primitive neuroectodermal tumors

Summary

Summary: A group of malignant tumors of the nervous system that feature primitive cells with elements of neuronal and/or glial differentiation. Use of this term is limited by some authors to central nervous system tumors and others include neoplasms of similar origin which arise extracranially (i.e., NEUROECTODERMAL TUMORS, PRIMITIVE, PERIPHERAL). This term is also occasionally used as a synonym for MEDULLOBLASTOMA. In general, these tumors arise in the first decade of life and tend to be highly malignant. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2059)

Top Publications

  1. ncbi Missense mutations in SMOH in sporadic basal cell carcinomas of the skin and primitive neuroectodermal tumors of the central nervous system
    J Reifenberger
    Hautklinik, Heinrich Heine Universitat, Dusseldorf, Germany
    Cancer Res 58:1798-803. 1998
  2. ncbi Molecular analysis of childhood primitive neuroectodermal tumors defines markers associated with poor outcome
    W G Scheurlen
    Children s Hospital, Mannheim, Germany
    J Clin Oncol 16:2478-85. 1998
  3. ncbi Centrosome amplification as a possible mechanism for numerical chromosome aberrations in cerebral primitive neuroectodermal tumors with TP53 mutations
    R G Weber
    Abteilung Organisation komplexer Genome, Deutsches Krebsforschungszentrum, Heidelberg, Germany
    Cytogenet Cell Genet 83:266-9. 1998
  4. ncbi Matrix metalloproteinase expression in childhood medulloblastomas/primitive neuroectodermal tumors
    B Bodey
    Department of Pathology, University of Southern California, Los Angeles, CA, USA
    In Vivo 14:667-73. 2000
  5. ncbi A role for fluorescence in situ hybridization detection of chromosome 22q dosage in distinguishing atypical teratoid/rhabdoid tumors from medulloblastoma/central primitive neuroectodermal tumors
    L A Bruch
    Department of Pathology (Neuropathology) and Anatomic Pathology, Washington University School of Medicine, St Louis, MO 63110, USA
    Hum Pathol 32:156-62. 2001
  6. ncbi Germ cell tumor associated primitive neuroectodermal tumors
    K N Ganjoo
    Division of Hematology-Oncology, Department of Medicine, Indiana University Medical Center, Indianapolis, Indiana, USA
    J Urol 165:1514-6. 2001
  7. ncbi Somatic mutations of WNT/wingless signaling pathway components in primitive neuroectodermal tumors
    A Koch
    Department of Neuropathology, University of Bonn Medical Center; Bonn, Germany
    Int J Cancer 93:445-9. 2001
  8. ncbi Intense p53 staining is a valuable prognostic indicator for poor prognosis in medulloblastoma/central nervous system primitive neuroectodermal tumors
    R T Woodburn
    Department of Radiation Oncology, Indiana University Medical Center, Indianapolis 46202, USA
    J Neurooncol 52:57-62. 2001
  9. ncbi Immunohistochemical detection of EWS and FLI-1 proteinss in Ewing sarcoma and primitive neuroectodermal tumors: comparative analysis with CD99 (MIC-2) expression
    A Llombart-Bosch
    Department of Pathology, Medical School, University of Valencia, Spain
    Appl Immunohistochem Mol Morphol 9:255-60. 2001
  10. pmc Medulloblastomas and primitive neuroectodermal tumors rarely contain polyomavirus DNA sequences
    John Y H Kim
    Division of Neuroscience, Department of Neurology, Children s Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA
    Neuro Oncol 4:165-70. 2002

Research Grants

  1. Inhibition of matrix proteases to sensitize medulloblastoma cells to radiation
    Andrei L Gartel; Fiscal Year: 2013
  2. Pesticide Exposure and Childhood Cancer Study
    Beate R Ritz; Fiscal Year: 2012
  3. Cellular plasticity and survival mechanisms in medulloblastoma stem cells
    Ronnie Yoo; Fiscal Year: 2013
  4. 'Exvading'Invasive Pediatric Brain Tumors
    Ravi V Bellamkonda; Fiscal Year: 2013
  5. Clinical pharmacology of cyclophosphamide in pediatric medulloblastoma patients
    Michael B Tagen; Fiscal Year: 2010
  6. Edward A Neuwelt; Fiscal Year: 2014
  7. Human Neurotropic JC Virus and CNS Diseases
    Kamel Khalili; Fiscal Year: 2006
  8. Academic Public Private Partnership Program
    M O DORISIO; Fiscal Year: 2004
  9. MOLECULAR ANALYSIS OF PEDIATRIC CANCERS
    Giovanni Rovera; Fiscal Year: 1993
  10. PRIMITIVE NEUROECTODERMAL TUMORS OF CHILDHOOD
    Peter Phillips; Fiscal Year: 2000

Detail Information

Publications221 found, 100 shown here

  1. ncbi Missense mutations in SMOH in sporadic basal cell carcinomas of the skin and primitive neuroectodermal tumors of the central nervous system
    J Reifenberger
    Hautklinik, Heinrich Heine Universitat, Dusseldorf, Germany
    Cancer Res 58:1798-803. 1998
    About one-third of sporadic basal cell carcinomas (BCCs) of the skin and 10-15% of primitive neuroectodermal tumors (PNETs) of the central nervous system show mutations in the PTCH tumor suppressor gene...
  2. ncbi Molecular analysis of childhood primitive neuroectodermal tumors defines markers associated with poor outcome
    W G Scheurlen
    Children s Hospital, Mannheim, Germany
    J Clin Oncol 16:2478-85. 1998
    The diagnostic and prognostic significance of well-defined molecular markers was investigated in childhood primitive neuroectodermal tumors (PNET).
  3. ncbi Centrosome amplification as a possible mechanism for numerical chromosome aberrations in cerebral primitive neuroectodermal tumors with TP53 mutations
    R G Weber
    Abteilung Organisation komplexer Genome, Deutsches Krebsforschungszentrum, Heidelberg, Germany
    Cytogenet Cell Genet 83:266-9. 1998
    ..1996). Here we report on cerebral primitive neuroectodermal tumors that lacked the wild-type p53 gene (TP53) and showed multiple numerical chromosome aberrations, as ..
  4. ncbi Matrix metalloproteinase expression in childhood medulloblastomas/primitive neuroectodermal tumors
    B Bodey
    Department of Pathology, University of Southern California, Los Angeles, CA, USA
    In Vivo 14:667-73. 2000
    ..Expression of MMP-2, -3, -9, -10, and -13 was investigated in human childhood medulloblastomas (MEDs)/primitive neuroectodermal tumors (PNETs) employing an indirect alkaline phosphatase conjugated immunohistochemical antigen detection ..
  5. ncbi A role for fluorescence in situ hybridization detection of chromosome 22q dosage in distinguishing atypical teratoid/rhabdoid tumors from medulloblastoma/central primitive neuroectodermal tumors
    L A Bruch
    Department of Pathology (Neuropathology) and Anatomic Pathology, Washington University School of Medicine, St Louis, MO 63110, USA
    Hum Pathol 32:156-62. 2001
    It has been postulated that infants with medulloblastomas/central primitive neuroectodermal tumors (MB/PNET) may fare worse than older patients because some of them harbor unrecognized atypical teratoid/rhabdoid tumors (AT/RT), rare ..
  6. ncbi Germ cell tumor associated primitive neuroectodermal tumors
    K N Ganjoo
    Division of Hematology-Oncology, Department of Medicine, Indiana University Medical Center, Indianapolis, Indiana, USA
    J Urol 165:1514-6. 2001
    ..Patients who present with metastatic primitive neuroectodermal tumor should be treated aggressively with surgical resection as an integral part of the therapeutic strategy...
  7. ncbi Somatic mutations of WNT/wingless signaling pathway components in primitive neuroectodermal tumors
    A Koch
    Department of Neuropathology, University of Bonn Medical Center; Bonn, Germany
    Int J Cancer 93:445-9. 2001
    b>Primitive neuroectodermal tumors (PNETs) represent the most frequent malignant brain tumors in childhood. The majority of these neoplasms occur in the cerebellum and are classified as medulloblastomas (MB)...
  8. ncbi Intense p53 staining is a valuable prognostic indicator for poor prognosis in medulloblastoma/central nervous system primitive neuroectodermal tumors
    R T Woodburn
    Department of Radiation Oncology, Indiana University Medical Center, Indianapolis 46202, USA
    J Neurooncol 52:57-62. 2001
    ..Intense p53 immunostaining may predict for a poor prognosis in central nervous system primitive neuroectodermal tumor of childhood...
  9. ncbi Immunohistochemical detection of EWS and FLI-1 proteinss in Ewing sarcoma and primitive neuroectodermal tumors: comparative analysis with CD99 (MIC-2) expression
    A Llombart-Bosch
    Department of Pathology, Medical School, University of Valencia, Spain
    Appl Immunohistochem Mol Morphol 9:255-60. 2001
    ..rearrangement involving EWS/FLI-1 genes is likely to be of diagnostic value in Ewing sarcoma (ES) and primitive neuroectodermal tumors (PNET)...
  10. pmc Medulloblastomas and primitive neuroectodermal tumors rarely contain polyomavirus DNA sequences
    John Y H Kim
    Division of Neuroscience, Department of Neurology, Children s Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA
    Neuro Oncol 4:165-70. 2002
    ..In contrast to childhood ependymomas and choroid plexus tumors, medulloblastomas and sPNETs infrequently express evidence of polyomavirus infection...
  11. ncbi Supratentorial primitive neuroectodermal tumors in adults
    Dong Gyu Kim
    Department of Neurosurgery, Seoul National University College of Medicine, Clinical Research Institute, Seoul National University Hospital, Republic of Korea
    J Neurooncol 60:43-52. 2002
    ..Intratumoral calcifications and the Ki-67 labelling index might be prognostic factors, however, it should be considered that the sample size is too small and not all patients were evaluated...
  12. ncbi Insulin-like growth factor I receptor signaling system in JC virus T antigen-induced primitive neuroectodermal tumors--medulloblastomas
    Luis Del Valle
    Center for Neurovirology and Cancer Biology, Temple University, Philadelphia, Pennsylvania 19122, USA
    J Neurovirol 8:138-47. 2002
    ....
  13. ncbi Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone
    Holcombe E Grier
    Department of Pediatric Hematology, Dana Farber Cancer Institute and Children s Hospital, Boston, MA 02115, USA
    N Engl J Med 348:694-701. 2003
    ..We designed a study to test whether the addition of these drugs to a standard regimen would improve the survival of patients with newly diagnosed disease...
  14. ncbi Farm-related exposures and childhood brain tumours in seven countries: results from the SEARCH International Brain Tumour Study
    Jimmy T Efird
    Department of Epidemiology and Biostatistics, University of California, San Francisco, California 94118, USA
    Paediatr Perinat Epidemiol 17:201-11. 2003
    ..0) and unprocessed wool (OR = 3.0). Our findings suggest that various farm-related exposures are positively associated with CBT and warrant further investigation into the public health importance of these associations...
  15. ncbi Morphophenotypic variation predicts clinical behavior in childhood non-desmoplastic medulloblastomas
    Charles S McManamy
    Northern Institute for Cancer Research, University of Newcastle, Newcastle upon Tyne, UK
    J Neuropathol Exp Neurol 62:627-32. 2003
    ..Our study supports the concept of an anaplastic variant among MBs, demonstrating that it has clinical utility...
  16. ncbi Evaluation of dietetic intervention in children with medulloblastoma or supratentorial primitive neuroectodermal tumors
    Jaimie Bakish
    Division of Haematology Oncology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
    Cancer 98:1014-20. 2003
    ..The goal of the current study was to evaluate the effect of dietetic intervention in a cohort of children treated for medulloblastoma and supratentorial primitive neuroectodermal tumors (PNET) over a 10-year period.
  17. ncbi Primitive neuroectodermal tumors, embryonal tumors, and other small cell and poorly differentiated malignant neoplasms of the central and peripheral nervous systems
    Hannes Vogel
    Department of Pathology Neuropathology, Stanford, CA, USA
    Ann Diagn Pathol 7:387-98. 2003
    ..One basic distinction is between primitive neuroectodermal tumors of the central nervous system (cPNETs) and primitive neuroectodermal tumors of the peripheral nervous ..
  18. ncbi High-dose chemotherapy with autologous stem cell rescue as first line of treatment in young children with medulloblastoma and supratentorial primitive neuroectodermal tumors
    Antonio Perez-Martinez
    Department of Pediatric Hematology and Oncology, Hospital Nino Jesus, Universidad Autonoma de Madrid, Madrid, Spain
    J Neurooncol 67:101-6. 2004
    ..the dismal prognosis of patients younger than 4 years old with medulloblastoma and supratentorial primitive neuroectodermal tumors (stPNET) seven young children were treated with high-dose chemotherapy (HDCT) and autologous stem ..
  19. ncbi Somatostatin receptor subtype 2 is expressed by supratentorial primitive neuroectodermal tumors of childhood and can be targeted for somatostatin receptor imaging
    Michael C Frühwald
    Department of Pediatric Hematology and Oncology, University Children s Hospital Muenster, Muenster, Germany
    Clin Cancer Res 10:2997-3006. 2004
    ..Novel and refined imaging methodologies are urgently needed...
  20. ncbi Patterns of failure in supratentorial primitive neuroectodermal tumors treated in Children's Cancer Group Study 921, a phase III combined modality study
    Theodore S Hong
    Department of Human Oncology, University of Wisconsin School of Medicine, Madison, WI, USA
    Int J Radiat Oncol Biol Phys 60:204-13. 2004
    To analyze the patterns of failure in patients with supratentorial primitive neuroectodermal tumors (ST-PNETs) treated with combined modality therapy in a large, randomized, multi-institutional study.
  21. ncbi Apurinic/apyrimidinic endonuclease activity is associated with response to radiation and chemotherapy in medulloblastoma and primitive neuroectodermal tumors
    Michael S Bobola
    Division of Neurosurgery, Department of Surgery, Children s Hospital and Regional Medical Center, Seattle, WA 98105, USA
    Clin Cancer Res 11:7405-14. 2005
    ..We assayed apurinic/apyrimidinic endonuclease activity in medulloblastomas and primitive neuroectodermal tumors (PNET) to establish correlates with tumor and patient characteristics and with response to adjuvant ..
  22. ncbi Promoter hypermethylation profile of RASSF1A, FHIT, and sFRP1 in intracranial primitive neuroectodermal tumors
    Qing Chang
    Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong SAR, People s Republic of China
    Hum Pathol 36:1265-72. 2005
    Medulloblastomas (MBs) and supratentorial primitive neuroectodermal tumors (SPNETs) are histologically alike intracranial PNETs found in different anatomical locations of the brain...
  23. ncbi Supratentorial primitive neuroectodermal tumors (S-PNET) in children: A prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy
    Maura Massimino
    Neuro Oncology Functional Unit, Department of Pediatric Oncology, Istituto Nazionale Tumori, Milan, Italy
    Int J Radiat Oncol Biol Phys 64:1031-7. 2006
    Supratentorial primitive neuroectodermal tumors (S-PNET) are rare and have a grim prognosis, frequently taking an aggressive course with local relapse and metastatic spread. We report the results of a mono-institutional therapeutic trial.
  24. ncbi Role of radiotherapy in supratentorial primitive neuroectodermal tumor in young children: results of the German HIT-SKK87 and HIT-SKK92 trials
    Beate Timmermann
    Department of Radiation Oncology and the Institute for Medical Information Processing, University of Tubingen, Tubingen, Germany
    J Clin Oncol 24:1554-60. 2006
    ..To assess the outcome of young children with supratentorial primitive neuroectodermal tumor (stPNET) treated by intensive postoperative chemotherapy alone compared with treatment with chemotherapy and delayed radiotherapy (RT)...
  25. ncbi Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype
    Christine Haberler
    Institute of Neurology, Medical University Vienna, Vienna, Austria
    Am J Surg Pathol 30:1462-8. 2006
    ..biopsy specimens of 289 malignant pediatric CNS tumors including medulloblastomas, supratentorial primitive neuroectodermal tumors, glioblastomas, anaplastic astrocytomas, anaplastic ependymomas, choroid plexus carcinomas, germ cell ..
  26. ncbi Supratentorial primitive neuroectodermal tumors of the central nervous system frequently harbor deletions of the CDKN2A locus and other genomic aberrations distinct from medulloblastomas
    Stefan Pfister
    Division Molecular Genetics, German Cancer Research Center, Im Neuenheimer Feld 280, 69120 Heidelberg, Germany
    Genes Chromosomes Cancer 46:839-51. 2007
    Supratentorial primitive neuroectodermal tumors (stPNETs) and medulloblastomas have long been thought to arise from a common cell type in the subventricular germinal matrix...
  27. ncbi Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report
    Donna L Johnston
    Division of Hematology Oncology, Children s Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON, Canada, K1H 8L1
    J Neurooncol 86:101-8. 2008
    Supratentorial primitive neuroectodermal tumors (SPNET) are rare tumors accounting for only 2.5% of childhood brain tumors...
  28. ncbi Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNETs): report of the Head Start I and I
    Jason Fangusaro
    Children s Hospital Los Angeles, Los Angeles, California, USA
    Pediatr Blood Cancer 50:312-8. 2008
    Children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNET) have poor outcomes compared to medulloblastoma patients, despite similar treatments...
  29. doi Surgical treatment and prognosis of primitive neuroectodermal tumors of the thorax
    Adalet Demir
    Department of Thoracic Surgery, Yedikule Teaching Hospital for Chest Diseases and Thoracic Surgery, Istanbul University, Istanbul, Turkey
    J Thorac Oncol 4:185-92. 2009
    b>Primitive neuroectodermal tumors (PNETs) are rare, rapidly progressive, small- round cell tumors with a poor prognosis despite multimodal therapy, including surgery and chemoradiotherapy...
  30. doi Boost radiosurgery as a strategy after failure of initial management of pediatric primitive neuroectodermal tumors
    Thomas Flannery
    Department of Neurological Surgery and Radiation Oncology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15123, USA
    J Neurosurg Pediatr 3:205-10. 2009
    ..this study was to determine the outcomes of boost stereotactic radiosurgery, specifically Gamma Knife surgery (GKS), for recurrent primitive neuroectodermal tumors (PNETs) in children in whom initial multimodality management had failed.
  31. doi 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
    Anna M Butturini
    Division of Hematology Oncology, Childrens Hospital Los Angeles and Keck School of Medicine, University of Southern California, Los Angeles, California, USA
    Cancer 115:2956-63. 2009
    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 ..
  32. pmc INSM1 promoter-driven adenoviral herpes simplex virus thymidine kinase cancer gene therapy for the treatment of primitive neuroectodermal tumors
    Hong Wei Wang
    Research Institute for Children, Children s Hospital, New Orleans, LA 70118, USA
    Hum Gene Ther 20:1308-18. 2009
    ..of an adenoviral INSM1 promoter-driven herpes simplex virus thymidine kinase (HSV-tk) construct in primitive neuroectodermal tumors (PNETs). We constructed an adenoviral INSM1 promoter-driven HSV-tk gene for therapy in PNETs...
  33. doi Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases
    Amir Behdad
    Department of Pathology, Cedar Sinai Medical Center, Los Angeles, Calif, USA
    Brain Pathol 20:441-50. 2010
    Central nervous system (CNS) primitive neuroectodermal tumors (PNETs) include supratentorial, brain stem, and spinal cord tumors with medulloblastoma-like histopathology...
  34. doi Amplification and overexpression of KIT, PDGFRA, and VEGFR2 in medulloblastomas and primitive neuroectodermal tumors
    Tea Blom
    Molecular Cancer Biology Program, Biomedicum Helsinki, University of Helsinki, Haartmaninkatu 8, P O Box 63, 00014, University of Helsinki, Finland
    J Neurooncol 97:217-24. 2010
    Medulloblastomas (MB) and primitive neuroectodermal tumors (PNET) are the most common malignant brain tumors in children. These two tumor types are histologically similar, but have different genetic backgrounds and clinical outcomes...
  35. doi Intraspinal primitive neuroectodermal tumors: Report of four cases and review of the literature
    Chen Jingyu
    Department of Neurosurgery, Southwest Hospital, Third Military Medical University, Chongqing 400 038, The People s Republic of China
    Neurol India 57:661-8. 2009
    Intraspinal primitive neuroectodermal tumors (PNETs) are extremely rare and are aggressive with poor outcomes. Till date only 41 cases of intraspinal PNET have been reported. Here we report four new cases...
  36. doi Imaging findings of primitive neuroectodermal tumors of the kidney
    Hyun Lee
    Department of Radiology, Hallym University Sacred Heart Hospital, Seoul, Republic of Korea
    J Comput Assist Tomogr 33:882-6. 2009
    ..This study was designed to present the radiological imaging findings of a renal primitive neuroectodermal tumor (PNET)...
  37. doi Non-cerebellar primitive neuroectodermal tumors (PNET): summary of the Milan consensus and state of the art workshop on marrow ablative chemotherapy with hematopoietic cell rescue for malignant brain tumors of childhood and adolescents
    Jason Fangusaro
    Pediatric Hematology Oncology and Stem Cell Transplantation, Children s Memorial Hospital Chicago, Chicago, Illinois 60614, USA
    Pediatr Blood Cancer 54:638-40. 2010
    CNS non-cerebellar primitive neuroectodermal tumors (PNET) represent <5% of all childhood brain tumors. Despite similar therapies, these patients have had significantly worse outcomes compared to patients with medulloblastoma...
  38. ncbi Treatment of high-risk medulloblastoma and other primitive neuroectodermal tumors with reduced dose craniospinal radiation therapy and multi-agent nitrosourea-based chemotherapy
    M D Prados
    Department of Neurosurgery, University of California, San Francisco, USA
    Pediatr Neurosurg 25:174-81. 1996
    ..survival (PFS) of children and adults with newly diagnosed medulloblastoma, pineoblastoma, and other primitive neuroectodermal tumors (PNET) with a combined modality regimen of radiation therapy and adjuvant nitrosourea-based ..
  39. ncbi Mutations of the INI1 rhabdoid tumor suppressor gene in medulloblastomas and primitive neuroectodermal tumors of the central nervous system
    J A Biegel
    Division of Human Genetics and Molecular Biology, The Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    Clin Cancer Res 6:2759-63. 2000
    ..Detection of these mutations suggests that the child has an AT/RT, rather than a MB/PNET, a finding with important prognostic value...
  40. ncbi Abundance of apoptotic neoplastic cells in diagnostic biopsy samples is not a prognostic factor in childhood primitive neuroectodermal tumors of the central nervous system
    M A Grotzer
    Division of Oncology, The Children's Hospital of Philadelphia, Pennsylvania 19104, USA
    J Pediatr Hematol Oncol 23:25-9. 2001
    PURPOSE: To assess if the abundance of apoptotic tumor cells is an independent prognostic factor in primitive neuroectodermal tumors (PNET) of the central nervous system...
  41. ncbi Prognostic significance of Ki-67 (MIB-1) proliferation index in childhood primitive neuroectodermal tumors of the central nervous system
    M A Grotzer
    Division of Neurology and Oncology, The Children's Hospital of Philadelphia, Pennsylvania 19104, USA
    Med Pediatr Oncol 36:268-73. 2001
    BACKGROUND: Primitive neuroectodermal tumors (PNET) of the central nervous system, including medulloblastomas, are the most common malignant brain tumors of childhood...
  42. ncbi Parental occupations and childhood brain tumors: results of an international case-control study
    S Cordier
    INSERM, Villejuif, France
    Cancer Causes Control 12:865-74. 2001
    ..sex, year of birth, and center, for all types of CBT combined and for the subgroups of astroglial, primitive neuroectodermal tumors (PNET), and other glial tumors...
  43. ncbi Overexpression of cyclooxygenase-2 (COX-2) in human primitive neuroectodermal tumors: effect of celecoxib and rofecoxib
    Ratnakar Patti
    Division of Neuro Oncology, Joseph Stokes Research Institute 515G, 3516 Civic Center Blvd, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Cancer Lett 180:13-21. 2002
    ..Growth inhibition by celecoxib and rofecoxib was independent of Bcl-2 expression. Celecoxib suppressed the expression of Akt and activated the caspase-3 in DAOY and PFSK, whereas rofecoxib did not have such an effect...
  44. ncbi Molecular genetic analysis of the TP53, PTEN, CDKN2A, EGFR, CDK4 and MDM2 tumour-associated genes in supratentorial primitive neuroectodermal tumours and glioblastomas of childhood
    J A Kraus
    Department of Neurology, University of Bonn, Medical Center, Bonn, Germany
    Neuropathol Appl Neurobiol 28:325-33. 2002
    ..In addition, the patterns of genetic alterations in sPNETs and paediatric GBMs appear to be distinct from those in cerebellar medulloblastomas and adult GBMs, respectively...
  45. ncbi Primitive neuroectodermal tumors in the central nervous system following cranial irradiation: a report of four cases
    Walter J Hader
    Division of Neurosurgery, Department of Clinical Neurosciences, University of Calgary, Calgary, Canada
    Cancer 97:1072-6. 2003
    ..The development of primitive neuroectodermal tumors (PNETs) following prophylactic craniospinal irradiation has been infrequently reported previously...
  46. ncbi Dynamic susceptibility contrast-enhanced perfusion and conventional MR imaging findings for adult patients with cerebral primitive neuroectodermal tumors
    Meng Law
    Department of Radiology, New York University Medical Center, New York, NY 10016, USA
    AJNR Am J Neuroradiol 25:997-1005. 2004
    Preoperative differentiation of primitive neuroectodermal tumors (PNETs) from other tumors is important for presurgical staging, intraoperative management, and postoperative treatment...
  47. ncbi High-dose chemotherapy with autologous stem cell rescue for children with high risk and recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors
    Antonio Perez-Martinez
    Department of Pediatric Hematology and Oncology, Hospital Nino Jesus, Universidad Autonoma de Madrid, Spain
    J Neurooncol 71:33-8. 2005
    Current treatment for high risk and recurrent medulloblastoma (MB) and supratentorial primitive neuroectodermal tumors (stPNET) has a very poor prognosis in children...
  48. ncbi Treatment strategies for high-risk medulloblastoma and supratentorial primitive neuroectodermal tumors. Review of the literature
    Regina I Jakacki
    Division of Pediatric Hematology Oncology, Children s Hospital of Pittsburgh, Pennsylvania 15213, USA
    J Neurosurg 102:44-52. 2005
    b>Primitive neuroectodermal tumors (PNETs) are malignant tumors with a high propensity to disseminate throughout the cerebrospinal fluid...
  49. ncbi Analysis of patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study
    Barry L Pizer
    Alder Hey s Children s Hospital Oncology Unit, Eaton Road, Liverpool L12 2AP, UK
    Eur J Cancer 42:1120-8. 2006
    ..Future treatment programs should be directed at the particular natural history of these tumours, to further define prognostic factors and to explore further biological characteristics...
  50. pmc High-resolution array-based comparative genomic hybridization of medulloblastomas and supratentorial primitive neuroectodermal tumors
    Martin Gerard McCabe
    Department of Pathology, University of Cambridge, Division of Molecular Histopathology, UK
    J Neuropathol Exp Neurol 65:549-61. 2006
    Medulloblastomas and supratentorial primitive neuroectodermal tumors are aggressive childhood tumors...
  51. ncbi RASSF1A promoter is highly methylated in primitive neuroectodermal tumors of the central nervous system
    Maria del Mar Inda
    Unidad de Biología de Tumores Cerebrales, Universidad de Navarra, Pamploma, Spain
    Neuropathology 27:341-6. 2007
    ..b>Primitive neuroectodermal tumors (PNET) of the central nervous system can be divided into infratentorial PNET or medulloblastoma (MB), ..
  52. doi Radiation is an important component of multimodality therapy for pediatric non-pineal supratentorial primitive neuroectodermal tumors
    Sean M McBride
    Department of Radiation Oncology, University of California San Francisco, San Francisco, CA
    Int J Radiat Oncol Biol Phys 72:1319-23. 2008
    To review a historical cohort of pediatric patients with supratentorial primitive neuroectodermal tumors (sPNET), to clarify the role of radiation in the treatment of these tumors.
  53. doi Role of high-dose chemotherapy for recurrent medulloblastoma and other CNS primitive neuroectodermal tumors
    Amar Gajjar
    St Jude Children s Research Hospital, Memphis, Tennessee, USA
    Pediatr Blood Cancer 54:649-51. 2010
    ..chemotherapy (HDCT) with stem cell rescue for patients with recurrent medulloblastoma and other primitive neuroectodermal tumors as presented at an international consensus and state of the art workshop held in Milan in 2006...
  54. doi Computed tomography imaging of anterior and middle mediastinal Ewing sarcoma/primitive neuroectodermal tumors
    Wei Dong Zhang
    State Key Laboratory of Oncology, South China, Guangdong 510060, PR China
    J Thorac Imaging 25:168-72. 2010
    We describe the computed tomography (CT) imaging features of Ewing sarcoma (EWS)/primitive neuroectodermal tumors (PNETs) arising in the anterior and middle mediastinum.
  55. pmc The molecular pathology of p53 in primitive neuroectodermal tumours of the central nervous system
    A S Y W Burns
    Cancer Research Unit, Medical School University of Newcastle, Newcastle upon Tyne, UK
    Br J Cancer 86:1117-23. 2002
    ..Multivariate analyses supported the prognostic value of p53 immunostaining in central primitive neuroectodermal tumours and also of age and gender of patients...
  56. ncbi Malignant teratoma of the thyroid with primitive neuroepithelial and mesenchymal sarcomatous components
    R D Craver
    Department of Pathology, Children's Hospital, Louisiana State University Medical Center, 200 Henry Clay Ave, New Orleans, LA 70118, USA
    Ann Diagn Pathol 5:285-92. 2001
    ..Trisomy 8 is the first cytogenetic abnormality described in malignant thyroid teratoma. Therapy should be tailored to the management of all transformed histologies...
  57. ncbi A novel case of a CAT to AAT transversion in codon 179 of the p53 gene in a supratentorial primitive neuroectodermal tumor harbored by a young girl. Case report and review of the literature
    Sergey Postovsky
    Department of Pedicatric Hematology Onclology, Rambam Medical Center, Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel
    Oncology 65:46-51. 2003
    The most common cytogenetic abnormality encountered in primitive neuroectodermal tumors (PNET)/medulloblastoma is loss of heterozygosity in the region of the short arm of chromosome 17...
  58. ncbi Central nervous system medulloepithelioma: a series of eight cases including two arising in the pons
    P T Molloy
    Department of Pathology, The Children s Hospital of Philadelphia, Department of Pathology, The Children s Hospital of Philadelphia Pennsylvania, USA
    J Neurosurg 84:430-6. 1996
    ..CNS) whose histopathological appearance has been confused with medulloblastoma and other childhood primitive neuroectodermal tumors (PNETs), but which has a vastly different clinical course...
  59. ncbi Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study
    D Strother
    Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA
    J Clin Oncol 19:2696-704. 2001
    ..The early outcome results of this approach are very encouraging...
  60. ncbi Antineuronal nuclei immunohistochemical staining patterns in childhood ependymomas
    J R Parker
    Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
    J Child Neurol 16:548-52. 2001
    ..Five astrocytomas, four primitive neuroectodermal tumors, and normal cerebral cortex and ependyma from autopsy brains of premature newborns, term infants, and ..
  61. ncbi Absence of germline and somatic p53 alterations in children with sporadic brain tumors
    C Portwine
    Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Ontario, Canada
    J Neurooncol 52:227-35. 2001
    ..However, the potential significance of LOH at codon 72 remains to be elucidated...
  62. pmc Ex vivo pediatric brain tumors express Fas (CD95) and FasL (CD95L) and are resistant to apoptosis induction
    C D Riffkin
    Department of Hematology and Oncology, Royal Children's Hospital, Melbourne, Victoria, Australia
    Neuro Oncol 3:229-40. 2001
    ..The results suggest that Fas expression may be a general feature of tumors of the CNS and that a significant degree of resistance to Fas-mediated apoptosis may exist in ex vivo pediatric brain tumor specimens...
  63. ncbi Angiogenic profile of childhood primitive neuroectodermal brain tumours/medulloblastomas
    H Huber
    Division of Oncology, The Children's Hospital of Philadelphia, PA 19104, USA
    Eur J Cancer 37:2064-72. 2001
    ..This suggests that anti-angiogenesis approaches targeting VEGF alone may be insufficient in PNET/MB...
  64. ncbi Loss of caspase-8 mRNA expression is common in childhood primitive neuroectodermal brain tumour/medulloblastoma
    T J Zuzak
    Division of Oncology, University Children s Hospital of Zurich, Switzerland
    Eur J Cancer 38:83-91. 2002
    ..We conclude that loss of caspase-8 gene expression is common in PNET/medulloblastoma suggesting that suppression of death receptor induced apoptosis may play an important role in the pathogenesis of this common childhood brain tumour...
  65. ncbi Diffusion-weighted imaging and fluid attenuated inversion recovery imaging in the evaluation of primitive neuroectodermal tumors
    E Erdem
    Department of Radiology, The Children's Hospital of Philadelphia, PA 19104, USA
    Neuroradiology 43:927-33. 2001
    ..recovery (FLAIR) imaging and diffusion-weighted imaging (DWI) would be helpful in characterizing primitive neuroectodermal tumors (PNET) from other pediatric brain tumors...
  66. ncbi Supratentorial embryonal tumors in children under 5 years of age: an SFOP study of treatment with postoperative chemotherapy alone
    P Marec-Berard
    Pediatric Unit, Centre Leon Berard, 69373 Lyon, France
    Med Pediatr Oncol 38:83-90. 2002
    ..To determine the effectiveness of multiagent chemotherapy as sole post-operative treatment of supratentorial central nervous system (CNS) embryonal tumors in young children...
  67. ncbi Risk assignment in childhood brain tumors: the emerging role of molecular and biologic classification
    Ian F Pollack
    Department of Neurosurgery, Children s Hospital of Pittsburgh, Main Tower, Floor 3, Room 3705, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA
    Curr Oncol Rep 4:114-22. 2002
    ..This paper summarizes these observations and discusses how they are being applied in a preliminary fashion as a foundation for risk-adapted stratification of childhood brain tumor therapy...
  68. ncbi Chlorotoxin, a scorpion-derived peptide, specifically binds to gliomas and tumors of neuroectodermal origin
    Susan A Lyons
    TransMolecular, Inc, Birmingham, Alabama 35294, USA
    Glia 39:162-73. 2002
    ..These results suggest that chlorotoxin is a reliable and specific histopathological marker for tumors of neuroectodermal origin and that chlorotoxin derivatives with cytolytic activity may have therapeutic potential for these cancers...
  69. ncbi Basal ganglia and thalamic tumours: an imaging approximation
    José M García-Santos
    Neuroradiology Section, Hospital General Universitario, J Ma Morales Meseguer, C Marques de los Vélez s n, 30008 Murcia, Spain
    Childs Nerv Syst 18:412-25. 2002
    ..In other occasions peripheral neoplasms developing in neighbouring structures like the cerebral lobes, the ventricular walls, choroidal plexus, pineal gland and the hypothalamic-chiasmatic-suprasellar region can spread to the deep brain...
  70. ncbi Relation between Irofulven (MGI-114) systemic exposure and tumor response in human solid tumor xenografts
    Markos Leggas
    Department of Pharmaceutical Sciences, St Jude Children s Research Hospital, Memphis, Tennessee 38105, USA
    Clin Cancer Res 8:3000-7. 2002
    ....
  71. pmc Primary intracranial atypical teratoid/rhabdoid tumor in a child: a case report
    You Nam Chung
    Department of Neurosurgery, Seoul National University College of Medicine and Neurological Research Institute, SNUMRC, Seoul, Korea
    J Korean Med Sci 17:723-6. 2002
    ..Despite aggressive therapy, he died 19 months after diagnosis. Clinical, radiological, and histopathological features of primary intracranial ATT/RhT are discussed with a special emphasis on the differential diagnosis from PNET-MB...
  72. pmc hTERT gene amplification and increased mRNA expression in central nervous system embryonal tumors
    Xing Fan
    Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Am J Pathol 162:1763-9. 2003
    ..Our data indicate that hTERT gene amplification is relatively common in embryonal brain tumors, and that increased expression of hTERT mRNA may be associated with biologically aggressive tumor behavior...
  73. ncbi Expression of alpha-, beta-, and gamma-synuclein in glial tumors and medulloblastomas
    Kar Ming Fung
    Department of Pathology, University of Oklahoma Health Science Center OUHSC, Rm 451, 940 Stanton L Young Blvd, Oklahoma City, OK 73104, USA
    Acta Neuropathol 106:167-75. 2003
    ..Of medulloblastomas, 76% have immunoreactivity for either alpha- or beta-synuclein or both; no immunoreactivity for gamma-synuclein is seen in medulloblastomas...
  74. ncbi Mediastinal T-cell lymphoma in a boy 7 years after treatment of supratentorial primitive neuroectodermal tumor
    Thomas Lehrnbecher
    Department of Pediatric Hematology and Oncology, Children s Hospital III, Frankfurt, Germany
    J Pediatr Hematol Oncol 25:657-9. 2003
    ..In addition, patients with second malignancies should be considered as a high-risk population for opportunistic infections...
  75. ncbi Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management
    Jochen P Windfuhr
    Department of Otorhinolaryngology Plastic Head and Neck Surgery, St Anna Hospital, Duisburg, Germany
    Ann Otol Rhinol Laryngol 113:533-43. 2004
    b>Primitive neuroectodermal tumors are in the Ewing's sarcoma family of tumors and are composed of small round cells. Because of their rare occurrence, optimal therapy is challenging, particularly if they occur in the head and neck...
  76. ncbi Results of a phase II upfront window of pharmacokinetically guided topotecan in high-risk medulloblastoma and supratentorial primitive neuroectodermal tumor
    Clinton F Stewart
    Department of Pharmaceutical Sciences, St Jude Children s Research Hospital, 332 N Lauderdale, Memphis, TN 38105 2794, USA
    J Clin Oncol 22:3357-65. 2004
    ..efficacy of pharmacokinetically guided topotecan dosing in previously untreated patients with medulloblastoma and supratentorial primitive neuroectodermal tumors, and to evaluate plasma and CSF disposition of topotecan in these patients.
  77. ncbi [Refractory partial epilepsy: morphological imaging in children]
    C Raybaud
    Neuroradiologie, CHU Timone, Universite de la Mediterranee, 13000 Marseille, France
    Rev Neurol (Paris) 160:5S106-16. 2004
    ..In the event of a simple lesion, the decision for surgery on the basis of morphological data alone is more easily made in children than in adults...
  78. ncbi Notch1 and notch2 have opposite effects on embryonal brain tumor growth
    Xing Fan
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Cancer Res 64:7787-93. 2004
    ..Our data indicate that Notch1 and Notch2 can have opposite effects on the growth of a single tumor type, and show that Notch2 can be overexpressed after gene amplification in human tumors...
  79. ncbi Atypical teratoid/rhabdoid tumor of the brain: cytopathologic characteristics and differential diagnosis
    Anil V Parwani
    Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA
    Cancer 105:65-70. 2005
    ..Although the clinicopathologic and radiologic features of AT/RT have been described previously, to the authors' knowledge the cytomorphologic profile of this tumor has not been studied well...
  80. ncbi Epigenetic inactivation of DLC-1 in supratentorial primitive neuroectodermal tumor
    Jesse Chung Sean Pang
    Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, People s Republic of China
    Hum Pathol 36:36-43. 2005
    Supratentorial primitive neuroectodermal tumors (SPNETs) and medulloblastomas (MBs) are histologically similar intracranial tumors found in different anatomic locations of the brain...
  81. pmc Increased p53 immunopositivity in anaplastic medulloblastoma and supratentorial PNET is not caused by JC virus
    Charles G Eberhart
    Department of Pathology, Johns Hopkins University School of Medicine, Ross Bldg 558, 720 Rutland Ave, Baltimore, MD 21205, USA
    BMC Cancer 5:19. 2005
    ..We correlated p53 protein expression with pathological subtype and clinical outcome in 75 embryonal brain tumors. The presence of JC virus, which results in p53 protein accumulation, was also examined...
  82. ncbi Patterns of treatment failure in infants with primitive neuroectodermal tumors who were treated on CCG-921: a phase III combined modality study
    Theodore S Hong
    Department of Human Oncology, School of Medicine, University of Wisconsin, Madison, WI 53792, USA
    Pediatr Blood Cancer 45:676-82. 2005
    To analyze patterns of treatment failure in infants with primitive neuroectodermal tumors (PNETs) who were treated primarily with chemotherapy in a large multi-institutional study.
  83. ncbi The therapy of infantile malignant brain tumors: current status?
    Chantal Kalifa
    Pediatric Department, Institut Gustave Roussy, 39 rue Camille Desmoulins, 94805, Villejuif Cedex, France
    J Neurooncol 75:279-85. 2005
    ..With these informations it is now possible to design therapeutic trials devoted to each histological types, adapted to pronostic factors and more accurate treatment to decrease long term sequelae...
  84. ncbi Genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumours of the central nervous system
    M M Inda
    Laboratoire de Génomique Cellulaire des Cancers, UMR1599, CNRS, Institut Gustave Roussy, Villejuif, France
    Histopathology 47:631-7. 2005
    ..We investigated the differences in genetic abnormalities between sPNET and MB...
  85. ncbi Outcome for children <4 years of age with malignant central nervous system tumors treated with high-dose chemotherapy and autologous stem cell rescue
    Halldora K Thorarinsdottir
    Division of Hematology and Oncology, Children s National Medical Center, Washington, District of Columbia, USA
    Pediatr Blood Cancer 48:278-84. 2007
    ....
  86. ncbi Boron neutron capture therapy using mixed epithermal and thermal neutron beams in patients with malignant glioma-correlation between radiation dose and radiation injury and clinical outcome
    Teruyoshi Kageji
    Department of Neurosurgery, Graduate School of Health Biosciences, The University of Tokushima, Tokushima, Japan
    Int J Radiat Oncol Biol Phys 65:1446-55. 2006
    ..To clarify the correlation between the radiation dose and clinical outcome of sodium borocaptate-based intraoperative boron neutron capture therapy in patients with malignant glioma...
  87. ncbi Role of spinal MRI in the follow-up of children treated for medulloblastoma
    Ute Bartels
    Division of Haematology Oncology, Hospital for Sick Children, Toronto, Ontario, Canada
    Cancer 107:1340-7. 2006
    ..The purpose of the current study was to describe the usefulness of spinal magnetic resonance imaging (MRI) in children with medulloblastoma or primitive neuroectodermal tumor (PNET) of the posterior fossa...
  88. ncbi Phase II study of oxaliplatin in children with recurrent or refractory medulloblastoma, supratentorial primitive neuroectodermal tumors, and atypical teratoid rhabdoid tumors: a pediatric brain tumor consortium study
    Maryam Fouladi
    St Jude Children s Research Hospital, Memphis, Tennessee 38105 2794, USA
    Cancer 107:2291-7. 2006
    ..II study of oxaliplatin was conducted to evaluate its safety and efficacy in children with recurrent or refractory medulloblastoma (MB), supratentorial primitive neuroectodermal tumors (SPNET), and atypical teratoid rhabdoid tumor (ATRT).
  89. ncbi High-dose chemotherapy and autologous stem cell rescue in children with newly diagnosed high-risk or relapsed medulloblastoma or supratentorial primitive neuroectodermal tumor
    Ki Woong Sung
    Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
    Pediatr Blood Cancer 48:408-15. 2007
    ....
  90. ncbi Paediatric PNET: pre-surgical MRI features
    A Chawla
    Department of Neuroradiology, National Neuroscience Institute, Singapore
    Clin Radiol 62:43-52. 2007
    ....
  91. ncbi Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: a prospective study by the Children's Oncology Group
    Patricia L Robertson
    Department of Pediatrics, University of Michigan Health System, Ann Arbor, Michigan 48109 0203, USA
    J Neurosurg 105:444-51. 2006
    ..Most descriptions have been limited to small institutional series using a retrospective chart review methodology...
  92. ncbi Frequent but borderline methylation of p16 (INK4a) and TIMP3 in medulloblastoma and sPNET revealed by quantitative analyses
    J Mühlisch
    Department of Pediatric Hematology and Oncology, University Children s Hospital Muenster, 48129 Muenster, Germany
    J Neurooncol 83:17-29. 2007
    ..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...
  93. ncbi The Azzopardi phenomenon
    Hidehiro Takei
    Arch Pathol Lab Med 131:518. 2007
  94. ncbi Tumor suppressor proteins are differentially affected in human ependymoblastoma and medulloblastoma cells exposed to nerve growth factor
    A Antonelli
    Institute of Neurobiology and Molecular Medicine, CNR EBRI, Rome, Italy
    Cancer Invest 25:94-101. 2007
    ..These observations indicate that NGF by interfering with mechanisms associated with cells proliferation and survival might induce the differentiation event through TrkA pathways...
  95. ncbi Thyroid-stimulating hormone suppression for protection against hypothyroidism due to craniospinal irradiation for childhood medulloblastoma/primitive neuroectodermal tumor
    Maura Massimino
    Department of Pediatrics, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
    Int J Radiat Oncol Biol Phys 69:404-10. 2007
    ..Hence, our study was launched in 1998 to evaluate the protective effect of TSH suppression during CSI for medulloblastoma/primitive neuroectodermal tumor...
  96. ncbi Type I collagen is overexpressed in medulloblastoma as a component of tumor microenvironment
    Yu Liang
    Department of Neurological Surgery, Brain Tumor Research Center, University of California San Francisco, San Francisco, CA 94143, USA
    J Neurooncol 86:133-41. 2008
    ....
  97. ncbi [Experience with the intrathecal use of liposomal cytarabine at the Besta Institute]
    Antonio Silvani
    Istituto Nazionale Neurologico Carlo Besta, Milano
    Tumori 93:suppl 6-8. 2007
  98. ncbi Phase 2 study of temozolomide in children and adolescents with recurrent central nervous system tumors: a report from the Children's Oncology Group
    H Stacy Nicholson
    Department of Pediatrics, Oregon Health Science and University, Portland, Oregon, USA
    Cancer 110:1542-50. 2007
    ..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...
  99. ncbi Brain magnetic resonance imaging after high-dose chemotherapy and radiotherapy for childhood brain tumors
    Filippo Spreafico
    Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
    Int J Radiat Oncol Biol Phys 70:1011-9. 2008
    ..Brain necrosis or other subacute iatrogenic reactions has been recognized as a potential complication of radiotherapy (RT), although the possible synergistic effects of high-dose chemotherapy and RT might have been underestimated...
  100. ncbi Feasibility of metronomic maintenance chemotherapy following high-dose chemotherapy for malignant central nervous system tumors
    L Mi Rim Choi
    Division of Hematology and Oncology, Children s National Medical Center, Washington, District of Columbia, USA
    Pediatr Blood Cancer 50:970-5. 2008
    ..Metronomic chemotherapy has also been reported as a potential new treatment strategy in solid tumors, particularly in adults...
  101. ncbi Hypofractionated stereotactic radiotherapy in the management of recurrent or residual medulloblastoma/PNET
    Frank Saran
    Neuro oncology Unit and the Academic Unit of Radiotherapy and Oncology, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, Sutton, United Kingdom
    Pediatr Blood Cancer 50:554-60. 2008
    To evaluate the efficacy and toxicity of hypofractionated stereotactic radiotherapy in the management of locally recurrent or residual central nervous system (CNS) primitive neuroectodermal tumors (PNETs).

Research Grants49

  1. Inhibition of matrix proteases to sensitize medulloblastoma cells to radiation
    Andrei L Gartel; Fiscal Year: 2013
    Medulloblastomas, which belong to a group of primitive neuroectodermal tumors, are invasive tumors with predominant neuronal differentiation...
  2. Pesticide Exposure and Childhood Cancer Study
    Beate R Ritz; Fiscal Year: 2012
    ..cancers (acute myeloid leukemia, acute lymphoblastic leukemia, non Hodgkin lymphoma, astrocytoma, primitive neuroectodermal tumors, Wilms tumor, soft tissue sarcomas) and population-based controls...
  3. Cellular plasticity and survival mechanisms in medulloblastoma stem cells
    Ronnie Yoo; Fiscal Year: 2013
    ..abstract_text> ..
  4. 'Exvading'Invasive Pediatric Brain Tumors
    Ravi V Bellamkonda; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Medulloblastomas are highly invasive primitive neuroectodermal tumors of the cerebellum and the most common childhood malignant brain tumor, constituting 20-40% of pediatric brain tumors...
  5. Clinical pharmacology of cyclophosphamide in pediatric medulloblastoma patients
    Michael B Tagen; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Medulloblastoma and primitive neuroectodermal tumors (PNETs) are childhood brain tumors where about 40% of patients die due to early tumor recurrence...
  6. Edward A Neuwelt; Fiscal Year: 2014
    ..potential for enhancing chemotherapy dose intensity for intracerebral medulloblastoma as a model of primitive neuroectodermal tumors within brain parenchyma, by using BBBD and by depleting glutathione stores in conjunctions with thiol ..
  7. Human Neurotropic JC Virus and CNS Diseases
    Kamel Khalili; Fiscal Year: 2006
    ..By using the early genome of JCV, we have created transgenic animals that develop cerebellar primitive neuroectodermal tumors with extraordinary similarity to human medulloblastoma...
  8. Academic Public Private Partnership Program
    M O DORISIO; Fiscal Year: 2004
    ..neuroendocrine tumors seen in adults, pediatric neuroendocrine tumors thus include neuroblastoma and primitive neuroectodermal tumors (PNET) such as medulloblastoma, supratentorial PNET and the Ewings sarcoma family of tumors...
  9. MOLECULAR ANALYSIS OF PEDIATRIC CANCERS
    Giovanni Rovera; Fiscal Year: 1993
    ..Project 3 will identify and characterize NGF induced immediate-early genes in primitive neuroectodermal tumors which are incompetent to trans activity other genes whose expression is required for terminal ..
  10. PRIMITIVE NEUROECTODERMAL TUMORS OF CHILDHOOD
    Peter Phillips; Fiscal Year: 2000
    Medulloblastoma (MB) and related CNS primitive neuroectodermal tumors (PNETs) are the most common pediatric malignant brain tumors and major source of neurological morbidity and mortality in children...
  11. CELL DEATH AND DIFFERENTIATION IN MEDULLOBLASTOMA
    John Trojanowski; Fiscal Year: 1999
    Medulloblastomas (MBs) are prototypical primitive neuroectodermal tumors (PNETs) of the cerebellum, and they are among the most common pediatric brain tumors...
  12. SONIC HEDGEHOG/PTCH PATHWAY IN PNET
    Corey Raffel; Fiscal Year: 2000
    ..b>Primitive neuroectodermal tumors are the most common of these...
  13. Parallel Peptide Tandem Mass Spectrometry (MS)
    David Goodlett; Fiscal Year: 2003
    ..This work will be done with medulloblastoma cell lines and primary cells lines derived from retinoid-sensitive and -resistant tumors. ..
  14. Mediators of EWS/FLI Oncogenesis in Ewing's Sarcoma
    William May; Fiscal Year: 2005
    ..This understanding will provide a valuable paradigm for the study of the wide array of malignancies that are associated with aberrant transcription factors. ..
  15. Mammalian genetic analysis of embryonic neural overgrow*
    Karen Artzt; Fiscal Year: 2006
    ..It is expected that this set of genes will define a new tumor suppressor that plays-a role in the development of primitive neuroectodermal tumors of children.
  16. Lapatinib in childhood CNS malignancies
    Maryam Fouladi; Fiscal Year: 2006
    ..These data will be used in the rational design of future phase I combination or frontline studies to improve the outcome in children with these poor prognosis tumors. [unreadable] [unreadable]..
  17. Myc Signaling in Medulloblastomas
    Charles Eberhart; Fiscal Year: 2006
    ..Finally, in Specific Aim 4 we propose developing a novel medulloblastoma transgenic model by overexpressing c-Myc in the cerebellum of transgenic mice. ..
  18. Regulation- Medulloblastoma Growth by alpha-v integrins
    Tobey Macdonald; Fiscal Year: 2006
    ..The long-range goal of this work is to broaden our understanding of medulloblastoma growth, so that novel biologic therapies may be developed into new treatments for medulloblastoma, the most common malignant brain tumor in children. ..
  19. CONTROL SELECTION METHODS FOR CHILDHOOD CANCER STUDIES
    Greta Bunin; Fiscal Year: 2009
    ..A feasible and valid control group is needed to ensure that studies of childhood cancer are scientifically valid and therefore, will provide results that can eventually lead to prevention of these cancers. ..
  20. Occupational Pesticide Exposure of Parents and Brain Tumor Risk in Children
    Greta Bunin; Fiscal Year: 2008
    ..The proposed study will provide information on the usefulness of these approaches. [unreadable] [unreadable] [unreadable]..
  21. Pirfenidone in Children with PNs in NF1
    Roger Packer; Fiscal Year: 2006
    ..Abstract Not Provided ..
  22. PATERNAL EXPOSURE AND SPORADIC BILATERAL RETINOBLASTOMA
    Greta Bunin; Fiscal Year: 2005
    ..Sporadic heritable RBL is a childhood cancer worth studying in its own right and an ideal model for the investigation of new germline mutation. ..
  23. CHILDRENS NATIONAL MEDICAL CENTER
    Roger Packer; Fiscal Year: 2003
    ....
  24. International Network and Registry for TMA
    Howard Trachtman; Fiscal Year: 2007
    ..These include essential hypertension, diabetes, hyperlipidemia, myocardial infarction, and chronic kidney disease. [unreadable] [unreadable]..
  25. PREMATURE MENOPAUSE IN SURVIVORS OF CHILDHOOD CANCER
    Charles Sklar; Fiscal Year: 2002
    ..The large size of the study population, the heterogeneity of diagnoses and exposures, combined with the extensive treatment data, will allow assessment of interaction between the major risk factors of interest. ..
  26. QUALITY OF LIFE FOLLOWING SUCCESSFUL THERAPY OF AML
    HENRY NICHOLSON; Fiscal Year: 2001
    ..Completion of this study should help pediatric oncologists fight AML in ways that optimize both survival and QOL. ..
  27. PEDIATRIC ONCOLOGY GROUP STUDIES
    Thomas McLean; Fiscal Year: 2002
    ....
  28. GENES INVOLVED IN AIDS LYMPHOMAGENESIS
    Michael Teitell; Fiscal Year: 2002
    ..Genes with significant involvement in the etiology and/or progression of lymphomagenesis, or those with reproducible in vitro biochemical effects, will be further characterized by molecular methods. ..
  29. Cellular Immunotherapy for Neuroblastoma With CTL clones
    Michael Jensen; Fiscal Year: 2002
    ..4.) To assess in this patient population the development of antibody and cellular immune responses against the scFvFc:zeta and HyTK chimeric proteins. ..
  30. Ethnic Differences in Survival after Childhood ALL
    Smita Bhatia; Fiscal Year: 2009
    ..abstract_text> ..
  31. Control of Eye Melanoma Metastsis
    Hans E Grossniklaus; Fiscal Year: 2010
    ..The third aim determines the mechanism of combined angiostatin/IFN control of the micrometastases using methods in the first two aims. ..
  32. BONE MARROW TRANSPLANT (BMT) SURVIVORS STUDY
    Smita Bhatia; Fiscal Year: 2003
    ..g., second cancers, births, cardiac complications, etc.) that may have occurred since the completion of the initial questionnaire. ..
  33. Vaso-occlusive Mechanisms that Induce Hypoxia and Cause Glioma Progression.
    Daniel J Brat; Fiscal Year: 2010
    ..This proposal introduces entirely novel concepts that may explain the highly aggressive properties of GBM and suggests therapeutic approaches that could potentially stabilize its progression. ..
  34. COLLABORATIVE OCULAR MELANOMA STUDY
    FREDERICK DAVIDORF; Fiscal Year: 2003
    ..Standardized clinical and data collection procedures will be employed, and standardized forms will be used in all centers. Central training and certification of all study staff will be required. ..
  35. Genetic Mutations in Gliomatosis Cerebri
    Marcus Ware; Fiscal Year: 2004
    ..We shall use this data to determine if there are specific patterns of chromosome aberrations that are characteristic of GC. Our final goal is to compare copy-number aberrations seen in GC to those seen in other non-GC astrocytic tumors. ..
  36. Development of an Aging and Cancer Program at MSKCC
    George Bosl; Fiscal Year: 2007
    ..Through this planning grant, MSKCC will formally expand its research activities in cancer biology and management in the aging population so that it will become a key component of its NCI-funded CCSG. ..
  37. GSK-3Beta and Associated Pathways in PNET
    Corey Raffel; Fiscal Year: 2004
    b>Primitive neuroectodermal tumors (PNET) are the most common malignant brain tumor of childhood. Using best available therapy, five-year survival for children with PNET is 60%...
  38. INVERSE METASTATIC MODALITIES BY HEPARANASES
    Dario Marchetti; Fiscal Year: 2005
    ..They will provide a framework for the development of polysaccharide-based anti-cancer molecules as important prognostic tools and diagnostic screens for metastatic detection, diagnosis and treatment. ..
  39. Imaging Kinase Activity In Vivo
    William Weiss; Fiscal Year: 2006
    ..unreadable] [unreadable]..
  40. Glioma Immunotherapy Usingengineered T-Cells
    Michael Jensen; Fiscal Year: 2006
    ..These data will form the basis for designing Phase I/II trials using enhanced T-cell dosing schedules, imaging techniques, and surrogate marker endpoints. ..
  41. MULTICENTER TRIAL OF SYNSORB PK IN E COLI RELATED HUS
    Howard Trachtman; Fiscal Year: 2003
    ..The outcome of this trial may justify the use of SYNSORB PK as a safe therapy to ameliorate D+HUS. ..
  42. GENETICS OF A TRANSGENIC MOUSE MODEL FOR NEUROBLASTOMA
    William Weiss; Fiscal Year: 2004
    ..Given the poor outcome associated with childhood neuroblastoma, this work has the potential to increase our understanding of the pathogenesis of neuroblastoma, and may result in improved therapies for children with this disorder. ..
  43. SUBMACULAR SURGERY TRIALS PATHOLOGY CENTER
    Hans Grossniklaus; Fiscal Year: 2003
    ..Additionally, eyes obtained post-mortem from patients enrolled in the SST will be studied for pathologic correlation with clinical findings and outcomes. ..
  44. Novel Therapies for Resistant FSGS
    Howard Trachtman; Fiscal Year: 2006
    ..The infrastructure that is established for the performance of this R21/R33 project should prove useful for the efficient assessment of additional novel therapies that will be developed in the future for patients with primary FSGS. ..
  45. Comparative DNA Microarray Analysis of Brain Tumors
    Daniel Brat; Fiscal Year: 2006
    ....
  46. Control of Eye Melanoma Metastasis
    Hans Grossniklaus; Fiscal Year: 2003
    ..Both the IFN and angiostatin will prevent the progression of micrometastases into metastases by anti-angiogenesis. Angiostatin will prevent metastases by an anti-melanoma invasion effect. ..
  47. Intravascular Thrombosis in Glioblastoma
    Daniel Brat; Fiscal Year: 2005
    ..Our emerging model represents a paradigm shift in the understanding of GBM and should lead directly to more effective therapies. ..
  48. OREGON CHILD HEALTH RESEARCH CENTER
    HENRY NICHOLSON; Fiscal Year: 2005
    ..An effective formal educational program has been implemented and will be provided during the first two years of training for each Junior Investigator. ..
  49. Human monoclonal antibody therapy for HUS
    Howard Trachtman; Fiscal Year: 2004
    ..abstract_text> ..