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1. McCabe MG, Ichimura K, Liu L, Plant K, Bäcklund LM, Pearson DM, Collins VP: High-resolution array-based comparative genomic hybridization of medulloblastomas and supratentorial primitive neuroectodermal tumors. J Neuropathol Exp Neurol; 2006 Jun;65(6):549-61
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  • [Title] High-resolution array-based comparative genomic hybridization of medulloblastomas and supratentorial primitive neuroectodermal tumors.
  • Medulloblastomas and supratentorial primitive neuroectodermal tumors are aggressive childhood tumors.
  • We report our findings using array comparative genomic hybridization (CGH) on a whole-genome BAC/PAC/cosmid array with a median clone separation of 0.97 Mb to study 34 medulloblastomas and 7 supratentorial primitive neuroectodermal tumors.
  • In addition, one supratentorial primitive neuroectodermal tumor had lost both copies of the tumor-suppressor genes CDKN2A and CDKN2B.
  • Significant differences were found in the patterns of copy number change between medulloblastomas and supratentorial primitive neuroectodermal tumors, providing further evidence that these tumors are genetically distinct despite their morphologic and behavioral similarities.
  • [MeSH-major] Brain Neoplasms / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Nucleic Acid Hybridization / methods. Supratentorial Neoplasms / genetics
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Chromosome Aberrations. Gene Expression Profiling. Humans. In Situ Hybridization, Fluorescence / methods. Infant. Reverse Transcriptase Polymerase Chain Reaction / methods

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  • (PMID = 16783165.001).
  • [ISSN] 0022-3069
  • [Journal-full-title] Journal of neuropathology and experimental neurology
  • [ISO-abbreviation] J. Neuropathol. Exp. Neurol.
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / / A6618
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2816352; NLM/ UKMS2695
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2. Qiu SJ, Guo YL, Zhang XL, Hu BS, Zhang YZ, Wen G: [High-field MRI and pathological diagnosis of supratentorial primitive neuroectodermal tumors]. Nan Fang Yi Ke Da Xue Xue Bao; 2007 Jun;27(6):863-5
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  • [Title] [High-field MRI and pathological diagnosis of supratentorial primitive neuroectodermal tumors].
  • OBJECTIVE: To analyze the magnetic resonance imaging (MRI) features of supratentorial primitive neuroectodermal tumors (sPNET) and improve the diagnosis of this disease.
  • RESULTS: The supratentorial lesions involved the occipital lobe in 4, frontal lobe in 3, fronto-occipital lobe in 2, temporo-occipital lobe in 3, lateral ventricle in 1 case and the saddle region in 1.
  • Pathologically, 10 cases of neuroblastomas were identified, along with 3 ganglioneuroblastomas and 1 atypical rhabdoid tumor.
  • [MeSH-major] Magnetic Resonance Imaging / methods. Neuroectodermal Tumors, Primitive / radiography. Supratentorial Neoplasms / radiography
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Female. Humans. Male. Middle Aged. Retrospective Studies. Sensitivity and Specificity. Young Adult

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  • (PMID = 17584656.001).
  • [ISSN] 1673-4254
  • [Journal-full-title] Nan fang yi ke da xue xue bao = Journal of Southern Medical University
  • [ISO-abbreviation] Nan Fang Yi Ke Da Xue Xue Bao
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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3. Chen AY, Lee H, Hartman J, Greco C, Ryu JK, O'Donnell R, Boggan J: Secondary supratentorial primitive neuroectodermal tumor following irradiation in a patient with low-grade astrocytoma. AJNR Am J Neuroradiol; 2005 Jan;26(1):160-2
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  • [Title] Secondary supratentorial primitive neuroectodermal tumor following irradiation in a patient with low-grade astrocytoma.
  • We report a case of a supratentorial primitive neuroectodermal tumor (PNET) that occurred 12 years after cranial irradiation for a grade II astrocytoma.
  • [MeSH-major] Astrocytoma / radiotherapy. Brain Neoplasms / radiotherapy. Cranial Irradiation. Frontal Lobe. Image Processing, Computer-Assisted. Magnetic Resonance Imaging. Neoplasms, Radiation-Induced / diagnosis. Neoplasms, Second Primary / diagnosis. Neuroectodermal Tumors, Primitive / diagnosis. Supratentorial Neoplasms / diagnosis. Tomography, X-Ray Computed
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Female. Follow-Up Studies. Glial Fibrillary Acidic Protein / analysis. Humans. Radiotherapy, Adjuvant. Synaptophysin / analysis


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4. Butturini AM, Jacob M, Aguajo J, Vander-Walde NA, Villablanca J, Jubran R, Erdreich-Epstein A, Marachelian A, Dhall G, Finlay JL: High-dose chemotherapy and autologous hematopoietic progenitor cell rescue in children with recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors: the impact of prior radiotherapy on outcome. Cancer; 2009 Jul 1;115(13):2956-63
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  • [Title] High-dose chemotherapy and autologous hematopoietic progenitor cell rescue in children with recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors: the impact of prior radiotherapy on outcome.
  • BACKGROUND: The role of myeloablative chemotherapy in children with recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors (MB/ST-PNET) is controversial, in particular in patients who develop recurrent disease after craniospinal radiotherapy.
  • Nine previously irradiated children experienced 4 toxic deaths and 6 tumor recurrences (1 patient had both): An interval of <1 year between initial radiotherapy and myeloablative chemotherapy predicted a greater risk of toxic death (P = .02), whereas a history of meningeal metastases at diagnosis and a poor response to the initial rescue therapy predicted a greater risk of post-transplant recurrence (P = .03 and P = .08, respectively).
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cranial Irradiation. Hematopoietic Stem Cell Transplantation. Medulloblastoma / therapy. Neuroectodermal Tumors, Primitive / therapy. Supratentorial Neoplasms / therapy. Thiotepa / administration & dosage
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Infant. Male. Neoplasm Recurrence, Local. Survival Rate

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  • (PMID = 19402050.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 905Z5W3GKH / Thiotepa
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5. Chang Q, Ng HK: [Different hypermethylation status of RASSF1A in medulloblastoma and supratentorial primitive neuroectodermal tumor]. Zhonghua Bing Li Xue Za Zhi; 2007 Jan;36(1):24-8
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  • [Title] [Different hypermethylation status of RASSF1A in medulloblastoma and supratentorial primitive neuroectodermal tumor].
  • OBJECTIVE: To investigate the epigenetic involvement of RASSF1A in intracranial primitive neuroectodermal tumors (PNETs) and compare the methylation patterns between medulloblastoma (MBs) and supratentorial PNETs (SPNETs).
  • These results demonstrated that such epigenetic alteration was tumor-specific.
  • [MeSH-major] Brain Neoplasms / genetics. Cerebellar Neoplasms / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Cell Line, Tumor. Child. Child, Preschool. DNA Methylation. Female. Gene Expression Regulation, Neoplastic. Gene Silencing. HeLa Cells. Humans. Infant. Male. Promoter Regions, Genetic / genetics. Reverse Transcriptase Polymerase Chain Reaction. Young Adult


6. Ulbright TM, Hattab EM, Zhang S, Ehrlich Y, Foster RS, Einhorn LH, Cheng L: Primitive neuroectodermal tumors in patients with testicular germ cell tumors usually resemble pediatric-type central nervous system embryonal neoplasms and lack chromosome 22 rearrangements. Mod Pathol; 2010 Jul;23(7):972-80
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  • [Title] Primitive neuroectodermal tumors in patients with testicular germ cell tumors usually resemble pediatric-type central nervous system embryonal neoplasms and lack chromosome 22 rearrangements.
  • Primitive neuroectodermal tumors (PNETs) are one of the most frequent types of 'non-germ cell' tumor in patients with testicular germ cell tumors and have a guarded prognosis when present in metastatic sites after cisplatin-based chemotherapy.
  • Using standard light microscopic criteria for central nervous system and peripheral PNETs, we classified nine tumors as medulloepithelioma, three as medulloblastoma/supratentorial PNET, one as neuroblastic tumor with abundant neuropil and true rosettes and one as small cell embryonal tumor/PNET (Ewing sarcoma-like).
  • Only 1 tumor, classified as medulloepithelioma, was scored positive for chromosome 22 translocation (22% rearranged cells) and the remaining 13 were negative, including the one case that resembled peripheral PNET.
  • [MeSH-major] Neoplasms, Germ Cell and Embryonal / pathology. Neoplasms, Multiple Primary / pathology. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Testicular Neoplasms / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Chromosomes, Human, Pair 22 / genetics. Gene Rearrangement. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. Young Adult

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  • (PMID = 20348883.001).
  • [ISSN] 1530-0285
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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7. Kouyialis AT, Boviatsis EI, Karampelas IK, Korfias S, Korkolopoulou P, Sakas DE: Primitive supratentorial neuroectodermal tumor in an adult. J Clin Neurosci; 2005 May;12(4):492-5
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  • [Title] Primitive supratentorial neuroectodermal tumor in an adult.
  • We report the case of a 32-year-old female with a diagnosis of supratentorial tumour.
  • Histopathology revealed a primitive neuroectodermal tumour (PNET), an unusual and highly malignant, mainly infratentorial tumour of childhood that is uncommonly described in the supratentorial compartment of adults.
  • [MeSH-major] Brain Neoplasms / pathology. Brain Neoplasms / surgery. Neuroectodermal Tumors, Primitive / pathology. Supratentorial Neoplasms
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging / methods. Neuroglia / pathology. Review Literature as Topic

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  • (PMID = 15925794.001).
  • [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
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8. Nordfors K, Haapasalo J, Korja M, Niemelä A, Laine J, Parkkila AK, Pastorekova S, Pastorek J, Waheed A, Sly WS, Parkkila S, Haapasalo H: The tumour-associated carbonic anhydrases CA II, CA IX and CA XII in a group of medulloblastomas and supratentorial primitive neuroectodermal tumours: an association of CA IX with poor prognosis. BMC Cancer; 2010;10:148
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  • [Title] The tumour-associated carbonic anhydrases CA II, CA IX and CA XII in a group of medulloblastomas and supratentorial primitive neuroectodermal tumours: an association of CA IX with poor prognosis.
  • BACKGROUND: Medulloblastomas (MBs) and supratentorial primitive neuroectodermal tumours (PNETs) are the most common highly aggressive paediatric brain tumours.
  • [MeSH-major] Antigens, Neoplasm / analysis. Biomarkers, Tumor / analysis. Carbonic Anhydrase II / analysis. Carbonic Anhydrases / analysis. Cerebellar Neoplasms / enzymology. Medulloblastoma / enzymology. Neuroectodermal Tumors, Primitive / enzymology. Supratentorial Neoplasms / enzymology
  • [MeSH-minor] Adolescent. Adult. Aged. Apoptosis. Chi-Square Distribution. Child. Child, Preschool. Cytoplasm / enzymology. Endothelial Cells / enzymology. Female. Finland. Humans. Immunohistochemistry. Infant. Infant, Newborn. Kaplan-Meier Estimate. Male. Middle Aged. Odds Ratio. Proportional Hazards Models. Time Factors. Treatment Outcome. Young Adult

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  • (PMID = 20398423.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; EC 4.2.1.- / Carbonic Anhydrase II; EC 4.2.1.1 / CA9 protein, human; EC 4.2.1.1 / Carbonic Anhydrases; EC 4.2.1.1 / carbonic anhydrase XII
  • [Other-IDs] NLM/ PMC2874782
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9. Pang JC, Chang Q, Chung YF, Teo JG, Poon WS, Zhou LF, Kong X, Ng HK: Epigenetic inactivation of DLC-1 in supratentorial primitive neuroectodermal tumor. Hum Pathol; 2005 Jan;36(1):36-43
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  • [Title] Epigenetic inactivation of DLC-1 in supratentorial primitive neuroectodermal tumor.
  • Supratentorial primitive neuroectodermal tumors (SPNETs) and medulloblastomas (MBs) are histologically similar intracranial tumors found in different anatomic locations of the brain.
  • The aim of this study was to evaluate whether DLC-1, a newly identified tumor-suppressor gene on chromosome 8p22, is involved in the tumorigenesis of MBs and the histologically similar SPNETs.
  • [MeSH-major] Epigenesis, Genetic. Gene Silencing. Neuroectodermal Tumors, Primitive / genetics. Supratentorial Neoplasms / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Base Sequence. Cell Line, Tumor. Cerebellar Neoplasms / genetics. Child. Child, Preschool. Chromosomes, Human, Pair 8 / genetics. CpG Islands. DNA Methylation. Female. GTPase-Activating Proteins. Gene Expression Regulation, Neoplastic. Humans. Infant. Lasers. Loss of Heterozygosity. Male. Medulloblastoma / genetics. Microdissection. Molecular Sequence Data. Promoter Regions, Genetic. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 15712180.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DLC1 protein, human; 0 / GTPase-Activating Proteins; 0 / Tumor Suppressor Proteins
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10. Terterov S, Krieger MD, Bowen I, McComb JG: Evaluation of intracranial cerebrospinal fluid cytology in staging pediatric medulloblastomas, supratentorial primitive neuroectodermal tumors, and ependymomas. J Neurosurg Pediatr; 2010 Aug;6(2):131-6
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  • [Title] Evaluation of intracranial cerebrospinal fluid cytology in staging pediatric medulloblastomas, supratentorial primitive neuroectodermal tumors, and ependymomas.
  • OBJECT: The objective of this study was to determine the role of intracranial CSF examination in detecting true cases of early tumor dissemination.
  • Cerebrospinal fluid dissemination is an ominous feature of pediatric brain tumors, occurring in as many as 30% of medulloblastomas, 25% of supratentorial primitive neuroectodermal tumors (PNETs), and 5% of ependymomas at diagnosis.
  • METHODS: Under an institutional review board-approved protocol, medical records, pathology reports, and radiology reports for 150 patients who had undergone resection of brain tumors (88 with medulloblastomas, 21 with supratentorial PNETs, and 41 with ependymomas) and who had been evaluated using neuraxis MR imaging studies in the last 15 years were retrospectively reviewed.
  • CONCLUSIONS: Discordance exists between the results of neuraxis MR imaging and lumbar and intracranial CSF cytology in perioperative detection of tumor dissemination for pediatric medulloblastoma, supratentorial PNETs, and ependymoma.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Cerebrospinal Fluid / cytology. Ependymoma / pathology. Medulloblastoma / pathology. Neuroectodermal Tumors, Primitive / pathology. Supratentorial Neoplasms / pathology
  • [MeSH-minor] Adolescent. Brain / pathology. Brain / surgery. Cerebellum / pathology. Cerebellum / surgery. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Infant. Kaplan-Meier Estimate. Magnetic Resonance Imaging. Male. Neoplasm Staging. Predictive Value of Tests. Radiotherapy, Adjuvant. Survival Rate. Young Adult

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  • (PMID = 20672933.001).
  • [ISSN] 1933-0715
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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11. Ohba S, Yoshida K, Hirose Y, Ikeda E, Kawase T: A supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature. J Neurooncol; 2008 Jan;86(2):217-24
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  • [Title] A supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature.
  • Supratentorial primitive neuroectodermal tumors (sPNET) occurring in adults are rare.
  • This case was the first case to detect a central PNET using both immunohistochemistry and the FISH assay in adult sPNET.
  • Though radiation therapy was performed, an MRI performed 2.5 months after the surgery revealed a regrowth of the tumor.
  • This case report is accompanied by a review of 57 cases of adult sPNET.
  • [MeSH-major] Neuroectodermal Tumors, Primitive / pathology. Supratentorial Neoplasms / pathology

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  • (PMID = 17713720.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules
  • [Number-of-references] 32
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12. Krampulz T, Hans VH, Oppel F, Dietrich U, Puchner MJ: Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult: a case report. J Neurooncol; 2006 May;77(3):291-4
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  • [Title] Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult: a case report.
  • OBJECTIVE: In adults, supratentorial primitive neuroectodermal tumor (sPNET) is a very rare undifferentiated embryoblastic neoplasm.
  • Survival longer than 15 years in an adult has only been reported once so far.
  • CT- and MRI-scans revealed a right occipital tumor with moderate contrast enhancement.
  • The tumor was completely removed.
  • Microscopy revealed a malignant, highly cellular, poorly differentiated tumor with a desmoplastic component.
  • Up to 20% of tumor nuclei were labeled for Ki-67.
  • Other tumor entities were excluded by immunohistochemistry.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Neuroectodermal Tumors, Primitive / pathology. Sarcoma / pathology. Supratentorial Neoplasms / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Diagnosis, Differential. Disease-Free Survival. Humans. Ki-67 Antigen / metabolism. Male. Receptor, Nerve Growth Factor / metabolism. Treatment Outcome

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  • (PMID = 16528456.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; 0 / Receptor, Nerve Growth Factor
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13. Phi JH, Kim JH, Eun KM, Wang KC, Park KH, Choi SA, Kim YY, Park SH, Cho BK, Kim SK: Upregulation of SOX2, NOTCH1, and ID1 in supratentorial primitive neuroectodermal tumors: a distinct differentiation pattern from that of medulloblastomas. J Neurosurg Pediatr; 2010 Jun;5(6):608-14
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Upregulation of SOX2, NOTCH1, and ID1 in supratentorial primitive neuroectodermal tumors: a distinct differentiation pattern from that of medulloblastomas.
  • OBJECT: Supratentorial primitive neuroectodermal tumor (PNET) and medulloblastoma are highly malignant embryonal brain tumors.
  • The authors compared the expression of specific genes involved in neuroglial differentiation in supratentorial PNETs and medulloblastomas to define the distinct characters of these tumors.
  • METHODS: The mRNA expression of 8 genes (SOX2, NOTCH1, ID1, ASCL-1, NEUROD1, NEUROG1, NEUROG2, and NRG1) was evaluated in 25 embryonal tumors (12 supratentorial PNETs and 13 medulloblastomas) by quantitative real-time polymerase chain reaction.
  • The expression levels of the transcripts of these genes were compared between the tumor groups.
  • RESULTS: Supratentorial PNETs expressed significantly higher levels of SOX2, NOTCH1, ID1, and ASCL-1 transcripts, whereas the transcription of proneural basic helix-loop-helix factors, NEUROD1, NEUROG1 (significantly), and NEUROG2 (not significantly) was upregulated in medulloblastomas.
  • The proportion of phosphorylated STAT3alpha relative to STAT3alpha was significantly greater in supratentorial PNETs than in medulloblastomas, indicating activation of the JAK/STAT3 pathway in supratentorial PNETs.
  • CONCLUSIONS: These results indicate that supratentorial PNET predominantly has glial features and medulloblastoma largely follows a neuronal differentiation pattern.
  • These divergent differentiation patterns may be related to the location and origin of each tumor.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Inhibitor of Differentiation Protein 1 / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Receptor, Notch1 / genetics. SOXB1 Transcription Factors / genetics. STAT3 Transcription Factor / genetics. Supratentorial Neoplasms / genetics. Up-Regulation / genetics
  • [MeSH-minor] Adolescent. Adult. Cerebellum / pathology. Cerebral Cortex / pathology. Child. Child, Preschool. Diagnosis, Differential. Female. Gene Expression Regulation, Neoplastic / genetics. Humans. Infant. Infant, Newborn. Male. Neuroglia / pathology. Neurons / pathology. RNA, Messenger / genetics. Reverse Transcriptase Polymerase Chain Reaction. Transcription, Genetic / genetics

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  • (PMID = 20515335.001).
  • [ISSN] 1933-0715
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ID1 protein, human; 0 / Inhibitor of Differentiation Protein 1; 0 / NOTCH1 protein, human; 0 / RNA, Messenger; 0 / Receptor, Notch1; 0 / SOX2 protein, human; 0 / SOXB1 Transcription Factors; 0 / STAT3 Transcription Factor; 0 / STAT3 protein, human
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14. Asano K, Kikuchi J, Munakata A, Ohkuma H, Kubo O: An infant case of intracranial peripheral-type primitive neuroectodermal tumor with long-term survival. Brain Tumor Pathol; 2007;24(2):69-74
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  • [Title] An infant case of intracranial peripheral-type primitive neuroectodermal tumor with long-term survival.
  • Supratentorial primitive neuroectodermal tumors (S-PNET) that develop in children have recently been classified into two types: central-type PNET (C-PNET), which has been reported over the years, and peripheral-type PNET (P-PNET), which develops intracranially and was referred to as Ewing's sarcoma in the past.
  • P-PNET is fundamentally a malignant tumor, but the patient reported here represents a case of long-term survival from onset without recurrence.
  • A CT scan revealed a cystic tumor attaching to the falx, and cyst drainage operation was immediately performed.
  • The intracranial tumor was then resected.
  • The tumor was an intradural extramedullary tumor, and it was totally excised with the falx attachment.
  • The tumor was initially diagnosed as a neuroblastoma, and postoperative treatment consisted of administration of radiotherapy and chemotherapy using cyclophosphamide and vincristine.
  • That is, in spite of the fact that P-PNET is a malignant tumor, patient survival can be comparatively long.
  • Because P-PNET originates intracranially, it is fundamentally an intradural extramedullary tumor.
  • [MeSH-major] Diagnostic Errors. Neuroblastoma / pathology. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Supratentorial Neoplasms / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Humans. Immunohistochemistry. Infant. Magnetic Resonance Imaging. Male. Neurosurgical Procedures. Radiotherapy. Tomography, X-Ray Computed

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  • (PMID = 18095134.001).
  • [ISSN] 1433-7398
  • [Journal-full-title] Brain tumor pathology
  • [ISO-abbreviation] Brain Tumor Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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15. Inda MM, Castresana JS: RASSF1A promoter is highly methylated in primitive neuroectodermal tumors of the central nervous system. Neuropathology; 2007 Aug;27(4):341-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] RASSF1A promoter is highly methylated in primitive neuroectodermal tumors of the central nervous system.
  • Primitive neuroectodermal tumors (PNET) of the central nervous system can be divided into infratentorial PNET or medulloblastoma (MB), and supratentorial (sPNET) tumors.
  • The RASSF1A (Ras Association Domain Family Protein 1) gene, located at 3p21.3, is highly methylated in multiple primary tumor samples, including neuroblastoma.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Promoter Regions, Genetic. Supratentorial Neoplasms / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. DNA Methylation. Female. Humans. Male. Middle Aged. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 17899687.001).
  • [ISSN] 0919-6544
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / RASSF1 protein, human; 0 / Tumor Suppressor Proteins
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16. Kagawa N, Maruno M, Suzuki T, Hashiba T, Hashimoto N, Izumoto S, Yoshimine T: Detection of genetic and chromosomal aberrations in medulloblastomas and primitive neuroectodermal tumors with DNA microarrays. Brain Tumor Pathol; 2006 Apr;23(1):41-7
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  • [Title] Detection of genetic and chromosomal aberrations in medulloblastomas and primitive neuroectodermal tumors with DNA microarrays.
  • Medulloblastoma (MB) is the most frequent infratentorial malignant brain tumor in children.
  • In contrast, primitive neuroectodermal tumor (PNET) is defined as a supratentorial malignant tumor generated from the cerebral hemisphere.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Cerebellar Neoplasms / pathology. Chromosome Aberrations. DNA, Neoplasm / genetics. Medulloblastoma / genetics. Medulloblastoma / pathology. Neuroectodermal Tumors, Primitive / genetics. Neuroectodermal Tumors, Primitive / pathology
  • [MeSH-minor] Biomarkers, Tumor. Cerebellum / pathology. Child. Child, Preschool. Female. Humans. In Situ Hybridization. Infant. Male. Microarray Analysis. Young Adult


17. Jeans AF, Frayling I, Jasani B, Side L, Blesing C, Ansorge O: Cerebral primitive neuroectodermal tumor in an adult with a heterozygous MSH2 mutation. Nat Rev Clin Oncol; 2009 May;6(5):295-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cerebral primitive neuroectodermal tumor in an adult with a heterozygous MSH2 mutation.
  • BACKGROUND: A 37-year-old woman presented with a supratentorial cerebral mass, which was diagnosed histologically as a primitive neuroectodermal tumor.
  • MANAGEMENT: Debulking of the cerebral tumor, craniospinal axis radiotherapy, and genetic counseling of family.
  • [MeSH-major] Colorectal Neoplasms, Hereditary Nonpolyposis / genetics. Heterozygote. MutS Homolog 2 Protein / genetics. Mutation. Neuroectodermal Tumors, Primitive / genetics
  • [MeSH-minor] Adult. DNA Mismatch Repair / genetics. DNA Mutational Analysis. Fatal Outcome. Female. Germ-Line Mutation. Humans. Immunohistochemistry. Pedigree. Sequence Analysis, DNA

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  • (PMID = 19390556.001).
  • [ISSN] 1759-4782
  • [Journal-full-title] Nature reviews. Clinical oncology
  • [ISO-abbreviation] Nat Rev Clin Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] EC 3.6.1.3 / MutS Homolog 2 Protein
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18. Chang Q, Pang JC, Li KK, Poon WS, Zhou L, Ng HK: Promoter hypermethylation profile of RASSF1A, FHIT, and sFRP1 in intracranial primitive neuroectodermal tumors. Hum Pathol; 2005 Dec;36(12):1265-72
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  • [Title] Promoter hypermethylation profile of RASSF1A, FHIT, and sFRP1 in intracranial primitive neuroectodermal tumors.
  • Medulloblastomas (MBs) and supratentorial primitive neuroectodermal tumors (SPNETs) are histologically alike intracranial PNETs found in different anatomical locations of the brain.
  • The aim of this study was to investigate whether promoter hypermethylation of putative tumor suppressor genes was involved in both types of intracranial PNETs.
  • [MeSH-major] Acid Anhydride Hydrolases / genetics. Brain Neoplasms / genetics. Cell Cycle Proteins / genetics. DNA Methylation. Neoplasm Proteins / genetics. Neuroectodermal Tumors, Primitive / genetics. Protein-Serine-Threonine Kinases / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Ataxia Telangiectasia Mutated Proteins. Cell Line, Tumor. Child. Child, Preschool. DNA Primers / chemistry. DNA, Neoplasm / analysis. Female. Humans. Infant. Male. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 16311119.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cell Cycle Proteins; 0 / DNA Primers; 0 / DNA, Neoplasm; 0 / Neoplasm Proteins; 0 / RASSF1 protein, human; 0 / Tumor Suppressor Proteins; 0 / fragile histidine triad protein; EC 2.7.11.1 / ATR protein, human; EC 2.7.11.1 / Ataxia Telangiectasia Mutated Proteins; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 3.6.- / Acid Anhydride Hydrolases
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19. Terheggen F, Troost D, Majoie CB, Leenstra S, Richel DJ: Local recurrence and distant metastasis of supratentorial primitive neuro-ectodermal tumor in an adult patient successfully treated with intensive induction chemotherapy and maintenance temozolomide. J Neurooncol; 2007 Mar;82(1):113-6
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  • [Title] Local recurrence and distant metastasis of supratentorial primitive neuro-ectodermal tumor in an adult patient successfully treated with intensive induction chemotherapy and maintenance temozolomide.
  • Supratentorial primitive neuro-ectodermal tumors (PNET) in adults are very rare.
  • We present a 26-year-old patient with local recurrence and distant metastasis of supratentorial PNET successfully treated with intensive induction chemotherapy and maintenance temozolomide.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow Neoplasms / secondary. Liver Neoplasms / secondary. Neoplasm Recurrence, Local / drug therapy. Neuroectodermal Tumors, Primitive / secondary. Supratentorial Neoplasms / pathology
  • [MeSH-minor] Adult. Cyclophosphamide / administration & dosage. Dacarbazine / administration & dosage. Dacarbazine / analogs & derivatives. Doxorubicin / administration & dosage. Humans. Male. Methotrexate / administration & dosage. Prednisone / administration & dosage. Remission Induction. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 16972187.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; 85622-93-1 / temozolomide; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone; YL5FZ2Y5U1 / Methotrexate; MEVAP protocol
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20. Stecher CW, Grønbaek K, Hasle H: A novel splice mutation in the TP53 gene associated with Leydig cell tumor and primitive neuroectodermal tumor. Pediatr Blood Cancer; 2008 Mar;50(3):701-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A novel splice mutation in the TP53 gene associated with Leydig cell tumor and primitive neuroectodermal tumor.
  • A 20-month-old boy presented with precocious puberty due to a Leydig cell tumor, and at the age of 6 years with a primitive neuroectodermal brain-tumor (PNET).
  • [MeSH-major] Genes, p53. Leydig Cell Tumor / genetics. Neoplasms, Multiple Primary / genetics. Neuroectodermal Tumors, Primitive / genetics. RNA Splice Sites / genetics. Supratentorial Neoplasms / genetics. Testicular Neoplasms / genetics
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Combined Modality Therapy. Cranial Irradiation. Exons / genetics. Fatal Outcome. Female. Humans. Infant, Newborn. Male. Orchiectomy. Pedigree. Radiosurgery

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17066464.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / RNA Splice Sites
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21. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • [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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22. 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.
  • 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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23. Eberhart CG, Chaudhry A, Daniel RW, Khaki L, Shah KV, Gravitt PE: Increased p53 immunopositivity in anaplastic medulloblastoma and supratentorial PNET is not caused by JC virus. BMC Cancer; 2005 Feb 17;5:19
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Increased p53 immunopositivity in anaplastic medulloblastoma and supratentorial PNET is not caused by JC virus.
  • METHODS: p53 protein levels were evaluated semi-quantitatively in 64 medulloblastomas, 3 atypical teratoid rhabdoid tumors (ATRT), and 8 supratentorial primitive neuroectodermal tumors (sPNET) using immunohistochemistry.
  • No JC virus was identified in the embryonal brain tumor samples, while an endogenous human retrovirus (ERV-3) was readily detected.

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  • (PMID = 15717928.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / K08 NS043279; United States / NINDS NIH HHS / NS / K08NS43279
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Tumor Suppressor Protein p53
  • [Other-IDs] NLM/ PMC554768
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24. 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
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Epigenetic repression of RASSF1A but not CASP8 in supratentorial PNET (sPNET) and atypical teratoid/rhabdoid tumors (AT/RT) of childhood.
  • 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.
  • 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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25. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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-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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26. Herrlinger U, Steinbrecher A, Rieger J, Hau P, Kortmann RD, Meyermann R, Schabet M, Bamberg M, Dichgans J, Bogdahn U, Weller M: Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse. J Neurol; 2005 Mar;252(3):291-9
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  • [Title] Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse.
  • Adult medulloblastoma is a rare tumor with few retrospective studies published so far.
  • This study reports therapy and outcome in all adult (>or=16 years old) medulloblastoma (n=34) and supratentorial primitive neuroectodermal tumor (PNET) patients (n=2) treated in 2 neuro-oncological centers between 1976 and 2002.
  • In conclusion, adjuvant chemotherapy may prolong survival in adult medulloblastoma patients.
  • [MeSH-minor] Adolescent. Adult. Aged. Analysis of Variance. Combined Modality Therapy. Demography. Disease Progression. Disease-Free Survival. Dose-Response Relationship, Radiation. Drug Therapy / methods. Female. Humans. Male. Middle Aged. Radiotherapy, High-Energy / methods. Recurrence. Regression Analysis. Retrospective Studies. Risk Factors. Time Factors. Treatment Outcome

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  • (PMID = 16189725.001).
  • [ISSN] 0340-5354
  • [Journal-full-title] Journal of neurology
  • [ISO-abbreviation] J. Neurol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] Germany
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27. Gutenberg A, Schulten HJ, Gunawan B, Ludwig HC, Brück W, Larsen J, Rohde V: CNS tumor 22 years after spinal neuroblastoma IV: diagnostic dilemma between recurrence and secondary malignancy. Pediatr Neurosurg; 2009;45(1):61-8
MedlinePlus Health Information. consumer health - Neuroblastoma.

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  • [Title] CNS tumor 22 years after spinal neuroblastoma IV: diagnostic dilemma between recurrence and secondary malignancy.
  • We present the very unusual case of a young woman suffering from a brain tumor 22 years after a stage IV spinal neuroblastoma as an infant, demonstrating the difficulties of differentiating late neuroblastoma relapse from secondary supratentorial primitive neuroectodermal tumor (sPNET).
  • Lacking specific immunohistochemical features, the first cerebral tumor at the age of 21 was regarded as sPNET, and we pursued a therapeutic approach consisting of neurosurgical resection as well as irradiation and high-dose alkylator-based chemotherapy according to the HIT2000 protocol.
  • [MeSH-minor] Adult. DNA, Neoplasm / genetics. Diagnosis, Differential. Female. Genetic Markers. Humans. Immunohistochemistry. Infant. Magnetic Resonance Imaging. Neoplasm Staging. Time Factors

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  • (PMID = 19258732.001).
  • [ISSN] 1423-0305
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Genetic Markers
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28. Lindsey JC, Lusher ME, Strathdee G, Brown R, Gilbertson RJ, Bailey S, Ellison DW, Clifford SC: Epigenetic inactivation of MCJ (DNAJD1) in malignant paediatric brain tumours. Int J Cancer; 2006 Jan 15;118(2):346-52
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • We examined the status of MCJ in intracranial primitive neuroectodermal tumours [PNETs, comprising cerebellar PNETs (medulloblastomas) and supratentorial PNETs (stPNETs)] and ependymomas, together representing the most common malignant brain tumours of childhood.
  • [MeSH-major] Brain Neoplasms / genetics. Ependymoma / genetics. Epigenesis, Genetic. HSP40 Heat-Shock Proteins / biosynthesis. Membrane Proteins / biosynthesis. Neuroectodermal Tumors, Primitive / genetics
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor. Child. Child, Preschool. DNA Methylation. Female. Gene Expression Profiling. Gene Silencing. Humans. Male. Reverse Transcriptase Polymerase Chain Reaction

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  • [Copyright] Copyright 2005 Wiley-Liss, Inc.
  • (PMID = 16049974.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNAJC1 protein, human; 0 / HSP40 Heat-Shock Proteins; 0 / Membrane Proteins
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29. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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.
  • [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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30. Gelabert-Gonzalez M, Serramito-García R, Arcos-Algaba A: Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature. Neurosurg Rev; 2010 Apr;34(2):151-8
MedlinePlus Health Information. consumer health - Childhood Brain Tumors.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • These tumors invariably arise in the supratentorial region and commonly involve more than one lobe, preferentially the temporal and frontal.
  • The histologic diagnosis is characterized by the presence of three different cell lines: astrocytic, neuronal, and primitive neuroectodermal marker sites, which were demonstrable.
  • Desmoplastic gangliogliomas represent a rare tumor group with two well-defined age groups, the children and non-children.
  • Surgery is the treatment of choice and no complementary treatment is needed in cases of complete tumor resection.
  • [MeSH-minor] Adolescent. Adult. Child, Preschool. Female. Humans. Immunohistochemistry. Infant. Infant, Newborn. Ki-67 Antigen / metabolism. Magnetic Resonance Imaging. Male. Microscopy, Electron. Neurosurgical Procedures. Skull / pathology. Supratentorial Neoplasms / pathology. Survival Analysis. Tomography, X-Ray Computed. Treatment Outcome


31. Wang FL, Li XH, Gui QP, Liu L: [Clinicopathologic and radiologic features of dysembryoplastic neuroepithelial tumors]. Zhonghua Bing Li Xue Za Zhi; 2005 Sep;34(9):566-8
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  • OBJECTIVE: To study the clinicopathologic features and radiologic findings of dysembryoplastic neuroepithelial tumor (DNT).
  • All tumors were located in the supratentorial cerebral cortex.
  • CONCLUSIONS: DNT is a benign tumor with excellent prognosis after surgical excision.
  • [MeSH-major] Brain Neoplasms / pathology. Cerebral Cortex / pathology. Neuroectodermal Tumors, Primitive / pathology
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Epilepsies, Partial / etiology. Epilepsies, Partial / metabolism. Epilepsies, Partial / pathology. Epilepsies, Partial / surgery. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neurofilament Proteins / metabolism. Oligodendroglia / pathology. Oligodendroglia / ultrastructure. S100 Proteins / metabolism. Survival Rate. Synaptophysin / metabolism

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  • (PMID = 16468306.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Neurofilament Proteins; 0 / S100 Proteins; 0 / Synaptophysin
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32. 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.
  • 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.
  • These results indicate that the antibody HFB-16 could be a useful marker for neuronal tumors and primitive neuroectodermal tumors that may originate from immature neural progenitor cells.
  • In addition, it could be a useful tool for performing the differential diagnosis between GBs and CNS supratentorial PNET.
  • [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] Netherlands
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Guanine Nucleotide Exchange Factors; 0 / RASGRP3 protein, human
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33. Phi JH, Park SH, Kim SK, Paek SH, Kim JH, Lee YJ, Cho BK, Park CK, Lee DH, Wang KC: Sox2 expression in brain tumors: a reflection of the neuroglial differentiation pathway. Am J Surg Pathol; 2008 Jan;32(1):103-12
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  • Sox2 immunohistochemistry was performed on 194 brain tumor tissues of various kinds.
  • Fetal and adult normal brain tissues obtained by autopsy and brain tissues of epilepsy patients with cortical dysplasia were used as controls.
  • In brain tumors of embryonal origin, supratentorial primitive neuroectodermal tumors showed robust Sox2 expression, whereas medulloblastomas and pineoblastomas did not.
  • The majority of Sox2-positive tumor cells coexpressed glial fibrillary acidic protein, and most Sox2-negative cells in medulloblastomas and pineoblastomas showed neuronal differentiation.
  • This study suggest that Sox2 may be a tumor marker of glial lineages rather than a universal brain tumor stem cell marker, because its expression pattern was found to correspond to differentiation pathways.
  • [MeSH-major] Biomarkers, Tumor / analysis. Brain Neoplasms / metabolism. HMGB Proteins / biosynthesis. Neuroglia / cytology. Neuroglia / metabolism. Transcription Factors / biosynthesis

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  • (PMID = 18162777.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Glial Fibrillary Acidic Protein; 0 / HMGB Proteins; 0 / RNA, Messenger; 0 / SOX2 protein, human; 0 / SOXB1 Transcription Factors; 0 / Transcription Factors
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34. Giunti L, Bernini G, Forni M, Tucci F, Wheeler E, Sardi I: Clonality analysis of pediatric multiple tumors: two case reports and laboratory investigation. J Pediatr Hematol Oncol; 2006 Apr;28(4):241-8
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  • We examined the possibility of using microsatellite and mitochondrial DNA polymorphisms as markers to detect the clonal origin of tumor cells found in the same patient.
  • We considered two children with complex tumor diseases: one with supratentorial primitive neuroectodermal tumors (PNET) and a hepatic rhabdoid tumor and another with brain and abdominal rhabdoid tumors.
  • In the first patient we found an mtDNA cytosine insertion both in the normal tissue and in the primary tumor, whereas in the hepatic tumor we detected an insertion of 2 cytosine.
  • In the second child, who had a constitutional mutation of hSNF5/INI-1, we identified the same mtDNA pattern both in normal tissue and in the abdominal tumor but not in the brain tumor, which presented three different mtDNA polymorphisms.
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Autopsy. Base Sequence. Child. DNA Primers. DNA, Mitochondrial / genetics. Fatal Outcome. Humans. Infant. Male. Polymerase Chain Reaction. Polymorphism, Genetic. Polymorphism, Single Nucleotide. Sequence Deletion

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  • (PMID = 16679923.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA Primers; 0 / DNA, Mitochondrial
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35. De Vos M, Hayward BE, Charlton R, Taylor GR, Glaser AW, Picton S, Cole TR, Maher ER, McKeown CM, Mann JR, Yates JR, Baralle D, Rankin J, Bonthron DT, Sheridan E: PMS2 mutations in childhood cancer. J Natl Cancer Inst; 2006 Mar 1;98(5):358-61
SciCrunch. OMIM: Data: Gene Annotation .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • This syndrome is characterized by café-au-lait skin pigmentation and a characteristic tumor spectrum, including leukemias, lymphomas, cerebral malignancies (such as supratentorial primitive neuroectodermal tumors, astrocytomas, and glioblastomas), and colorectal neoplasia with an onset in early adult life.
  • This cancer syndrome can be mistaken for neurofibromatosis type 1, with important management implications including the risk of the disorder occurring in siblings and the likelihood of tumor development in affected individuals.

  • Genetic Alliance. consumer health - Childhood Cancer.
  • MedlinePlus Health Information. consumer health - Cancer in Children.
  • COS Scholar Universe. author profiles.
  • Hazardous Substances Data Bank. (L)-ARGININE .
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  • (PMID = 16507833.001).
  • [ISSN] 1460-2105
  • [Journal-full-title] Journal of the National Cancer Institute
  • [ISO-abbreviation] J. Natl. Cancer Inst.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 94ZLA3W45F / Arginine; EC 3.6.1.- / Adenosine Triphosphatases; EC 3.6.1.- / PMS2 protein, human; EC 6.5.1.- / DNA Repair Enzymes
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36. Balss J, Meyer J, Mueller W, Korshunov A, Hartmann C, von Deimling A: Analysis of the IDH1 codon 132 mutation in brain tumors. Acta Neuropathol; 2008 Dec;116(6):597-602
The Lens. Cited by Patents in .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • We analyzed the genomic region spanning wild type R132 of IDH1 by direct sequencing in 685 brain tumors including 41 pilocytic astrocytomas, 12 subependymal giant cell astrocytomas, 7 pleomorphic xanthoastrocytomas, 93 diffuse astrocytomas, 120 adult glioblastomas, 14 pediatric glioblastomas, 105 oligodendrogliomas, 83 oligoastrocytomas, 31 ependymomas, 58 medulloblastomas, 9 supratentorial primitive neuroectodermal tumors, 17 schwannomas, 72 meningiomas and 23 pituitary adenomas.
  • The very high frequency of IDH1 mutations in WHO grade II astrocytic and oligodendroglial gliomas suggests a role in early tumor development.

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  • (PMID = 18985363.001).
  • [ISSN] 1432-0533
  • [Journal-full-title] Acta neuropathologica
  • [ISO-abbreviation] Acta Neuropathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] EC 1.1.1.41 / Isocitrate Dehydrogenase
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