked Immunosorbent Assay. Humans. Infant. Infant, Newborn. Medulloblastoma / metabolism. Medulloblastoma / mortality. Medulloblastoma / pathology. Neoplasm Recurrence, Local. Osteopontin. Survival Rate

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  • (PMID = 15842057.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / SPP1 protein, human; 0 / Sialoglycoproteins; 106441-73-0 / Osteopontin
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24. Menon G, Krishnakumar K, Nair S: Adult medulloblastoma: clinical profile and treatment results of 18 patients. J Clin Neurosci; 2008 Feb;15(2):122-6
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  • [Title] Adult medulloblastoma: clinical profile and treatment results of 18 patients.
  • The objective of this article is to examine the clinicoradiological features and surgical outcomes of adult patients (>16 years) with medulloblastoma.
  • An attempt was made to identify the predictors of poor outcome and assess patterns of relapse and to compare these with pediatric medulloblastoma.
  • Retrospective case record analyses were performed on 18 adults (>16 years) and 79 children (<16 years) operated upon after January 1990, who had at least 5 years of follow-up.
  • The tumor was located in the vermis in 12 patients (66.6%) and in the cerebellar hemisphere in six (16.6%).
  • In spite of recent advances in management, patients with medulloblastoma still have a poor prognosis.
  • However, adults fared better than children.
  • Vermian location had a better outcome in adults, but not in children.
  • Desmoplastic variant histology was not observed to be a significant prognostic factor in the adult group while brain stem invasion carried a poor prognosis.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Clinical Trials as Topic. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Age Factors. Female. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Statistics, Nonparametric. Treatment Outcome

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  • (PMID = 18078755.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Scotland
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25. Poelen J, Bernsen HJ, Prick MJ: Metastatic medulloblastoma in an adult; treatment with temozolomide. Acta Neurol Belg; 2007 Jun;107(2):51-4
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  • [Title] Metastatic medulloblastoma in an adult; treatment with temozolomide.
  • Medulloblastoma is a malignant brain tumour most frequently seen in children.
  • Relapses of medulloblastoma are sensitive to chemotherapy and treatment with chemotherapeutics in children has increased the survival rates.
  • A medulloblastoma at adult age is extremely rare, and there is no overall accepted treatment, especially not in the case of a relapse.
  • This observation encouraged us to decide to treat an adult patient with a recurrent medulloblastoma with temozolomide.
  • This female patient showed a recurrence of a medulloblastoma 7 years after the initial presentation with metastatic spread along the neuraxis and progressive neurological deterioration.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Cerebellar Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Medulloblastoma / drug therapy. Neoplasm Recurrence, Local / drug therapy
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging. Spinal Cord Neoplasms / drug therapy. Spinal Cord Neoplasms / secondary

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  • (PMID = 17710841.001).
  • [ISSN] 0300-9009
  • [Journal-full-title] Acta neurologica Belgica
  • [ISO-abbreviation] Acta Neurol Belg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Belgium
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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26. Brandes AA, Franceschi E, Tosoni A, Blatt V, Ermani M: Long-term results of a prospective study on the treatment of medulloblastoma in adults. Cancer; 2007 Nov 1;110(9):2035-41
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  • [Title] Long-term results of a prospective study on the treatment of medulloblastoma in adults.
  • BACKGROUND: Because medulloblastoma (MB) is rare in adults, the few studies on this condition have been retrospective, and the follow-up has tended to be short.
  • METHODS: In 1989, a prospective Phase II trial was initiated to evaluate the efficacy of treatment for adults with MB.
  • Patients were staged completely with a neuroradiologic examination of the brain and neuroaxis and by cerebrospinal fluid cytology, according to Chang's staging system.
  • RESULTS: After a median follow up of 7.6 years, among a total of 36 adults with MB, the overall progression-free survival (PFS) and overall survival (OS) rates at 5 years were 72% and 75%, respectively.
  • CONCLUSIONS: In adult patients with MB, long-term follow-up was essential for evaluating the real impact of treatments.
  • [MeSH-major] Cerebellar Neoplasms / drug therapy. Cerebellar Neoplasms / radiotherapy. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Kaplan-Meier Estimate. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local / epidemiology. Neoplasm Recurrence, Local / mortality. Neoplasm Recurrence, Local / therapy. Neoplasm Staging. Prospective Studies. Radiotherapy. Survival Rate. Time. Treatment Outcome

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  • (PMID = 17823910.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article
  • [Publication-country] United States
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27. Furtado SV, Venkatesh PK, Dadlani R, Reddy K, Hegde AS: Adult medulloblastoma and the "dural-tail" sign: rare mimic of a posterior petrous meningioma. Clin Neurol Neurosurg; 2009 Jul;111(6):540-3
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  • [Title] Adult medulloblastoma and the "dural-tail" sign: rare mimic of a posterior petrous meningioma.
  • The histological diagnosis was classical medulloblastoma.
  • We review literature of this atypical presentation of medulloblastoma and "dural-tail" sign, which can be associated with other benign or malignant lesions.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Cerebellopontine Angle / pathology. Dura Mater / pathology. Infratentorial Neoplasms / pathology. Medulloblastoma / pathology. Meningioma / pathology
  • [MeSH-minor] Adult. Humans. Magnetic Resonance Spectroscopy. Male. Petrous Bone. Treatment Outcome

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  • (PMID = 19285790.001).
  • [ISSN] 1872-6968
  • [Journal-full-title] Clinical neurology and neurosurgery
  • [ISO-abbreviation] Clin Neurol Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 19
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28. Lasky JL 3rd, Choe M, Nakano I: Cancer stem cells in pediatric brain tumors. Curr Stem Cell Res Ther; 2009 Dec;4(4):298-305
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  • [Title] Cancer stem cells in pediatric brain tumors.
  • Central nervous system (CNS) tumors remain the leading cause of death among pediatric neoplasms.
  • Although standard therapies cure many pediatric CNS tumors, the long-term cognitive and physical consequences of these therapies are devastating.
  • Although recent studies have focused on molecular mechanisms that underlie the initiation and progression of adult glioblastoma multiforme (GBM), these tumors differ phenotypically and at a molecular level from pediatric brain tumors.
  • Recent investigations have identified a stem cell population, termed "brain tumor stem cells" (BTSC) within the heterogeneous cell populations that comprise malignant brain tumors which may be partly responsible for the resistance to current therapies.
  • These have been identified in several pediatric tumors including medulloblastoma, ependymomas, and malignant gliomas.
  • By exploiting molecular differences present within these heterogeneous populations of brain tumor cells, we may be able to achieve specific eradication of BTSC and long-lasting remissions, while causing less toxicity to normal tissues.
  • In this review, we describe the issues surrounding the identification and characterization of BTSC, the molecular biology of BTSC for different pediatric brain tumors, and suggest future avenues for the development of treatments for this devastating disease.
  • [MeSH-major] Brain Neoplasms / pathology. Ependymoma / pathology. Medulloblastoma / pathology. Neoplastic Stem Cells / pathology. Optic Nerve Glioma / pathology
  • [MeSH-minor] Adult Stem Cells / pathology. Biomarkers / metabolism. Cell Differentiation. Chemotherapy, Adjuvant. Child. Humans. Surgical Procedures, Operative


29. Nicholson HS, Kretschmar CS, Krailo M, Bernstein M, Kadota R, Fort D, Friedman H, Harris MB, Tedeschi-Blok N, Mazewski C, Sato J, Reaman GH: Phase 2 study of temozolomide in children and adolescents with recurrent central nervous system tumors: a report from the Children's Oncology Group. Cancer; 2007 Oct 1;110(7):1542-50
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  • [Title] Phase 2 study of temozolomide in children and adolescents with recurrent central nervous system tumors: a report from the Children's Oncology Group.
  • BACKGROUND: Effective chemotherapy is lacking for most types of central nervous system (CNS) tumors in children.
  • Temozolomide, an agent with activity against adult brain tumors, was investigated in children and adolescents with recurrent CNS tumors.
  • RESULTS: The cohort comprised 122 patients, including 113 with CNS tumors.
  • Among 104 evaluable patients with CNS tumors, 5 PRs and 1 CR were observed.
  • PRs occurred in 1 of 23 evaluable patients with high-grade astrocytoma, 1 of 21 with low-grade astrocytoma, and 3 of 25 with medulloblastoma/primitive neuroectodermal tumor (PNET).
  • The CR occurred in an additional patient with medulloblastoma/PNET.
  • No responses were observed in patients with ependymoma, brain-stem glioma, or other CNS tumors.
  • CONCLUSIONS: Although overall objective responses were limited, further exploration of temozolomide may be warranted in children with medulloblastoma and other PNETs, or in patients with low-grade astrocytoma, perhaps in a setting of less pretreatment than the patients in the current study, or in the context of multiagent therapy.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Brain Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Neoplasm Recurrence, Local / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Adult. Astrocytoma / drug therapy. Central Nervous System Neoplasms / drug therapy. Child. Child, Preschool. Drug Administration Schedule. Ependymoma / drug therapy. Female. Humans. Infant. Male. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Treatment Outcome


30. Ueba T, Kadota E, Kano H, Yamashita K, Kageyama N: MATH-1 production by an adult medulloblastoma suggestive of a cerebellar external granule cell precursor origin. J Clin Neurosci; 2008 Jan;15(1):84-7
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  • [Title] MATH-1 production by an adult medulloblastoma suggestive of a cerebellar external granule cell precursor origin.
  • Radiological, histological and molecular findings in an uncommon adult case of cerebellar medulloblastoma suggested an external granular cell precursor origin.
  • Postoperative neuronal imaging studies showed that the tumor located in the cerebellar folia had been removed totally.
  • Pathological examination identified it as a desmoplastic medulloblastoma with subpial and subarachnoid infiltration and some infiltration into the molecular and granular layer via the perivascular space.
  • Polymerase chain reaction and immunohistochemical findings revealed the presence of MATH-1, expressed in cerebellar external granule cell precursors during fetal development, in the tumor cells.
  • These findings suggest that the tumor arose from external granule cell precursors of the cerebellum and that it was therefore of neuronal lineage.
  • [MeSH-major] Basic Helix-Loop-Helix Transcription Factors / metabolism. Cerebellar Neoplasms / metabolism. Cerebellar Neoplasms / pathology. Medulloblastoma / metabolism. Medulloblastoma / pathology. Neurons / physiology
  • [MeSH-minor] Adult. Female. Gene Expression Regulation, Neoplastic. Humans. Magnetic Resonance Imaging / methods. Neoplastic Stem Cells / physiology. Phosphopyruvate Hydratase / metabolism

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  • (PMID = 18032051.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
  • [Chemical-registry-number] 0 / ATOH1 protein, human; 0 / Basic Helix-Loop-Helix Transcription Factors; EC 4.2.1.11 / Phosphopyruvate Hydratase
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31. Sasayama T, Nishihara M, Tanaka K, Mizukawa K, Ehara K, Kanomata N, Kohmura E: Two metachronous tumors induced by radiation therapy: case report and review of the literature. J Neurooncol; 2008 Jul;88(3):315-20
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  • A 1-year-old boy had undergone tumor removal and craniospinal radiation therapy (30 Gy) for cerebellar medulloblastoma.
  • Six years later, an infiltrative tumor was newly found in the right fronto-temporal white matter.
  • The patient underwent stereotactic biopsy, and the tumor was found to be an anaplastic astrocytoma.
  • To our knowledge, this case is the fourth case of radiation-induced double CNS tumors arising after radiotherapy to be described in the literature.
  • Whenever radiation is administered to children or young adults, careful serial screening studies are needed.
  • [MeSH-major] Astrocytoma / pathology. Brain Neoplasms / pathology. Meningeal Neoplasms / pathology. Meningioma / pathology. Neoplasms, Radiation-Induced / pathology. Neoplasms, Second Primary / pathology
  • [MeSH-minor] Adult. Age of Onset. Cerebellar Neoplasms / radiotherapy. Cranial Irradiation / adverse effects. Humans. In Situ Hybridization, Fluorescence. Infant. Loss of Heterozygosity. Magnetic Resonance Imaging. Male. Medulloblastoma / radiotherapy

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  • (PMID = 18373066.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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32. Kieran MW, Packer RJ, Onar A, Blaney SM, Phillips P, Pollack IF, Geyer JR, Gururangan S, Banerjee A, Goldman S, Turner CD, Belasco JB, Broniscer A, Zhu Y, Frank E, Kirschmeier P, Statkevich P, Yver A, Boyett JM, Kun LE: Phase I and pharmacokinetic study of the oral farnesyltransferase inhibitor lonafarnib administered twice daily to pediatric patients with advanced central nervous system tumors using a modified continuous reassessment method: a Pediatric Brain Tumor Consortium Study. J Clin Oncol; 2007 Jul 20;25(21):3137-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phase I and pharmacokinetic study of the oral farnesyltransferase inhibitor lonafarnib administered twice daily to pediatric patients with advanced central nervous system tumors using a modified continuous reassessment method: a Pediatric Brain Tumor Consortium Study.
  • PURPOSE: A dose-escalation phase I and pharmacokinetic study of the farnesyltransferase inhibitor lonafarnib (SCH66336) was conducted in children with recurrent or progressive CNS tumors.
  • RESULTS: Fifty-three children with progressive or recurrent brain tumors were enrolled, with a median age of 12.2 years (range, 3.9 to 19.5 years).
  • Both radiographic response (one anaplastic astrocytoma) and stable disease (one medulloblastoma, two high-grade and four low-grade gliomas, one ependymoma, and one sarcoma) were noted, and seven patients remained on treatment for 1 year or longer.
  • [MeSH-major] Central Nervous System Neoplasms / drug therapy. Central Nervous System Neoplasms / mortality. Enzyme Inhibitors / pharmacokinetics. Farnesyltranstransferase / antagonists & inhibitors. Neoplasm Invasiveness / pathology. Piperidines / pharmacokinetics. Pyridines / pharmacokinetics
  • [MeSH-minor] Administration, Oral. Adolescent. Adult. Child. Child, Preschool. Dose-Response Relationship, Drug. Drug Administration Schedule. Female. Follow-Up Studies. Humans. Infant. Infant, Newborn. Male. Maximum Tolerated Dose. Neoplasm Staging. Risk Assessment. Survival Analysis. Treatment Outcome

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  • (PMID = 17634493.001).
  • [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
  • [Grant] United States / NCI NIH HHS / CA / U01 CA81457
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Enzyme Inhibitors; 0 / Piperidines; 0 / Pyridines; 193275-84-2 / lonafarnib; EC 2.5.1.29 / Farnesyltranstransferase
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33. Yasuda K, Taguchi H, Sawamura Y, Ikeda J, Aoyama H, Fujieda K, Ishii N, Kashiwamura M, Iwasaki Y, Shirato H: Low-dose craniospinal irradiation and ifosfamide, cisplatin and etoposide for non-metastatic embryonal tumors in the central nervous system. Jpn J Clin Oncol; 2008 Jul;38(7):486-92
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  • [Title] Low-dose craniospinal irradiation and ifosfamide, cisplatin and etoposide for non-metastatic embryonal tumors in the central nervous system.
  • OBJECTIVE: The current study was conducted to evaluate the effects of low-dose craniospinal irradiation (CSI) combined with chemotherapy on non-metastatic embryonal tumors in the central nervous system (CNS), including medulloblastoma and supra-tentorial primitive neuroectodermal tumors (ST-PNET).
  • The dose to the primary tumor bed was 39.6-54 Gy.
  • Both 5-year OAS and PFS were 82% (CI: 59-100%) for the patients with medulloblastoma and 80% (CI: 45-100%) for the patients with ST-PNET.
  • CONCLUSION: Low-dose CSI and ICE chemotherapy may have a role as a treatment option for a subset of patients with non-metastatic embryonal tumors in the CNS.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Brain Neoplasms / therapy. Cranial Irradiation. Medulloblastoma / therapy. Neuroectodermal Tumors, Primitive / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Dose-Response Relationship, Radiation. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Infant. Male. Radiotherapy Dosage. Supratentorial Neoplasms / pathology. Supratentorial Neoplasms / therapy. Survival Analysis

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  • (PMID = 18573848.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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34. Hambardzumyan D, Becher OJ, Holland EC: Cancer stem cells and survival pathways. Cell Cycle; 2008 May 15;7(10):1371-8
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  • Gliomas and medulloblastomas are the most frequent malignant brain tumors in adult and children respectively.
  • Although both tumors arise in the CNS, there is a significant difference in their therapeutic response.
  • Medulloblastomas are relatively curable, while glioblastomas are basically incurable.
  • During the last decade several reports have demonstrated the existence of cancer stem cells in brain tumors, their location and their response to treatment.
  • We have recently described the therapeutic response of medulloblastomas to radiation in their native microenvironment, illustrating how p53 and Pi3K signaling pathways lead to the evasion of cell death by the nestin-expressing cells in the perivascular stem cell niche, even while the bulk of tumor succumbs to apoptosis.(1) It remains to be determined whether this mechanism of tumor resistance applies to the more complex stem-cell niche and tumor bulk of gliomas.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Medulloblastoma / radiotherapy. Neoplastic Stem Cells / radiation effects. Signal Transduction / radiation effects
  • [MeSH-minor] Animals. Humans. Intermediate Filament Proteins / metabolism. Mice. Models, Biological. Nerve Tissue Proteins / metabolism. Nestin. Phosphatidylinositol 3-Kinases / metabolism. Receptors, Notch / metabolism. Tumor Suppressor Protein p53 / metabolism

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  • (PMID = 18421251.001).
  • [ISSN] 1551-4005
  • [Journal-full-title] Cell cycle (Georgetown, Tex.)
  • [ISO-abbreviation] Cell Cycle
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 5R01CA100688-2; United States / NCI NIH HHS / CA / R01CA 5R01CA099489-1
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Intermediate Filament Proteins; 0 / NES protein, human; 0 / Nerve Tissue Proteins; 0 / Nes protein, mouse; 0 / Nestin; 0 / Receptors, Notch; 0 / Tumor Suppressor Protein p53; EC 2.7.1.- / Phosphatidylinositol 3-Kinases
  • [Number-of-references] 112
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35. Biswas S, Burke A, Cherian S, Williams D, Nicholson J, Horan G, Jefferies S, Williams M, Earl HM, Burnet NG, Hatcher H: Non-pineal supratentorial primitive neuro-ectodermal tumors (sPNET) in teenagers and young adults: Time to reconsider cisplatin based chemotherapy after cranio-spinal irradiation? Pediatr Blood Cancer; 2009 Jul;52(7):796-803
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  • [Title] Non-pineal supratentorial primitive neuro-ectodermal tumors (sPNET) in teenagers and young adults: Time to reconsider cisplatin based chemotherapy after cranio-spinal irradiation?
  • BACKGROUND: Supratentorial PNET (sPNET) are rare CNS tumors of embryonal origin arising in children and adults.
  • The treatment of sPNET for all age groups at our cancer center has been based on the management of medulloblastoma (MB), involving neurosurgical debulking followed by cranio-spinal irradiation (CSI) and systemic chemotherapy.
  • METHODS: Medical records were reviewed to gather demographic and clinical data about all embryonal CNS tumors in children and adults from 2001 to 2007.
  • Tumor pathology, clinical management and survival data were also assessed, particularly as regards those patients who received the Packer chemotherapy regimen for either sPNET or MB.
  • RESULTS: Eleven patients (five children and six adults) were identified with non-pineal sPNET, three children with pineal sPNET, and 19 patients (18 children and 1 adult) with MB.
  • There was no difference in overall survival (OS) rates between pediatric and adult sPNET.
  • We suggest that it is time to reconsider the use of this regimen in teenage and young adult non-pineal sPNET and to investigate the utility of alternative approaches.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / therapy. Cranial Irradiation. Medulloblastoma / therapy. Neuroectodermal Tumors, Primitive / therapy. Supratentorial Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Cisplatin / administration & dosage. Follow-Up Studies. Humans. Lomustine / administration & dosage. Neoplasm Staging. Retrospective Studies. Survival Rate. Treatment Outcome. Vincristine / administration & dosage. Young Adult

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  • [Copyright] (c) 2009 Wiley-Liss, Inc.
  • (PMID = 19202566.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase I; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine; Q20Q21Q62J / Cisplatin
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36. Kieran MW, Walker D, Frappaz D, Prados M: Brain tumors: from childhood through adolescence into adulthood. J Clin Oncol; 2010 Nov 10;28(32):4783-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Brain tumors: from childhood through adolescence into adulthood.
  • Clinical care is currently divided into adult or pediatric care; adolescent patients require specific expertise that most clinical practices do not have.
  • When illness coincides with the adolescent transition, the health system is severely challenged.
  • Health systems historically have varied widely in the age they choose for allocating an individual to the adult model of health care.
  • Tumors of the CNS complicate the difficult adjustments required in adolescents and young adults by virtue of their morbidity, complex treatment, and prognosis.
  • Some brain tumors are unique to children, some occur predominantly in adults, and others peak in adolescence.
  • Delays in the diagnosis of brain tumors can occur at any age but are particularly common in adolescence because of difficulties of accessing health systems, the difficulties of discriminating pathologic from typical adolescent behavioral characteristics, and changing endocrine function.
  • This article will discuss the changing brain tumor profile of children, adolescents, and adults, with a focus on our limited understanding of the adolescent/young adult transition period.
  • [MeSH-major] Brain Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adolescent Medicine. Adult. Brain / growth & development. Child. Continuity of Patient Care. Glioma / diagnosis. Glioma / therapy. Humans. Medulloblastoma / diagnosis. Medulloblastoma / therapy. Neoplasms, Germ Cell and Embryonal / diagnosis. Neoplasms, Germ Cell and Embryonal / therapy

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  • (PMID = 20458039.001).
  • [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] Journal Article; Review
  • [Publication-country] United States
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37. Sugita Y, Nakamura Y, Yamamoto M, Ogasawara S, Ohshima K, Shigemori M: Expression of KIAA 0864 protein in neuroepithelial tumors: an analysis based on the presence of monoclonal antibody HFB-16. J Neurooncol; 2008 Sep;89(2):151-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The KIAA 0864 (KA) protein is a putative protein of a cDNA from 100 cDNA clones that was newly determined from a set of size-fractionated human brain cDNA libraries and their coding potentials of large proteins (180-200 kD) by using in vitro transcription assays.
  • Among the 55 NETs, a moderate-to-intense KA protein immunoreactivity was observed in 8 of 8 medulloblastomas, 1 of 1 central nervous system supratentorial primitive neuroectodermal tumor (CNS supratentorial PNET), 4 of 4 retinoblastomas, 1 of 1 neuroblastoma, 8 of 8 central neurocytomas, 4 of 4 oligodendrogliomas, 4 of 4 oligoastrocytomas, 1 of 1 extraventricular neurocytoma, and 1 of 1 gangliocytoma.
  • In addition, it could be a useful tool for performing the differential diagnosis between GBs and CNS supratentorial PNET.
  • [MeSH-major] Cerebellar Neoplasms / metabolism. Guanine Nucleotide Exchange Factors / metabolism. Medulloblastoma / metabolism. Neoplasms, Neuroepithelial / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antibodies, Monoclonal. Child. Child, Preschool. Female. Humans. Male. Middle Aged. Retrospective Studies

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  • (PMID = 18458818.001).
  • [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 / Antibodies, Monoclonal; 0 / Guanine Nucleotide Exchange Factors; 0 / RASGRP3 protein, human
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38. Brandes AA, Franceschi E: Neuro-oncology: Genetic variation in pediatric and adult brain tumors. Nat Rev Neurol; 2010 Dec;6(12):653-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Neuro-oncology: Genetic variation in pediatric and adult brain tumors.
  • Two new studies suggest that pediatric medulloblastomas and high-grade gliomas are genetically different from the same tumors in adults.
  • Age-dependent gene expression might affect tumor biology; therefore, therapies for adult medulloblastomas or gliomas might not produce the same clinical outcomes in pediatric patients, and vice versa.
  • [MeSH-major] Brain Neoplasms / genetics. Cerebellar Neoplasms / genetics. Gene Expression Profiling. Glioma / genetics. Medulloblastoma / genetics
  • [MeSH-minor] Adult. Child. Genetic Variation. Humans

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  • (PMID = 21131914.001).
  • [ISSN] 1759-4766
  • [Journal-full-title] Nature reviews. Neurology
  • [ISO-abbreviation] Nat Rev Neurol
  • [Language] eng
  • [Publication-type] News
  • [Publication-country] England
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39. Kramer K, Humm JL, Souweidane MM, Zanzonico PB, Dunkel IJ, Gerald WL, Khakoo Y, Yeh SD, Yeung HW, Finn RD, Wolden SL, Larson SM, Cheung NK: Phase I study of targeted radioimmunotherapy for leptomeningeal cancers using intra-Ommaya 131-I-3F8. J Clin Oncol; 2007 Dec 1;25(34):5465-70
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  • PURPOSE: Tumors metastasizing to the CNS and leptomeninges (LM) are associated with significant mortality.
  • We tested the toxicity, pharmacokinetics, and dosimetry of intraventricular iodine-131-labeled monoclonal antibody 3F8 (131I-3F8) targeting GD2-positive CNS/LM disease in a phase I clinical trial.
  • Tumor response was determined by clinical, radiographic, and cytologic criteria.
  • Radioimmunoconjugates targeting GD2 may have clinical utility in the treatment of CNS/LM malignancies.
  • [MeSH-minor] Adolescent. Adult. Animals. Cerebellar Neoplasms / immunology. Cerebellar Neoplasms / radionuclide imaging. Cerebellar Neoplasms / radiotherapy. Child. Child, Preschool. Humans. Infant. Medulloblastoma / immunology. Medulloblastoma / radionuclide imaging. Medulloblastoma / radiotherapy. Mice. Mice, Inbred BALB C. Middle Aged. Neuroblastoma / immunology. Neuroblastoma / radionuclide imaging. Neuroblastoma / radiotherapy

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  • (PMID = 18048828.001).
  • [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
  • [Grant] United States / NCI NIH HHS / CA / CA72868
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 3F8 antibody; 0 / Antibodies, Monoclonal; 0 / Immunoglobulin G; 0 / Immunotoxins; 0 / Iodine Radioisotopes
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40. Roussos I, Balaña C, Cuadras P, Ballester R, Etxaniz O, Hostalot C: Medulloblastoma in young adults. Must we give adjuvant chemotherapy? Clin Transl Oncol; 2007 Feb;9(2):121-3
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  • [Title] Medulloblastoma in young adults. Must we give adjuvant chemotherapy?
  • Medulloblastoma is a rare entity in adult patients.
  • Reports of medulloblastoma in adults are scarce but in all of them the prognosis seems similar to the prognosis of children.
  • We present our experience in five cases of medulloblastoma in young adults, treated at the University Hospital "Germans Trias i Pujol" from June 1994 to October 2003.
  • We have reviewed the literature, concluding that we have to adapt the findings in children to our adult patients, offering them adjuvant chemotherapy after surgery.
  • [MeSH-major] Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Female. Humans. Male

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  • (PMID = 17329226.001).
  • [ISSN] 1699-048X
  • [Journal-full-title] Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico
  • [ISO-abbreviation] Clin Transl Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Spain
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41. Brandes AA, Franceschi E, Tosoni A, Reni M, Gatta G, Vecht C, Kortmann RD: Adult neuroectodermal tumors of posterior fossa (medulloblastoma) and of supratentorial sites (stPNET). Crit Rev Oncol Hematol; 2009 Aug;71(2):165-79
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  • [Title] Adult neuroectodermal tumors of posterior fossa (medulloblastoma) and of supratentorial sites (stPNET).
  • Medulloblastoma and supratentorial primitive neuroectodermal tumors are rare diseases in adults.
  • Due to this rarity, few prospective clinical trials have been conducted on medulloblastoma in adults, investigations being based exclusively on retrospective studies; the populations considered in literature are small, and the different treatments given span decades, during which diagnostic procedures, neurosurgical skills and radiotherapy techniques have changed.
  • Unlike pediatric patients, adult medulloblastoma patients have been treated according to risk-adapted therapeutic strategies in only a few series and despite risk-tailored treatments, 20-30% of patients experience recurrence.
  • An important challenge for the future will be the biological characterization of medulloblastoma, with the identification of specific genetic patterns of patients with a better or a worse prognosis.
  • [MeSH-major] Brain Neoplasms. Medulloblastoma. Neuroectodermal Tumors, Primitive
  • [MeSH-minor] Adolescent. Adult. Child. Female. Genetic Predisposition to Disease. Humans. Incidence. Male. Middle Aged. Neoplasm Recurrence, Local / prevention & control. Neoplasm Staging. Prognosis. Young Adult

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  • (PMID = 19303318.001).
  • [ISSN] 1879-0461
  • [Journal-full-title] Critical reviews in oncology/hematology
  • [ISO-abbreviation] Crit. Rev. Oncol. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 71
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42. Muñoz-Mármol AM, Mola G, Ruiz-Larroya T, Fernández-Vasalo A, Vela E, Mate JL, Ariza A: Rarity of JC virus DNA sequences and early proteins in human gliomas and medulloblastomas: the controversial role of JC virus in human neurooncogenesis. Neuropathol Appl Neurobiol; 2006 Apr;32(2):131-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rarity of JC virus DNA sequences and early proteins in human gliomas and medulloblastomas: the controversial role of JC virus in human neurooncogenesis.
  • Moreover, JCV genomic DNA and early viral protein T-antigen have been detected in various types of human central nervous system (CNS) neoplasms.
  • To further explore this association we have studied paraffin-embedded brain biopsy tissue from 60 neoplasms (55 gliomas and five medulloblastomas) and 15 reactive gliosis cases for the presence of JCV DNA sequences and proteins.
  • Additionally, IHC with both antibodies was applied to a tissue micro-array including 109 CNS tumours and 21 reactive gliosis samples.
  • The rarity of JCV DNA sequences and early proteins in our brain tumours enriches the controversy over the role of JCV in human neurooncogenesis, whose clarification is in need of further molecular and epidemiologic studies.
  • [MeSH-major] Brain Neoplasms / virology. DNA, Viral / isolation & purification. Glioma / virology. JC Virus / genetics. Medulloblastoma / virology
  • [MeSH-minor] Adult. Animals. Antigens, Viral, Tumor / isolation & purification. Cell Transformation, Neoplastic. Child. Female. Humans. Immunohistochemistry. Male. Middle Aged. Polymerase Chain Reaction

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  • (PMID = 16599942.001).
  • [ISSN] 0305-1846
  • [Journal-full-title] Neuropathology and applied neurobiology
  • [ISO-abbreviation] Neuropathol. Appl. Neurobiol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, Viral, Tumor; 0 / DNA, Viral
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43. Gonçalves MI, Radzinsky TC, da Silva NS, Chiari BM, Consonni D: Speech-language and hearing complaints of children and adolescents with brain tumors. Pediatr Blood Cancer; 2008 Mar;50(3):706-8
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  • [Title] Speech-language and hearing complaints of children and adolescents with brain tumors.
  • Central nervous system (CNS) tumors generally leave sequelae that may compromise speech, language, swallowing, hearing, and voice functions.
  • This report describes the incidence of speech-language and hearing complaints and disorders in children and adolescents with CNS tumor under treatment at one of the most important Brazilian reference center for pediatric cancer.
  • [MeSH-major] Brain Neoplasms / complications. Hearing Loss / etiology. Language Disorders / etiology. Speech Disorders / etiology
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / adverse effects. Antineoplastic Agents / therapeutic use. Astrocytoma / complications. Astrocytoma / drug therapy. Child. Child, Preschool. Craniopharyngioma / complications. Craniopharyngioma / drug therapy. Deglutition Disorders / etiology. Ependymoma / complications. Ependymoma / drug therapy. Facial Paralysis / etiology. Female. Humans. Infant. Male. Medulloblastoma / complications. Medulloblastoma / drug therapy

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17534932.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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44. Padovani L, Sunyach MP, Perol D, Mercier C, Alapetite C, Haie-Meder C, Hoffstetter S, Muracciole X, Kerr C, Wagner JP, Lagrange JL, Maire JP, Cowen D, Frappaz D, Carrie C: Common strategy for adult and pediatric medulloblastoma: a multicenter series of 253 adults. Int J Radiat Oncol Biol Phys; 2007 Jun 1;68(2):433-40
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  • [Title] Common strategy for adult and pediatric medulloblastoma: a multicenter series of 253 adults.
  • PURPOSE: To assess prognostic factors for adults with medulloblastoma in a multicenter, retrospective study.
  • METHODS AND MATERIALS: Data were collected by file review or mail inquiry for 253 adults treated between 1975 to 2004.
  • CONCLUSION: We report the largest series of medulloblastoma in adults.
  • [MeSH-major] Cerebellar Neoplasms / drug therapy. Cerebellar Neoplasms / radiotherapy. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Age Factors. Analysis of Variance. Combined Modality Therapy / methods. Female. Follow-Up Studies. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Survival Rate

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  • (PMID = 17498567.001).
  • [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
  • [Publication-country] United States
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45. Mühlisch J, Schwering A, Grotzer M, Vince GH, Roggendorf W, Hagemann C, Sörensen N, Rickert CH, Osada N, Jürgens H, Frühwald MC: Epigenetic repression of RASSF1A but not CASP8 in supratentorial PNET (sPNET) and atypical teratoid/rhabdoid tumors (AT/RT) of childhood. Oncogene; 2006 Feb 16;25(7):1111-7
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  • Supratentorial primitive neuroectodermal tumors (sPNET) and atypical teratoid/rhabdoid tumors (AT/RT) of the CNS represent a biological and clinical enigma, despite advances in both molecular techniques and clinical management for these two rare embryonal brain tumors of childhood.
  • In all, 17 samples of autopsy-derived normal appearing brain served as controls.
  • Treatment of cell lines using 5-Aza-2'-deoxycytidine (5AZA) alone or in combination with trichostatin A (TSA) succeeded in re-establishing expression of RASSF1A in cell lines derived from a renal rhabdoid, an AT/RT and a medulloblastoma.
  • However, CASP8 showed inconsistent expression patterns in normal and tumor tissues.
  • [MeSH-major] Brain Neoplasms / genetics. DNA Methylation. Gene Silencing. Neuroectodermal Tumors, Primitive / genetics. Rhabdoid Tumor / genetics. Teratoma / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Azacitidine / analogs & derivatives. Azacitidine / pharmacology. Caspase 8. Caspases / genetics. Child. Child, Preschool. CpG Islands. Epigenesis, Genetic. Female. Humans. Hydroxamic Acids / pharmacology. Infant. Male. Promoter Regions, Genetic

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  • (PMID = 16186793.001).
  • [ISSN] 0950-9232
  • [Journal-full-title] Oncogene
  • [ISO-abbreviation] Oncogene
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Hydroxamic Acids; 0 / RASSF1 protein, human; 0 / Tumor Suppressor Proteins; 3X2S926L3Z / trichostatin A; 776B62CQ27 / decitabine; EC 3.4.22.- / CASP8 protein, human; EC 3.4.22.- / Caspase 8; EC 3.4.22.- / Caspases; M801H13NRU / Azacitidine
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46. Onilude OE, Lusher ME, Lindsey JC, Pearson AD, Ellison DW, Clifford SC: APC and CTNNB1 mutations are rare in sporadic ependymomas. Cancer Genet Cytogenet; 2006 Jul 15;168(2):158-61
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  • The ependymoma is the second most common malignant brain tumor of childhood; however, its molecular basis is poorly understood.
  • The formation of multiple ependymomas has been reported as an occasional feature of Turcot syndrome type 2 (TS2), a familial cancer syndrome caused by inherited mutations of the APC tumor suppressor gene, and characterised by the concurrence of a primary CNS tumor (predominantly medulloblastoma) and multiple colorectal adenomas.
  • APC is a critical component of the Wnt/Wingless signaling pathway, which is disrupted in sporadic cancers (e.g., colorectal adenomas, hepatocellular carcinomas, and medulloblastomas) by somatic mutations affecting multiple genes encoding alternative pathway components, including APC and CTNNB1 (encoding beta-catenin).
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Chromatography, High Pressure Liquid. DNA Mutational Analysis. Female. Humans. Male. Middle Aged

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  • (PMID = 16843107.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein; 0 / CTNNB1 protein, human; 0 / beta Catenin
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47. De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A: Temozolomide in resistant or relapsed pediatric solid tumors. Pediatr Blood Cancer; 2006 Jul;47(1):30-6
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  • Tumor types were: neuroblastoma (NB; n = 17), medulloblastoma (MB; 8), brain stem glioma (BSG; 8), extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor (EOES; 4), Ewing's sarcoma (ES; 4), anaplastic astrocytoma (AA; 3), rhabdomyosarcoma (RMS; 2), ependymoma (EP; 2), cerebral primitive neuroectodermal tumor (cPNET; 2), hepatocarcinoma (HC; 1), and osteosarcoma (OS; 1).
  • CONCLUSION: Oral TMZ was well tolerated in children with resistant or relapsed solid tumors and showed activity in NB and CNS tumours refractory to standard chemotherapy.
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Disease-Free Survival. Dose-Response Relationship, Drug. Female. Humans. Male. Survival Analysis

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  • [Copyright] Copyright 2006 Wiley-Liss, Inc.
  • [ErratumIn] Pediatr Blood Cancer. 2006 Oct 15;47(5):647-8
  • (PMID = 16047361.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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48. Attard TM, Giglio P, Koppula S, Snyder C, Lynch HT: Brain tumors in individuals with familial adenomatous polyposis: a cancer registry experience and pooled case report analysis. Cancer; 2007 Feb 15;109(4):761-6
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  • [Title] Brain tumors in individuals with familial adenomatous polyposis: a cancer registry experience and pooled case report analysis.
  • They are at an increased risk of brain tumors, including cerebellar medulloblastoma, when compared with the general population (Brain Tumor Polyposis-BTP Type 2).
  • Genotype-phenotype correlations between APC gene mutations and central nervous system (CNS) tumors have, thus far not been successful.
  • METHODS: The authors analyzed their established hereditary CRC Registry for brain tumors in FAP pedigrees (56 families, 213 individuals), pooled their patients with BTP and known APC mutations with those reported thus far elsewhere, and compared the resulting mutation distribution of FAP-BTP with the mutation distribution for APC mutations in the US.
  • RESULTS: Twenty-eight patients from 24 families were accrued, the most common brain tumor in BTP was medulloblastoma (60%) predominantly in females (12:5) under the age of 20 (mean age 14.7 SD 9.2).
  • Analysis of the pooled APC mutation data by Chi-square test of association shows an odds ratio of 3.7 (P < .005) for all brain tumor subtypes and 13.1 (P < .001) for medulloblastoma in patients harboring segment 2 APC mutation (codons 679-1224) compared to nonsegment 2 mutation.
  • CONCLUSIONS: In patients with FAP and identifiable APC gene mutation, CNS tumors, especially medulloblastoma which developed in most cases during childhood, are more common in females with FAP and APC gene mutation in codons 686-1217.
  • Further studies are necessary to determine if this observation and the natural history of medulloblastoma in children justifies novel, aggressive, targeted screening of at-risk individuals.
  • [MeSH-major] Adenomatous Polyposis Coli / complications. Adenomatous Polyposis Coli Protein / genetics. Brain Neoplasms / complications. Mutation / genetics
  • [MeSH-minor] Adolescent. Adult. Child. Codon. Female. Humans. Male. Pedigree. Registries


49. Dagostino C, Clara E, Chio A, Giordana MT: Morphophenotype of medulloblastoma in children and adults. The size of nuclei. Clin Neuropathol; 2006 Sep-Oct;25(5):227-31
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  • [Title] Morphophenotype of medulloblastoma in children and adults. The size of nuclei.
  • OBJECTIVE: Uniform cells with round, regular nuclei characterize the typical histologic aspect of medulloblastoma.
  • Enlargement of nuclei distinguishes the large-cell medulloblastoma variant and is associated with a poor prognosis in pediatric medulloblastomas.
  • The aim of the present study was to compare the size of nuclei between pediatric and adult medulloblastomas by a morphometric analysis.
  • MATERIAL AND METHODS: In 79 neurosurgical specimens of cerebellar medulloblastomas, the maximum nuclear diameter of the largest nuclei was measured.
  • Measurements were performed with a digital-image analysis system.
  • RESULTS: The difference between the mean values in children and adults was statistically significant (p = 0,001).
  • The distribution of maximum values measured in each case had two distinct peaks in the two age groups, in 3.5% of adult cases and in more than 30% of pediatric cases the maximum nuclear size was superior to 12 microm.
  • CONCLUSIONS: The present results show that nuclei of tumor cells in pediatric medulloblastomas are larger than those in adult medulloblastomas and confirm that the phenotype of medulloblastoma is different in the two age groups.
  • Distinct genetic events can, thus, underlie medulloblastoma in childhood and adult age, the prognostic role of genetic variables can differ by age.
  • [MeSH-major] Cell Nucleus / ultrastructure. Cerebellar Neoplasms / ultrastructure. Medulloblastoma / ultrastructure
  • [MeSH-minor] Adolescent. Adult. Age Factors. Aged. Child. Child, Preschool. Female. Humans. Image Processing, Computer-Assisted. Infant. Male. Middle Aged. Prognosis

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  • (PMID = 17007445.001).
  • [ISSN] 0722-5091
  • [Journal-full-title] Clinical neuropathology
  • [ISO-abbreviation] Clin. Neuropathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
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50. Glassmann A, Molly S, Surchev L, Nazwar TA, Holst M, Hartmann W, Baader SL, Oberdick J, Pietsch T, Schilling K: Developmental expression and differentiation-related neuron-specific splicing of metastasis suppressor 1 (Mtss1) in normal and transformed cerebellar cells. BMC Dev Biol; 2007;7:111
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  • Given the prime importance of this pathway for cerebellar development and tumorigenesis, we assessed expression of Mtss1 in the developing murine cerebellum and human medulloblastoma specimens.
  • It is also expressed by granule cell precursor-derived medulloblastomas.
  • In the adult CNS, Mtss1 is found exclusively in cerebellar Purkinje cells.
  • Whereas immature granule cells express a Mtss1 variant observed also in peripheral tissues and comprising exon 12, this exon is replaced by a CNS-specific exon, 12a, in more mature granule cells and in adult Purkinje cells.
  • [MeSH-minor] Animals. Cerebellar Neoplasms / pathology. Exons. Humans. Mice. Mice, Inbred C57BL. Mice, Transgenic. Polymerase Chain Reaction. Protein Splicing / genetics. Purkinje Cells / pathology. Tumor Cells, Cultured

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  • (PMID = 17925019.001).
  • [ISSN] 1471-213X
  • [Journal-full-title] BMC developmental biology
  • [ISO-abbreviation] BMC Dev. Biol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Microfilament Proteins; 0 / Mtss1 protein, mouse; 0 / Neoplasm Proteins
  • [Other-IDs] NLM/ PMC2194783
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51. Behdad A, Perry A: Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases. Brain Pathol; 2010 Mar;20(2):441-50
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  • [Title] Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases.
  • Central nervous system (CNS) primitive neuroectodermal tumors (PNETs) include supratentorial, brain stem, and spinal cord tumors with medulloblastoma-like histopathology.
  • After re-diagnosis of three infantile cases as atypical teratoid/rhabdoid tumor (AT/RT), 33 remaining CNS PNETs were retrieved for clinicopathologic and fluorescence in situ hybridization studies.
  • We conclude that in CNS PNET: (i) routine application of INI1 immunohistochemistry helps rule out AT/RT, particularly in infants;.
  • (iii) involvement of CNS parenchyma by Ewing sarcoma/peripheral PNET is rare enough that EWS gene testing is not necessary unless significant dural involvement is present; and (iv) both anaplastic/large cell features and polysomies of 2 and 8 are associated with more aggressive clinical behavior.
  • [MeSH-major] Brain Neoplasms / genetics. Brain Neoplasms / pathology. Neuroectodermal Tumors, Primitive / genetics. Neuroectodermal Tumors, Primitive / pathology. Spinal Cord Neoplasms / genetics. Spinal Cord Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aneuploidy. Child. Child, Preschool. Chromosomes, Human, Pair 2. Chromosomes, Human, Pair 22. Chromosomes, Human, Pair 8. Female. Humans. Infant. Male. Middle Aged. Nuclear Proteins / genetics. Oncogene Proteins / genetics. RNA-Binding Protein EWS / genetics. Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / genetics. Rhabdoid Tumor / pathology. Teratoma / diagnosis. Teratoma / genetics. Teratoma / pathology. Young Adult

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  • (PMID = 19725831.001).
  • [ISSN] 1750-3639
  • [Journal-full-title] Brain pathology (Zurich, Switzerland)
  • [ISO-abbreviation] Brain Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / MYCN protein, human; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / RNA-Binding Protein EWS
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52. Spreafico F, Massimino M, Gandola L, Cefalo G, Mazza E, Landonio G, Pignoli E, Poggi G, Terenziani M, Pedrazzoli P, Siena S, Fossati-Bellani F: Survival of adults treated for medulloblastoma using paediatric protocols. Eur J Cancer; 2005 Jun;41(9):1304-10
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  • [Title] Survival of adults treated for medulloblastoma using paediatric protocols.
  • We retrospectively studied 26 consecutive adults treated for medulloblastoma using paediatric protocols.
  • Although the number of patients is limited, our data suggest that the sandwich sequential, moderately intensive chemotherapy in combination with HART is an effective treatment for medulloblastoma in adults, and this approach seems to overcome previously-recognised risk factors.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Adult. Chemotherapy, Adjuvant / methods. Cranial Irradiation / methods. Disease-Free Survival. Humans. Infusions, Intravenous. Lomustine / administration & dosage. Lomustine / adverse effects. Methotrexate / administration & dosage. Methotrexate / adverse effects. Middle Aged. Neoplasm Recurrence, Local / etiology. Neoplasm Recurrence, Local / mortality. Retrospective Studies. Treatment Outcome. Vincristine / administration & dosage. Vincristine / adverse effects

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  • (PMID = 15869875.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine; YL5FZ2Y5U1 / Methotrexate
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53. Yong RL, Kavanagh EC, Fenton D, Dorovini-Zis K, Heran MK, Haw CS: Midline cerebellar medulloblastoma in a seventy-one-year-old patient. Can J Neurol Sci; 2006 Feb;33(1):101-4
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  • [Title] Midline cerebellar medulloblastoma in a seventy-one-year-old patient.
  • BACKGROUND: Medulloblastoma is the most common malignant central nervous system tumour in children but, in contrast, quite rare in adults.
  • Hemispheric, rather than midline, cerebellar medulloblastomas are more common in older children and adults.
  • We present the unusual case of a 71-year-old man who presented with a fourth ventricular mass that proved to be a medulloblastoma.
  • A CT scan of the brain revealed a hyperattenuating, partially calcified, avidly enhancing mass within the fourth ventricle.
  • These findings were consistent with a classical medulloblastoma.
  • CONCLUSION: Adult medulloblastoma should be considered in the differential diagnosis of a partially calcified hyperattenuating mass within the fourth ventricle.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Cerebral Ventricle Neoplasms / pathology. Medulloblastoma / pathology
  • [MeSH-minor] Aged. Brain Neoplasms / pathology. Diagnosis, Differential. Diffusion Magnetic Resonance Imaging. Fourth Ventricle / pathology. Humans. Immunohistochemistry. Male. Microscopy, Electron, Transmission. Phosphopyruvate Hydratase / metabolism. Synaptophysin / metabolism. Tomography, X-Ray Computed

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  • (PMID = 16583731.001).
  • [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
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Synaptophysin; EC 4.2.1.11 / Phosphopyruvate Hydratase
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54. Langevin AM, Bernstein M, Kuhn JG, Blaney SM, Ivy P, Sun J, Chen Z, Adamson PC, Children's Oncology Group: A phase II trial of rebeccamycin analogue (NSC #655649) in children with solid tumors: a Children's Oncology Group study. Pediatr Blood Cancer; 2008 Mar;50(3):577-80
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  • BACKGROUND: Rebeccamycin Analogue (NSC #655649), a chemically synthesized glycosyl-dichloro-indolocarbazole derivative of rebeccamycin with topoisomerase inhibiting activity, has in vitro activity against pediatric tumor cell lines and tumor specimens including rhabdomyosarcoma, neuroblastoma, Ewing's sarcoma and medulloblastoma.
  • PROCEDURE: The primary objective of this trial was to determine the response rate to Rebeccamycin analogue NSC #655649 in children with refractory solid and CNS tumors.
  • With a global response rate of 3% observed in children with relapsed CNS and non-CNS solid tumors, further development of Rebeccamycin analogue in pediatric solid tumors is not recommended.
  • [MeSH-minor] Adolescent. Adult. Bone Marrow Diseases / chemically induced. Carbazoles. Central Nervous System Neoplasms / drug therapy. Child. Child, Preschool. Drug-Induced Liver Injury / etiology. Female. Glucosides. Humans. Infant. Infant, Newborn. Infusions, Intravenous. Male. Neoplasm Proteins / antagonists & inhibitors. Pancreatitis / chemically induced. Rhabdomyosarcoma / drug therapy. Salvage Therapy. Topoisomerase II Inhibitors

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17610262.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA98543; United States / NCI NIH HHS / CA / P30CA-54174
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Aminoglycosides; 0 / Antibiotics, Antineoplastic; 0 / Carbazoles; 0 / Glucosides; 0 / Neoplasm Proteins; 0 / Topoisomerase II Inhibitors; A60X6MBU6G / becatecarin
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55. Schiebel P, Stippich C, Unterberg A: [Adult medulloblastoma]. Rofo; 2010 Sep;182(9):808-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Adult medulloblastoma].
  • [Transliterated title] Adultes Medulloblastom.
  • [MeSH-major] Cerebellar Neoplasms / diagnosis. Diffusion Magnetic Resonance Imaging. Image Processing, Computer-Assisted. Magnetic Resonance Imaging. Medulloblastoma / diagnosis
  • [MeSH-minor] Adult. Cerebellum / pathology. Cerebellum / surgery. Contrast Media / administration & dosage. Craniotomy. Diagnosis, Differential. Humans. Male

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  • (PMID = 20544581.001).
  • [ISSN] 1438-9010
  • [Journal-full-title] RöFo : Fortschritte auf dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
  • [ISO-abbreviation] Rofo
  • [Language] ger
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Contrast Media
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56. Infante JR, Rayo JI, Serrano J, Domínguez ML, García L, Sánchez R: Adult medulloblastoma relapse visualized by in-111 octreotide scintigraphy. Clin Nucl Med; 2006 Oct;31(10):633-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adult medulloblastoma relapse visualized by in-111 octreotide scintigraphy.
  • [MeSH-major] Cerebellar Neoplasms / radionuclide imaging. Medulloblastoma / radionuclide imaging. Neoplasm Recurrence, Local / radionuclide imaging. Octreotide / analogs & derivatives. Pentetic Acid / analogs & derivatives
  • [MeSH-minor] Adult. Female. Humans. Radiopharmaceuticals

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  • (PMID = 16985373.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 111In-octreotide, DTPA(0)-; 0 / Radiopharmaceuticals; 7A314HQM0I / Pentetic Acid; RWM8CCW8GP / Octreotide
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57. Franceschi E, Tosoni A, Paioli A, Cavallo G, Spagnolli F, Brandes AA: Challenges and progress in the treatment of adult medulloblastomas. Future Oncol; 2007 Apr;3(2):115-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Challenges and progress in the treatment of adult medulloblastomas.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Medulloblastoma / therapy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant / trends. Humans. Risk Factors

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  • (PMID = 17381408.001).
  • [ISSN] 1479-6694
  • [Journal-full-title] Future oncology (London, England)
  • [ISO-abbreviation] Future Oncol
  • [Language] eng
  • [Publication-type] Editorial
  • [Publication-country] England
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58. Cakar M, Aksoy S, Kilickap S, Harputluoglu H, Erman M: Procarbazine, lomustine, vincristine combination may be effective in adult medulloblastoma patients with systemic metastases. J Neurooncol; 2005 Nov;75(2):233-4
Hazardous Substances Data Bank. PROCARBAZINE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Procarbazine, lomustine, vincristine combination may be effective in adult medulloblastoma patients with systemic metastases.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / secondary. Lomustine / therapeutic use. Lymphatic Metastasis / pathology. Medulloblastoma / drug therapy. Procarbazine / therapeutic use. Vincristine / therapeutic use
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Antineoplastic Agents, Alkylating / therapeutic use. Antineoplastic Agents, Phytogenic / therapeutic use. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Time Factors. Tomography, X-Ray Computed. Treatment Outcome

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  • [Cites] Med Pediatr Oncol. 2003 Jun;40(6):396-7 [12692812.001]
  • [Cites] J Neurol Neurosurg Psychiatry. 1991 Jan;54(1):80-6 [2010766.001]
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  • (PMID = 16132496.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Alkylating; 0 / Antineoplastic Agents, Phytogenic; 35S93Y190K / Procarbazine; 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine
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