nerve sheath neoplasms

Summary

Alias: nerve sheath tumors
Summary: Neoplasms which arise from nerve sheaths formed by SCHWANN CELLS in the PERIPHERAL NERVOUS SYSTEM or by OLIGODENDROCYTES in the CENTRAL NERVOUS SYSTEM. Malignant peripheral nerve sheath tumors, NEUROFIBROMA, and NEURILEMMOMA are relatively common tumors in this category.

Top Publications

  1. ncbi International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis
    Rosalie E Ferner
    Department of Neuroimmunology, Guy s, King s and St Thomas s School of Medicine, London, United Kingdom
    Cancer Res 62:1573-7. 2002
  2. ncbi Germline and somatic NF1 mutations in sporadic and NF1-associated malignant peripheral nerve sheath tumours
    Irene Bottillo
    IRCCS CSS, San Giovanni Rotondo and CSS Mendel Institute, 00198 Rome, Italy
    J Pathol 217:693-701. 2009
  3. pmc Molecular profiling of malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1, based on large-scale real-time RT-PCR
    Pascale Lévy
    Laboratoire de Génétique Moléculaire UPRES EA 3618, Faculte des Sciences Pharmaceutiques et Biologiques, Universite Paris V, Paris, France
    Mol Cancer 3:20. 2004
  4. ncbi Identification of a novel amplicon at distal 17q containing the BIRC5/SURVIVIN gene in malignant peripheral nerve sheath tumours
    C T Storlazzi
    Department of Genetics and Microbiology, University of Bari, Italy
    J Pathol 209:492-500. 2006
  5. pmc cDNA microarray analysis of cancer associated gene expression profiles in malignant peripheral nerve sheath tumours
    K Karube
    Department of Pathology, School of Medicine, Fukuoka University, Nanakuma 7 45 1, Jonan ku, Fukuoka 814 0180, Japan
    J Clin Pathol 59:160-5. 2006
  6. ncbi Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues
    Shyra J Miller
    Division of Experimental Hematology, Cincinnati Children s Hospital Research Foundation, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA
    Cancer Res 66:2584-91. 2006
  7. pmc Genetically engineered mouse models shed new light on the pathogenesis of neurofibromatosis type I-related neoplasms of the peripheral nervous system
    Nicole M Brossier
    Department of Cell Biology, University of Alabama at Birmingham, Birmingham, AL 35294 0017, USA
    Brain Res Bull 88:58-71. 2012
  8. pmc Genome-wide transcriptome analyses reveal p53 inactivation mediated loss of miR-34a expression in malignant peripheral nerve sheath tumours
    Subbaya Subramanian
    Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota, USA
    J Pathol 220:58-70. 2010
  9. ncbi Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience
    Chee Chee H Stucky
    Department of General Surgery, Mayo Clinic Arizona, Phoenix, AZ, USA
    Ann Surg Oncol 19:878-85. 2012
  10. ncbi Malignant peripheral nerve sheath tumours in NF1: improved survival in women and in recent years
    Sarah Ingham
    Genetic Medicine, The University of Manchester, Manchester Academic Health Science Centre, Central Manchester Foundation Trust, St Mary s Hospital, UK
    Eur J Cancer 47:2723-8. 2011

Detail Information

Publications241 found, 100 shown here

  1. ncbi International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis
    Rosalie E Ferner
    Department of Neuroimmunology, Guy s, King s and St Thomas s School of Medicine, London, United Kingdom
    Cancer Res 62:1573-7. 2002
    ..syndrome in which affected individuals have a greatly increased risk of developing malignant peripheral nerve sheath tumors (MPNSTs). These cancers are difficult to detect and have a poor prognosis...
  2. ncbi Germline and somatic NF1 mutations in sporadic and NF1-associated malignant peripheral nerve sheath tumours
    Irene Bottillo
    IRCCS CSS, San Giovanni Rotondo and CSS Mendel Institute, 00198 Rome, Italy
    J Pathol 217:693-701. 2009
    ..However, the somatic events share common characteristics with the NF1-related and the sporadic tumours...
  3. pmc Molecular profiling of malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1, based on large-scale real-time RT-PCR
    Pascale Lévy
    Laboratoire de Génétique Moléculaire UPRES EA 3618, Faculte des Sciences Pharmaceutiques et Biologiques, Universite Paris V, Paris, France
    Mol Cancer 3:20. 2004
    ..Plexiform neurofibromas can give rise to malignant peripheral nerve sheath tumors (MPNSTs), and the underlying molecular mechanisms are largely unknown.
  4. ncbi Identification of a novel amplicon at distal 17q containing the BIRC5/SURVIVIN gene in malignant peripheral nerve sheath tumours
    C T Storlazzi
    Department of Genetics and Microbiology, University of Bari, Italy
    J Pathol 209:492-500. 2006
    ....
  5. pmc cDNA microarray analysis of cancer associated gene expression profiles in malignant peripheral nerve sheath tumours
    K Karube
    Department of Pathology, School of Medicine, Fukuoka University, Nanakuma 7 45 1, Jonan ku, Fukuoka 814 0180, Japan
    J Clin Pathol 59:160-5. 2006
    ..Little is known about the underlying biology of MPNST, especially the mechanisms involved in cell proliferation, invasion, or escape from apoptosis...
  6. ncbi Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues
    Shyra J Miller
    Division of Experimental Hematology, Cincinnati Children s Hospital Research Foundation, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA
    Cancer Res 66:2584-91. 2006
    Malignant peripheral nerve sheath tumors (MPNST) are highly invasive soft tissue sarcomas that arise within the peripheral nerve and frequently metastasize...
  7. pmc Genetically engineered mouse models shed new light on the pathogenesis of neurofibromatosis type I-related neoplasms of the peripheral nervous system
    Nicole M Brossier
    Department of Cell Biology, University of Alabama at Birmingham, Birmingham, AL 35294 0017, USA
    Brain Res Bull 88:58-71. 2012
    ..plexiform, but not dermal, neurofibromas often undergo malignant progression to form malignant peripheral nerve sheath tumors (MPNSTs), the most common malignancy occurring in NF1 patients...
  8. pmc Genome-wide transcriptome analyses reveal p53 inactivation mediated loss of miR-34a expression in malignant peripheral nerve sheath tumours
    Subbaya Subramanian
    Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota, USA
    J Pathol 220:58-70. 2010
    ..Collectively, our findings suggest that deregulation of miRNAs has a potential role in the malignant transformation process in peripheral nerve sheath tumours...
  9. ncbi Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience
    Chee Chee H Stucky
    Department of General Surgery, Mayo Clinic Arizona, Phoenix, AZ, USA
    Ann Surg Oncol 19:878-85. 2012
    Malignant peripheral nerve sheath tumors (MPNST) are a rare form of soft tissue sarcoma with few studies reporting on patient outcomes and prognostic variables.
  10. ncbi Malignant peripheral nerve sheath tumours in NF1: improved survival in women and in recent years
    Sarah Ingham
    Genetic Medicine, The University of Manchester, Manchester Academic Health Science Centre, Central Manchester Foundation Trust, St Mary s Hospital, UK
    Eur J Cancer 47:2723-8. 2011
    ..These uncommon tumours are known to occur at high frequency and lead to poor survival. Our aim was to determine risk of MPNST in NF1 patients, and survival rates...
  11. ncbi Expression and significance of EGFR in malignant peripheral nerve sheath tumor
    Daniel Keizman
    Unit of Bone and Soft Tissue Oncology, Division of Oncology, Tel Aviv Sourasky Medical Center TASMC, Affiliated with Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
    J Neurooncol 94:383-8. 2009
    ..EGFR appeared to play a role in MPNST progression. EGFR overexpression was correlated with worse prognostic variables and course. Clinical trials of targeting EGFR in MPNST are warranted...
  12. ncbi Genomic changes in chromosomes 10, 16, and X in malignant peripheral nerve sheath tumors identify a high-risk patient group
    Helge R Brekke
    Department of Cancer Prevention, Institute for Cancer Research, The Norwegian Radium Hospital, Rikshospitalet, Oslo University Hospital, Oslo, Norway
    J Clin Oncol 28:1573-82. 2010
    The purpose of this study was to identify genetic aberrations contributing to clinical aggressiveness of malignant peripheral nerve sheath tumors (MPNSTs).
  13. pmc Genomic and molecular characterization of malignant peripheral nerve sheath tumor identifies the IGF1R pathway as a primary target for treatment
    Jilong Yang
    Departments of Bone and Soft Tissue Tumor, Pathology, and Epidemiology and Biostatistics, Tianjin Medical University Cancer Hospital and Institute, Tianjin, China
    Clin Cancer Res 17:7563-73. 2011
    ..Malignant peripheral nerve sheath tumor (MPNST) is a rare sarcoma that lacks effective therapeutic strategies. We gain insight into the most recurrent genetically altered pathways with the purpose of scanning possible therapeutic targets...
  14. ncbi Immunohistochemical evaluation of canine peripheral nerve sheath tumors and other soft tissue sarcomas
    K Chijiwa
    Department of Veterinary Pathology, Faculty of Agriculture, Miyazaki University, Japan
    Vet Pathol 41:307-18. 2004
    Seventeen cases of canine peripheral nerve sheath tumors (PNSTs), 11 malignant PNSTs (MPNSTs), and six benign PNSTs (BPNSTs) were examined...
  15. pmc CXCR4/CXCL12 mediate autocrine cell- cycle progression in NF1-associated malignant peripheral nerve sheath tumors
    Wei Mo
    Department of Developmental Biology, University of Texas Southwestern Medical Center, Dallas, TX 75390 9133, USA
    Cell 152:1077-90. 2013
    Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise in connective tissue surrounding peripheral nerves. They occur sporadically in a subset of patients with neurofibromatosis type 1 (NF1)...
  16. ncbi High-resolution DNA copy number profiling of malignant peripheral nerve sheath tumors using targeted microarray-based comparative genomic hybridization
    Kiran K Mantripragada
    Institute of Medical Genetics, Cardiff University, School of Medicine, Heath Park, Cardiff, United Kingdom
    Clin Cancer Res 14:1015-24. 2008
    ....
  17. pmc Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1
    Matthias Kolberg
    Department of Cancer Prevention, Institute for Cancer Research, The Norwegian Radium Hospital, Montebello, Oslo University Hospital, NO 0424 Oslo, Norway
    Neuro Oncol 15:135-47. 2013
    ..These observations support the hypothesis that MPNSTs arising in NF1 and non-NF1 patients are not different per se. Consequently, we suggest that the choice of treatment for MPNST should be independent of NF1 status...
  18. ncbi Prognostic significance of AKT/mTOR and MAPK pathways and antitumor effect of mTOR inhibitor in NF1-related and sporadic malignant peripheral nerve sheath tumors
    Makoto Endo
    Departments of Anatomic Pathology and Orthopaedic Surgery, Graduate School of Medical Sciences, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka, Japan
    Clin Cancer Res 19:450-61. 2013
    ..Moreover, we assessed the antitumor effects of the inhibitor of candidate target...
  19. ncbi Zebrafish with mutations in mismatch repair genes develop neurofibromas and other tumors
    Harma Feitsma
    Hubrecht Institute for Developmental Biology and Stem Cell Research, Cancer Genomics Center, Utrecht University, Utrecht, The Netherlands
    Cancer Res 68:5059-66. 2008
    ..Predominantly, neurofibromas/malignant peripheral nerve sheath tumors were observed; however, a range of other tumor types was also observed...
  20. ncbi Delayed rectifier K currents in NF1 Schwann cells. Pharmacological block inhibits proliferation
    Lynne A Fieber
    Division of Marine Biology and Fisheries, University of Miami Rosenstiel School, 4600 Rickenbacker Cswy, Miami, FL 33149, USA
    Neurobiol Dis 13:136-46. 2003
    ....
  21. ncbi Comparative pathology of nerve sheath tumors in mouse models and humans
    Anat O Stemmer-Rachamimov
    Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA
    Cancer Res 64:3718-24. 2004
    ..A novel classification scheme for peripheral nerve sheath tumors in murine models was therefore devised.
  22. ncbi Association between benign and malignant peripheral nerve sheath tumors in NF1
    T Tucker
    Department of Medical Genetics, University of British Columbia, Vancouver, Canada
    Neurology 65:205-11. 2005
    ..The authors conducted a study to determine whether people with NF1 who have benign neurofibromas of various kinds are at greater risk of developing MPNSTs than patients with NF1 who lack these benign tumors...
  23. ncbi Gains in chromosomes 7, 8q, 15q and 17q are characteristic changes in malignant but not in benign peripheral nerve sheath tumors from patients with Recklinghausen's disease
    H Schmidt
    Institute of Pathology, Martin Luther University Halle Wittenberg, Magdeburger Strasse 14, D 06097, Halle, Germany
    Cancer Lett 155:181-90. 2000
    ..genomic hybridization (CGH) gained results from six benign neurofibromas and 14 malignant peripheral nerve sheath tumors (MPNSTs) were compared with four benign peripheral nerve sheath tumors (BPNSTs) from patients without NF1...
  24. ncbi Genetic basis of tumorigenesis in NF1 malignant peripheral nerve sheath tumors
    Meena Upadhyaya
    Institute of Medical Genetics, Cardiff University, Heath Park Campus, Cardiff CF14 4XN, United Kingdom
    Front Biosci (Landmark Ed) 16:937-51. 2011
    Malignant peripheral nerve sheath tumors (MPNSTs), often found associated with neurofibromatosis type 1 (NF1), are aggressive tumors that pose significant diagnostic and therapeutic challenges...
  25. pmc Activated MET is a molecular prognosticator and potential therapeutic target for malignant peripheral nerve sheath tumors
    Keila E Torres
    Department of Cancer Biology, MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 1104, Houston TX 77030, USA
    Clin Cancer Res 17:3943-55. 2011
    MET signaling has been suggested a potential role in malignant peripheral nerve sheath tumors (MPNST). Here, MET function and blockade were preclinically assessed.
  26. ncbi Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution
    Matteo Anghileri
    Department of Surgery, Istituto Nazionale per lo Studio e la Cura dei Tumori, Milan, Italy
    Cancer 107:1065-74. 2006
    The authors explored the prognostic factors and clinical outcomes of patients who had malignant peripheral nerve sheath tumors (MPNST) with and without neurofibromatosis type 1 (NF-1).
  27. ncbi Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome
    Changye Zou
    Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
    Ann Surg 249:1014-22. 2009
    ..Consequently, we sought to identify clinical, pathologic, and molecular predictors of outcome in patients with/without neurofibromatosis type 1 (NF-1) associated MPNST...
  28. pmc First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: an EORTC soft tissue and bone sarcoma group study
    J R Kroep
    Department of Medical Oncology, Leiden University Medical Center, Leiden, The Netherlands
    Ann Oncol 22:207-14. 2011
    ..the role of chemotherapy in advanced malignant peripheral nerve sheath tumor (MPNST) is unclear...
  29. ncbi MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas
    Junji Wasa
    Department of Orthopaedic Surgery, Nagoya University Graduate School and School of Medicine, Showa, Nagoya 466 8550, Japan
    AJR Am J Roentgenol 194:1568-74. 2010
    The objective of this study was to identify the MRI criteria that best differentiate malignant peripheral nerve sheath tumors from benign neurofibromas.
  30. ncbi Gene expression profiling reveals unique molecular subtypes of Neurofibromatosis Type I-associated and sporadic malignant peripheral nerve sheath tumors
    Mark A Watson
    Department of Pathology and Immunology, Washington University School of Medicine, St Louis, MO 63110, USA
    Brain Pathol 14:297-303. 2004
    Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive Schwann cell neoplasms that are frequently associated with Type I Neurofibromatosis (NF1) and respond poorly to current therapeutic regimens...
  31. pmc EGFR and erbB2 in malignant peripheral nerve sheath tumors and implications for targeted therapy
    Nikola Holtkamp
    Institute of Neuropathology, Charite Universitatsmedizin Berlin, CVK, Augustenburger Platz 1, D 13353 Berlin, Germany
    Neuro Oncol 10:946-57. 2008
    Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas with poor prognosis and limited treatment options...
  32. pmc Cancer stem cell-like cells derived from malignant peripheral nerve sheath tumors
    Melanie Spyra
    Department of Maxillofacial Surgery, University Medical Center Hamburg Eppendorf, Hamburg, Germany
    PLoS ONE 6:e21099. 2011
    This study aims to examine whether or not cancer stem cells exist in malignant peripheral nerve sheath tumors (MPNST)...
  33. ncbi TP53 mutation analysis of malignant peripheral nerve sheath tumors
    Robert M Verdijk
    Department of Pathology, Erasmus MC, University, Rotterdam, The Netherlands
    J Neuropathol Exp Neurol 69:16-26. 2010
    Mutations in TP53 underlie the development of malignant peripheral nerve sheath tumors (MPNSTs) in animal models, but there is controversy regarding the extent of TP53 mutations in human MPNSTs...
  34. ncbi Establishment and characterization of a novel human malignant peripheral nerve sheath tumor cell line, FMS-1, that overexpresses epidermal growth factor receptor and cyclooxygenase-2
    Michiyuki Hakozaki
    First Department of Pathology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima shi, Fukushima, 960 1295, Japan
    Virchows Arch 455:517-26. 2009
    ..Thus, FMS-1 cells might be useful for investigating biological behaviors and developing new molecular-targeting antitumor drugs for MPNST expressing EGFR or COX-2...
  35. ncbi Germline and somatic NF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs)
    M Upadhyaya
    Institute of Medical Genetics, Cardiff University, Cardiff, United Kingdom
    Hum Mutat 29:74-82. 2008
    About 10% of neurofibromatosis type 1 (NF1) patients develop malignant peripheral nerve sheath tumors (MPNSTs) and represent considerable patient morbidity and mortality...
  36. pmc Identification of p53 as a strong predictor of survival for patients with malignant peripheral nerve sheath tumors
    Helge R Brekke
    Department of Cancer Prevention, Institute for Cancer Research, Norwegian Radium Hospital, Rikshospitalet University Hospital, Montebello, Oslo, Norway
    Neuro Oncol 11:514-28. 2009
    ..Patients in complete remission with a primary p53-positive MPNST diagnosis may be considered in a high-risk subgroup and candidates for adjuvant treatment...
  37. ncbi Feline peripheral nerve sheath tumors: histologic, immunohistochemical, and clinicopathologic correlation (59 tumors in 53 cats)
    F Y Schulman
    Department of Veterinary Pathology, Armed Forces Institute of Pathology, Washington, DC, USA
    Vet Pathol 46:1166-80. 2009
    Feline peripheral nerve sheath tumors are uncommonly reported, and their clinical behavior has not been well documented. Fifty-nine peripheral nerve sheath tumors were collected from 53 cats...
  38. pmc The 4q12 amplicon in malignant peripheral nerve sheath tumors: consequences on gene expression and implications for sunitinib treatment
    Jan Zietsch
    Department of Neuropathology, Charite Universitatsmedizin Berlin, Berlin, Germany
    PLoS ONE 5:e11858. 2010
    Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive tumors which originate from Schwann cells and develop in about 10% of neurofibromatosis type 1 (NF1) patients...
  39. ncbi Malignant peripheral nerve sheath tumor with divergent mesenchymal differentiations in a dog
    D Y Kim
    Department of Pathobiological Sciences, School of Veterinary Medicine, Louisiana State University, Baton Rouge, LA 70803, USA
    J Vet Diagn Invest 15:174-8. 2003
    ..5-year-old male Labrador Retriever dog. The tumor involved the sixth and seventh cervical spinal cord segments and nerves. The diagnosis was based on the results of histopathology, electron microscopy, and immunohistochemistry...
  40. ncbi Immunohistochemistry for 2',3'-cyclic nucleotide-3'-phosphohydrolase in 63 bovine peripheral nerve sheath tumors
    A B Nielsen
    Department of Veterinary Disease Biology, Section of Pathology, Faculty of Life Sciences, University of Copenhagen, Ridebanevej 3, DK 1870 Frederiksberg C, Denmark
    Vet Pathol 48:796-802. 2011
    To establish a simple and uniform classification of bovine peripheral nerve sheath tumors (PNSTs), 63 tumors from 44 cattle were examined histologically and immunohistochemically with antibodies against S100 protein and 2',3'-cyclic ..
  41. ncbi Malignant nerve-sheath tumor with divergent and glandular differentiation in a dog: a case report
    A K Patnaik
    Department of Pathology, The Animal Medical Center, New York, NY 10021, USA
    Vet Pathol 39:406-10. 2002
    ..The tumor was located in the peripheral nerve roots at the first and second lumbar vertebrae. The dog survived for 161 days after resection of the tumor...
  42. ncbi Genomic imbalances of 7p and 17q in malignant peripheral nerve sheath tumors are clinically relevant
    H Schmidt
    Institute of Pathology, Martin Luther University Halle Wittenberg, Halle, Germany
    Genes Chromosomes Cancer 25:205-11. 1999
    We investigated 31 malignant peripheral nerve sheath tumors (MPNSTs) from 23 patients by means of comparative genomic hybridization (CGH) in order to study quantitative genomic aberrations of these tumors...
  43. pmc PTEN and NF1 inactivation in Schwann cells produces a severe phenotype in the peripheral nervous system that promotes the development and malignant progression of peripheral nerve sheath tumors
    Vincent W Keng
    Masonic Cancer Center, Department of Genetics, Cell Biology and Development, University of Minnesota, Minneapolis, Minnesota 55455, USA
    Cancer Res 72:3405-13. 2012
    ..Pten) and neurofibromatosis 1 (Nf1) genes recently were found to be comutated in high-grade peripheral nerve sheath tumors (PNST) in mice...
  44. pmc Mortality associated with neurofibromatosis 1: a cohort study of 1895 patients in 1980-2006 in France
    Tu anh Duong
    AP HP, Hopital Henri Mondor, Service de Dermatologie, F 94010 Creteil, France
    Orphanet J Rare Dis 6:18. 2011
    ..However, data on mortality in NF1 are limited. Our aim was to evaluate mortality in a large retrospective cohort of NF1 patients seen in France between 1980 and 2006...
  45. pmc Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors
    Gunnar Johansson
    Division of Experimental Hematology, University of Cincinnati, Cincinnati, OH, USA
    Mol Cancer Ther 7:1237-45. 2008
    Malignant peripheral nerve sheath tumors (MPNST) are chemoresistant sarcomas with poor 5-year survival that arise in patients with neurofibromatosis type 1 (NF1) or sporadically...
  46. pmc Autophagic survival in resistance to histone deacetylase inhibitors: novel strategies to treat malignant peripheral nerve sheath tumors
    Gonzalo Lopez
    Department of Cancer Biology, University of Texas, MD Anderson Cancer Center, Houston, Texas 77030, USA
    Cancer Res 71:185-96. 2011
    ..Here we report findings that strongly rationalize clinical evaluation of HDACis in malignant peripheral nerve sheath tumors (MPNST), a class of highly aggressive, therapeutically resistant, and commonly fatal malignancies that ..
  47. ncbi Aberrant expression of CHFR in malignant peripheral nerve sheath tumors
    Chikashi Kobayashi
    Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Maidashi, Fukuoka, Japan
    Mod Pathol 19:524-32. 2006
    ..In addition, MPNST with normal karyotype showed a strong (score, =5) expression of CHFR. Our results support the assertion that CHFR functions as an inhibitor of tumor proliferation...
  48. ncbi Clinical relevance of pathological grades of malignant peripheral nerve sheath tumor: a multi-institution TMTS study of 56 cases in Northern Japan
    Kyoji Okada
    Tohoku Musculoskeletal Tumor Society, Yamagata, Japan
    Ann Surg Oncol 14:597-604. 2007
    ..Malignant peripheral nerve sheath tumor (MPNST) is a relatively rare soft tissue tumor, and its clinical relevance of pathological grades remains obscure...
  49. ncbi Mouse models of tumor development in neurofibromatosis type 1
    K Cichowski
    Department of Biology and Center for Cancer Research and Howard Hughes Medical Institute, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
    Science 286:2172-6. 1999
    ..Hallmark features of the disease are the development of benign peripheral nerve sheath tumors (neurofibromas), which can progress to malignancy...
  50. ncbi Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment
    Joachim M Baehring
    Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
    Neurology 61:696-8. 2003
    Malignant peripheral nerve sheath tumors (MPNST) are derived from Schwann cells or pluripotent cells of the neural crest. Delay of diagnosis is common, especially in lesions affecting proximal parts of the peripheral nervous system...
  51. ncbi Array-based comparative genomic hybridization identifies CDK4 and FOXM1 alterations as independent predictors of survival in malignant peripheral nerve sheath tumor
    Jinsheng Yu
    Department of Pathology and Immunology, Washington University School of Medicine, Saint Louis, Missouri, USA
    Clin Cancer Res 17:1924-34. 2011
    Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive sarcomas with variable patient survival and few known prognostically relevant genomic biomarkers...
  52. pmc Diagnostic and prognostic gene expression signatures in 177 soft tissue sarcomas: hypoxia-induced transcription profile signifies metastatic potential
    Princy Francis
    Department of Oncology, Institute of Clinical Sciences, Lund University, Lund, Sweden
    BMC Genomics 8:73. 2007
    ..We assessed the diagnostic and prognostic value of array-based gene expression profiles using 27 k cDNA microarrays in 177, mainly high-grade, STS of 13 histopathological subtypes...
  53. ncbi Expression patterns of cell cycle components in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors
    Trude Holmeide Agesen
    Department of Genetics, Institute for Cancer Research, Norwegian Radium Hospital, Montebello, 0310 Oslo, Norway
    J Neuropathol Exp Neurol 64:74-81. 2005
    The molecular biology underlying the development of highly malignant peripheral nerve sheath tumors (MPNSTs) remains mostly unknown...
  54. pmc Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: results of a pooled analysis from United States and European groups
    Andrea Ferrari
    Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy
    Eur J Cancer 47:724-31. 2011
    ..Various international research groups pooled their data in a joint study in order to investigate prognostic variables and treatment modalities...
  55. ncbi Prognostic significance of p14ARF, p15INK4b, and p16INK4a inactivation in malignant peripheral nerve sheath tumors
    Makoto Endo
    Departments of Anatomic Pathology and Orthopaedic Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka Sanno Hospital, Fukuoka, Japan
    Clin Cancer Res 17:3771-82. 2011
    ..Here we carried out a genetic, epigenetic, and expression analysis of p14(ARF), p15(INK4b), and p16(INK4a), and clarified the prognostic significance of their inactivation in MPNSTs...
  56. ncbi Cytogenetic study of malignant triton tumor: a case report
    Mary H Haddadin
    Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, 600 N Wolfe Street Carnegie 367, Baltimore MD 21287, USA
    Cancer Genet Cytogenet 144:100-5. 2003
    ..2, 17q11.2, and 19q13.1. FISH showed high increase of copy number for MYC and loss of a single copy for TP53...
  57. ncbi Immunohistochemical demonstration of EMA/Glut1-positive perineurial cells and CD34-positive fibroblastic cells in peripheral nerve sheath tumors
    Takanori Hirose
    Department of Pathology, Saitama Medical School, Saitama, Japan
    Mod Pathol 16:293-8. 2003
    ..Glut1 as well as EMA are specific to perineurial cells, and CD34 seems to be immunoreactive to endoneurial fibroblasts...
  58. pmc Identification of genes potentially involved in the increased risk of malignancy in NF1-microdeleted patients
    Eric Pasmant
    Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, Paris, France
    Mol Med 17:79-87. 2011
    ..develop more neurofibromas at a younger age, and have an increased risk of malignant peripheral nerve sheath tumors (MPNSTs)...
  59. ncbi Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review
    Amir Minovi
    Department of Otorhinolaryngology, Head and Neck and Facial Plastic Surgery, Klinikum Fulda gAG, Teaching Hospital of the Philipps University Marburg, Pacelliallee 4, 36043 Fulda, Germany
    Head Neck 29:439-45. 2007
    This study analyzes the management and outcomes of a series of 10 malignant peripheral nerve sheath tumors (MPNST) of the head and neck.
  60. ncbi Characterization of the somatic mutational spectrum of the neurofibromatosis type 1 (NF1) gene in neurofibromatosis patients with benign and malignant tumors
    Meena Upadhyaya
    Institute of Medical Genetics, University of Wales College of Medicine, Cardiff, UK
    Hum Mutat 23:134-46. 2004
    ..type 1 (NF1) is benign neurofibromas, 10-20% of which become transformed into malignant peripheral nerve sheath tumors (MPNSTs). The molecular basis of NF1 tumorigenesis is, however, still unclear...
  61. ncbi Mutation and expression of PDGFRA and KIT in malignant peripheral nerve sheath tumors, and its implications for imatinib sensitivity
    Nikola Holtkamp
    Institute of Neuropathology, Charite Universitatsmedizin Berlin, Germany
    Carcinogenesis 27:664-71. 2006
    ..In summary, PDGFRA, PDGF and KIT dysregulation as well as growth inhibition of cell culture S462 by imatinib may suggest that MPNST patients benefit from treatment with imatinib...
  62. pmc PDGFRA, PDGFRB, EGFR, and downstream signaling activation in malignant peripheral nerve sheath tumor
    Federica Perrone
    Experimental Molecular Pathology, Department of Pathology, Fondazione IRCCS Istituto Nazionale dei Tumori, 20133 Milan, Italy
    Neuro Oncol 11:725-36. 2009
    ..B (PDGFRB), epidermal growth factor receptor (EGFR), and their downstream pathways in malignant peripheral nerve sheath tumors (MPNSTs)...
  63. ncbi Malignant peripheral nerve sheath tumors
    Gaurav Gupta
    Department of Neurological Surgery, University of Medicine and Dentistry of New Jersey, 90 Bergen Street, Suite DOC 8100, Newark, NJ 07103, USA
    Neurosurg Clin N Am 19:533-43, v. 2008
    Malignant peripheral nerve sheath tumors (MPNSTs) are a rare variety of soft tissue sarcoma of ectomesenchymal origin...
  64. ncbi Immunohistochemical and molecular analysis of p53, RB, and PTEN in malignant peripheral nerve sheath tumors
    Christian Mawrin
    Department of Neuropathology, Otto von Guericke University, Magdeburg, Germany
    Virchows Arch 440:610-5. 2002
    The molecular basis of both sporadic and neurofibromatosis type 1 (NF1)-associated malignant peripheral nerve sheath tumors (MPNSTs) is yet largely undetermined...
  65. ncbi Cytogenetic characterization of peripheral nerve sheath tumours: a report of the CHAMP study group
    F Mertens
    Department of Clinical Genetics, University Hospital, Lund, Sweden
    J Pathol 190:31-8. 2000
    ..The cytogenetic findings in peripheral nerve sheath tumours show that the karyotype is a good discriminator between BPNSTs and MPNSTs, and that the pattern of aberrations among the latter may add prognostic information...
  66. ncbi Prognostic relevance of FDG PET in patients with neurofibromatosis type-1 and malignant peripheral nerve sheath tumours
    Winfried Brenner
    Department of Nuclear Medicine, University Medical Center Hamburg Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany
    Eur J Nucl Med Mol Imaging 33:428-32. 2006
    ..Since histopathology and tumour grading are not well correlated with prognosis, we aimed to evaluate the potential of (18)F-fluorodeoxyglucose positron emission tomography (FDG PET) for prediction of patient outcome in MPNST...
  67. ncbi Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors
    Brigitte C Widemann
    Pediatric Oncology Branch, National Cancer Institute, 10 Center Drive, Bethesda, MD 20892, USA
    Curr Oncol Rep 11:322-8. 2009
    Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive soft tissue sarcomas that rarely occur in the general population but have a lifetime incidence of 8% to 13% in those with neurofibromatosis type 1 (NF1)...
  68. pmc Further evidence of the increased risk for malignant peripheral nerve sheath tumour from a Scottish cohort of patients with neurofibromatosis type 1
    J A McCaughan
    J Med Genet 44:463-6. 2007
    ..This may be due to patients with NF1 presenting later, because the tumour is mistaken for a neurofibroma, or due to MPNST having a more aggressive course in NF1...
  69. ncbi Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1
    L Valeyrie-Allanore
    Department of Pathology, Paris XII University, Henri Mondor Hospital, AP HP, F 94010 Créteil Cedex, France
    Br J Dermatol 153:79-82. 2005
    ..Plexiform neurofibromas are common complications of NF1. Their malignant transformation is the main cause of mortality in adult patients with NF1...
  70. ncbi Establishment of three malignant peripheral nerve sheath tumor cell lines, FU-SFT8611, 8710 and 9817: conventional and molecular cytogenetic characterization
    Mikiko Aoki
    Department of Pathology, Fukuoka University School of Medicine, Jonan ku, Fukuoka 814 0180, Japan
    Int J Oncol 29:1421-8. 2006
    ..These newly established cell lines provide a valuable resource for biological and pathological investigations into new treatment regimes for MPNST...
  71. ncbi Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases
    Bernd W Scheithauer
    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA
    Am J Surg Pathol 33:325-38. 2009
    Malignant peripheral nerve sheath tumors (MPNSTs) arising from cranial nerves or their branches are very uncommon. The literature consists mainly of isolated case reports and small series...
  72. ncbi Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review
    Arbia Ziadi
    Department of Otolaryngology Head and Neck Surgery, Montreal University Hospital Center CHUM Notre Dame Hospital, Montreal University, 1560 Sherbrooke East, Montreal, Quebec H2L 4M1, Canada
    Auris Nasus Larynx 37:539-45. 2010
    ....
  73. ncbi Computer-assisted cytogenetic analysis of 51 malignant peripheral-nerve-sheath tumors: sporadic vs. neurofibromatosis-type-1-associated malignant schwannomas
    B E Plaat
    Department of Pathology, University of Groningen, Groningen, The Netherlands
    Int J Cancer 83:171-8. 1999
    ..2), is not a common cytogenetic finding in NF-1-associated MPNSTs. The observed differences between NF-1-associated and sporadic MPNSTs might reflect different oncogenetic pathways...
  74. ncbi Expression of insulin-like growth-factor-1 receptor (IGF-1R) in peripheral nerve sheath tumors in neurofibromatosis type 1
    R E Friedrich
    Maxillofacial Surgery Clinic, Eppendorf University Hospital, University of Hamburg, Germany
    Anticancer Res 27:2085-90. 2007
    ..type 1 (NF1) is an autosomal-dominant inherited disease, characterised by the development of nerve sheath tumors. NF1 is the most frequently inherited disease associated with a predisposition for cancer (in particular ..
  75. ncbi High grade malignant peripheral nerve sheath tumors: outcome of 62 patients with localized disease and review of the literature
    A Longhi
    Musculoskeletal Department, Chemotherapy Division, Istituto Ortopedico Rizzoli, Bologna, Italy
    J Chemother 22:413-8. 2010
    ..New drugs employed successfully in advanced mpNSt should be employed also in the adjuvant setting...
  76. ncbi Tumor suppressor mutations and growth factor signaling in the pathogenesis of NF1-associated peripheral nerve sheath tumors: II. The role of dysregulated growth factor signaling
    Steven L Carroll
    Division of Neuropathology, Department of Pathology, University of Alabama School of Medicine, Birmingham, Alabama 35294 0017, USA
    J Neuropathol Exp Neurol 64:1-9. 2005
    ..develop multiple neurofibromas that can transform into aggressive sarcomas known as malignant peripheral nerve sheath tumors (MPNSTs)...
  77. ncbi The value of MDM2 and CDK4 amplification levels using real-time polymerase chain reaction for the differential diagnosis of liposarcomas and their histologic mimickers
    Shio Shimada
    Department of Pathology, Saitama Medical University School of Medicine, Saitama 350 0495, Japan
    Hum Pathol 37:1123-9. 2006
    ..we analyzed 19 liposarcomas, 21 malignant fibrous histiocytomas, 3 leiomyosarcomas, 5 malignant peripheral nerve sheath tumors, 23 lipomas, and 28 nonneoplastic fat tissues using real-time polymerase chain reaction (PCR) and ..
  78. pmc Expression of p27(kip) and other cell cycle regulators in malignant peripheral nerve sheath tumors and neurofibromas: the emerging role of p27(kip) in malignant transformation of neurofibromas
    H P Kourea
    Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
    Am J Pathol 155:1885-91. 1999
    There is little information regarding the status of cell cycle regulators in malignant peripheral nerve sheath tumors (MPNSTs) and neurofibromas (NFs)...
  79. ncbi Malignant triton tumor of cervical spine with hemorrhage
    Amrita Ghosh
    Department of Neuropathology, National Institute of Mental Health and Neurosciences NIMHANS, Hosur Road, Bangalore 560 029, India
    J Clin Neurosci 18:721-3. 2011
    ..He underwent surgery with decompression of the intradural, and a small part of the extradural, component. To our knowledge this is the first report of a cervical triton tumor presenting with an intratumoral bleed...
  80. ncbi Cytogenetic analysis of a malignant triton tumor and a malignant peripheral nerve sheath tumor and a review of the literature
    E N McComb
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha 68198 5440, USA
    Cancer Genet Cytogenet 91:8-12. 1996
    Cytogenetic analyses of malignant peripheral nerve sheath tumors (MPNST) and malignant triton tumors (MTT) are few to date...
  81. ncbi [Malignant Triton tumor of the nose--case report]
    Małgorzata Potocka
    Katedra i Klinika Chorób Uszu, Nosa, Gardła i Krtani AM w Gdańsku
    Otolaryngol Pol 63:165-7. 2009
    ..Histopathological diagnosis of the tumor was made on the surgical specimen, based on immunohistochemical analysis. Radiation therapy as the next step of treatment of choice was performed in presented case...
  82. ncbi Malignant peripheral nerve sheath tumors in childhood: 13 cases from a single center
    Hac Ahmet Demir
    Department of Pediatric Oncology, Hacettepe University, Institute of Oncology, Ankara, Turkey
    J Pediatr Hematol Oncol 34:204-7. 2012
    We aimed to review clinical characteristics, treatment results, and outcome for pediatric patients with malignant peripheral nerve sheath tumors (MPNSTs).
  83. ncbi Acquisition of i(8q) as an early event in malignant triton tumors
    Elisabetta Magrini
    Department of Oncologic Science, Section of Anatomic Pathology, Bellaria Hospital, University of Bologna, Via Altura 3, 40139 Bologna, Italy
    Cancer Genet Cytogenet 154:150-5. 2004
    ..MTT is a histological variant of malignant peripheral nerve sheath tumors (MPNST). No characteristic cytogenetic anomaly has been detected in MPNST or MTT...
  84. ncbi An orthotopic xenograft model of intraneural NF1 MPNST suggests a potential association between steroid hormones and tumor cell proliferation
    George Q Perrin
    Department of Neuroscience, College of Medicine, University of Florida, Gainesville, FL 32610 0244, USA
    Lab Invest 87:1092-102. 2007
    Malignant peripheral nerve sheath tumors (MPNST) are the most aggressive cancers associated with neurofibromatosis type 1 (NF1)...
  85. pmc Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass
    Jong Gwang Kim
    Department of Oncology Hematology, Kyungpook National University Hospital, Daegu, Korea
    Korean J Intern Med 20:100-4. 2005
    ..The patients were treated with chemotherapy followed by radiotherapy. However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy...
  86. ncbi Genetic and phenotypic characterization of tumor cells derived from malignant peripheral nerve sheath tumors of neurofibromatosis type 1 patients
    Silke Frahm
    Laboratory of Brain Tumor Biology, Department of Neurosurgery, University Hospital Eppendorf, Hamburg, Germany
    Neurobiol Dis 16:85-91. 2004
    Neurofibromatosis type 1 (NF1) patients have an 8-13% lifetime risk of developing malignant peripheral nerve sheath tumors (MPNST) which have a very poor prognosis...
  87. pmc Tamoxifen inhibits malignant peripheral nerve sheath tumor growth in an estrogen receptor-independent manner
    Stephanie J Byer
    Department of Pathology, University of Alabama at Birmingham, 1720 Seventh Avenue South, SC930G3, Birmingham, AL 35294 0017, USA
    Neuro Oncol 13:28-41. 2011
    Few therapeutic options are available for malignant peripheral nerve sheath tumors (MPNSTs), the most common malignancy associated with neurofibromatosis type 1 (NF1)...
  88. ncbi Cytohistologic correlations of 24 malignant peripheral nerve sheath tumor (MPNST) in 17 patients: the Institut Curie experience
    Jerzy Klijanienko
    Department of Tumor Biology, Unité de Cytopathologie et Cytométrie Clinique, Institut Curie, Paris, France
    Diagn Cytopathol 27:103-8. 2002
    ....
  89. ncbi Podoplanin and CD34 in peripheral nerve sheath tumours: focus on neurofibromatosis 1-associated atypical neurofibroma
    Urs Naber
    Institute of Neuropathology, University Medical Center Hamburg Eppendorf, Martinistr 52, 20246, Hamburg, Germany
    J Neurooncol 103:239-45. 2011
    ..Podoplanin expression in atypical neurofibroma was not associated with tumour progression towards MPNST...
  90. ncbi Differential NF1, p16, and EGFR patterns by interphase cytogenetics (FISH) in malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms
    Arie Perry
    Department of Pathology, Washington University School of Medicine, St Louis, Missouri 63110 1093, USA
    J Neuropathol Exp Neurol 61:702-9. 2002
    Malignant peripheral nerve sheath tumors (MPNSTs) are diagnostically challenging neoplasms for which sensitive and specific immunohistochemical markers are lacking...
  91. ncbi Oncolytic HSV and erlotinib inhibit tumor growth and angiogenesis in a novel malignant peripheral nerve sheath tumor xenograft model
    Yonatan Y Mahller
    Division of Hematology Oncology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio, USA
    Mol Ther 15:279-86. 2007
    Malignant peripheral nerve sheath tumors (MPNSTs), driven in part by hyperactive Ras and epidermal growth factor receptor (EGFR) signaling, are often incurable...
  92. ncbi Malignant peripheral nerve sheath tumor: an unusual cause of intussusception
    Dana A Telem
    The Mount Sinai Hospital, Department of Surgery, One Gustave Levy Plaza Box 1259, New York, NY 10029, USA
    J Gastrointest Surg 12:1609-11. 2008
    Malignant peripheral nerve sheath tumors (MPNST) are defined as any tumor arising from a peripheral nerve or showing nerve sheath differentiation. The majority of these tumors arise on the trunk, extremities, or head and neck region...
  93. ncbi Molecular, genetic, and cellular pathogenesis of neurofibromas and surgical implications
    Oren N Gottfried
    Department of Neurosurgery, University of Utah, Salt Lake City, Utah, USA
    Neurosurgery 58:1-16; discussion 1-16. 2006
    ..summarizes the most recent NF1 and neurofibroma literature describing the pathogenesis and treatment of nerve sheath tumors. Understanding the biological underpinnings of tumorigenesis in NF1 has implications for future surgical ..
  94. ncbi p15INK4b, p14ARF, and p16INK4a inactivation in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors
    Federica Perrone
    Unit of Experimental Molecular Pathology, Department of Pathology, Istituto Nazionale per lo Studio e la Cura dei Tumori di Milano, Via G Venezian 1, 20133 Milan, Italy
    Clin Cancer Res 9:4132-8. 2003
    ....
  95. ncbi Molecular evolution of a neurofibroma to malignant peripheral nerve sheath tumor (MPNST) in an NF1 patient: correlation between histopathological, clinical and molecular findings
    Gill Spurlock
    Institute of Medical Genetics, Cardiff University, Heath Park, Cardiff, CF14 4XN Wales, UK
    J Cancer Res Clin Oncol 136:1869-80. 2010
    ..This study analyzed the genetic alterations associated with this pathological heterogeneity in order to identify the genetic processes involved in transformation from a benign to an aggressive malignant tumor...
  96. pmc MMP-13 and p53 in the progression of malignant peripheral nerve sheath tumors
    Nikola Holtkamp
    Institute of Neuropathology, Charite Universitatsmedizin Berlin, Berlin, Germany
    Neoplasia 9:671-7. 2007
    Malignant peripheral nerve sheath tumors (MPNST) are sarcomas with poor prognosis and limited treatment options. Factors contributing to tumor progression are largely unknown...
  97. ncbi Surgical management of malignant peripheral nerve sheath tumors
    John Gachiani
    Department of Neurosurgery, Louisiana State University Health Sciences Center, New Orleans, Louisiana, USA
    Neurosurg Focus 22:E13. 2007
    The aim of this study was to describe the presentation of patients harboring soft tissue sarcomas involving the nerves, most of which were malignant peripheral nerve sheath tumors (MPNSTs), and provide an algorithm for their treatment.
  98. ncbi Chromosomal imbalances in malignant peripheral nerve sheath tumor detected by metaphase and microarray comparative genomic hybridization
    Yasuko Nakagawa
    Science of Functional Recovery and Reconstruction, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700 8558, Japan
    Oncol Rep 15:297-303. 2006
    Malignant peripheral nerve sheath tumors (MPNSTs) are highly malignant tumors affecting adolescents and adults. There have been a few reports on chromosomal aberrations of MPNSTs; however, the tumor-specific alteration remains unknown...
  99. ncbi Malignant peripheral nerve sheath tumor of the breast
    Heriberto Medina-Franco
    Department of Surgery, National Institute of Medical Sciences and Nutrition Salvador Zubiran, Mexico City, Mexico
    Breast J 9:332. 2003
  100. ncbi Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): a 10-year experience
    J N Vauthey
    Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York 10021, USA
    Ann Surg Oncol 2:126-31. 1995
    Prior studies have shown a high rate of local recurrence and a dismal overall prognosis in malignant peripheral nerve sheath tumors (MPNSTs).
  101. ncbi Malignant peripheral nerve sheath tumor of breast in patient without Von Recklinghausen's neurofibromatosis: a case report
    C Thanapaisal
    Department of Surgery, Faculty of Medicine, Khon Kaen University, Thailand
    J Med Assoc Thai 89:377-9. 2006
    ..The patient was referred for radiation therapy after simple mastectomy...

Research Grants38

  1. Study of Anti-Survival signals in NF1
    CHANGYAN CHEN; Fiscal Year: 2013
    ....
  2. The Roles of Tumor Microenvironment in Neurofibroma Development and Therapeutics
    Lu Le; Fiscal Year: 2013
    ..Dermal and plexiform neurofibromas, as well as malignant peripheral nerve sheath tumors and other malignant tumors are serious complications of NF1...
  3. Evaluating the role of NRG-1 receptors in MPNST tumorigenesis
    STEPHANIE NICOLE BROSIUS; Fiscal Year: 2013
    ..the role of neuregulin-1 (NRG-1) receptors in the initiation and propagation of malignant peripheral nerve sheath tumors (MPNSTs)...
  4. Novel Treatment of NF-1 Associated Malignant Peripheral Nerve Sheath Tumors
    Steven L Carroll; Fiscal Year: 2012
    ..type 1 (NF1) develop benign tumors of peripheral nerve known as neurofibromas and malignant peripheral nerve sheath tumors (MPNSTs), a highly aggressive form of Schwann cell neoplasm that arises from neurofibromas...
  5. p21-Activated Kinases as New Therapeutic Targets in Neurofibromatosis Type 1
    JONATHAN D CHERNOFF; Fiscal Year: 2013
    ..alterations in vitro;and 3) We will cross Krox20-cre/Nf1flox/- mice, which develop malignant peripheral nerve sheath tumors, with Pak1 knock out mice, or transplant such NF1 mice with Pak1-/- bone marrow cells, to determine if loss ..
  6. The role of mTORC1 in the development and therapeutic targeting of NF1-associated
    Yuan Zhu; Fiscal Year: 2013
    ..The hallmark feature of Neurofibromatosis type 1 (NF1) is the development of multiple benign peripheral nerve sheath tumors (PNST), also known as neurofibromas, in the peripheral nervous system (PNS)...
  7. Characterizing the role of specific Ras proteins in neurofibroma and MPNST format
    Preeti Tandon; Fiscal Year: 2013
    ..The overall goal of this research is to delineate the functions of Ras proteins in NF1 pathogenesis and to identify novel therapeutic strategies based on inhibition of specific Ras proteins. ..
  8. NF Center: from animal models to therapeutics
    Luis F Parada; Fiscal Year: 2013
    ..and cellular mechanisms by which NF1 mutation engenders plexiform neurofibromas and malignant peripheral nerve sheath tumors. Project 2 (PI, D...
  9. Axl receptor signaling in MPNST;potential node for therapy
    KEILA ENITT TORRES; Fiscal Year: 2013
    ..I have chosen to accept the sarcoma challenge, focusing specifically on malignant peripheral nerve sheath tumors (MPNST)...
  10. MITOGENIC ACTIVITIES IN NEUROFIBROMATOSIS
    Nancy Ratner; Fiscal Year: 2013
    ..type 1 (NF1) carry mutations in the NF1 tumor suppressor gene and develop benign peripheral nerve sheath tumors called neurofibromas which can transform to malignant peripheral nerve sheath tumors (MPNST)...
  11. Identification of Drug Targets for NF1
    Yolanda Sanchez; Fiscal Year: 2010
    ..Progression of plexiform neurofibromas to malignant peripheral nerve sheath tumors (MPNST) occurs in 8-13% of patients...
  12. CANCER IN DAMSELFISH--IMMUNOLOGY AND IN VITRO MODELS
    MICHAEL SCHMALE; Fiscal Year: 1993
    ..Model systems of peripheral nerve sheath tumors (such as neurofibromas and schwannomas) using fishes are of special interest because mammalian models of ..
  13. Identifying Therapeutic Targets for MPNST
    Shyra Miller; Fiscal Year: 2007
    ..analysis of tumor cells by microarray technology identifies therapeutic targets for malignant peripheral nerve sheath tumors (MPNST)...
  14. Targeting Oncolytic HSV to Neuroblastoma & Malig. Peripheral Nerve Sheath Tumors
    ARTURO RAFAEL MALDONADO; Fiscal Year: 2010
    ..5 construct is enhanced by hypoxia, via HIF-1 upregulation. AIM 3: To determine the anti-tumor effects and biosafety of this novel oHSV-34.5 vector xenograft models of Nb and MPNST. ..
  15. Role of Pak kinases in survival and motility
    JEFFREY M FIELD; Fiscal Year: 2010
    ..and cell motility (3) Determine the roles of Pak and Akt in migration and survival of malignant Peripheral Nerve Sheath Tumors (MPNST) and the Schwann cells from which they originate: Relevance to public health-Several properties ..
  16. Phenotyping & genotype-phenotype correlations in NF1
    Bruce Korf; Fiscal Year: 2006
    ..abstract_text> ..
  17. Identification and Preclinical Evaluation of New Brain Tumor Therapies
    David Gutmann; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  18. PRECLINICAL MODELS FOR HUMAN ASTROCYTOMAS
    David Gutmann; Fiscal Year: 2004
    ..abstract_text> ..
  19. Neurofibromatosis Foundation International Consortium
    David Gutmann; Fiscal Year: 2003
    ..abstract_text> ..
  20. PROTEIN 4.1 TUMOR SUPPRESSORS IN MENINGIOMA PATHOGENESIS
    David Gutmann; Fiscal Year: 2003
    ..and (3) analyzing the ability of DAL-1 to impair cell growth and motility. These studies are collectively designed to define the role of this novel family of growth regulators in meningioma tumorigenesis and progression. ..
  21. FUNCTIONAL ANALYSIS OF NF2 GENE MUTATIONS
    David Gutmann; Fiscal Year: 2003
    ..Our ability to design rational therapies for schwannomas and meningiomas is dependent on an improved understanding of the mechanisms by which loss of merlin expression and function promotes tumor formation. ..
  22. NEUROFIBROMIN AS A NEGATIVE REGULATOR FOR ASTROCYTES
    David Gutmann; Fiscal Year: 2002
    ....
  23. Molecular Determinants of Neural Stem Cell Function
    David Gutmann; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable] [unreadable]..
  24. Role of Neuregulin-1 in Schwann Cell Neoplasia
    Steven Carroll; Fiscal Year: 2008
    unreadable] DESCRIPTION (provided by applicant): Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive Schwann cell neoplasms that occur in patients with neurofibromatosis type 1 (NF1), the most common genetic disease ..
  25. NEUREGULINS AND THE PATHOLOGY OF NERVE INJURY
    Steven Carroll; Fiscal Year: 2002
    ....
  26. Uptake and Processing of Foreign DNA by Mitochondria, a Model System
    MICHAEL SCHMALE; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable]..
  27. CONFERENCE--AQUATIC ANIMAL MODELS OF HUMAN DISEASE
    MICHAEL SCHMALE; Fiscal Year: 2003
    ..The investigators strongly believe the forum provided by this type of interdisciplinary meeting is unique and is critical to the advancement of these diverse model systems as useful resources for the biomedical research community. ..
  28. ENZYMATIC DE-INHIBITION OF AXONAL REGENERATION
    David Muir; Fiscal Year: 2003
    ..The main goal of this project is to prove that application of CSPG-degrading enzymes within injured tissues is an effective therapy to improve axonal regrowth and recovery of function throughout the nervous system. ..
  29. Targeting Vasculogenesis and Interstitial Hypertension in Neurofibromatosis Type1
    Ugur Ozerdem; Fiscal Year: 2007
    ..Malignant peripheral nerve sheath tumors (MPNST) are the most common malignant tumor, and the leading cause of mortality in neurofibromatosis type 1 ..
  30. PTEN and Prostate Cancer Progression
    Hong Wu; Fiscal Year: 2008
    ..abstract_text> ..
  31. MUTANT LINES OF ZEBRAFISH HIGHLY SENSITIVE TO NEOPLASIA
    Jan Spitsbergen; Fiscal Year: 2005
    ..These lines of zebrafish will provide an efficient and inexpensive system for development of new treatment and prevention strategies for cancer, and will help identify new genes controlling growth and differentiation. ..
  32. Animal Model of Transmissable Neurofibromas
    MICHAEL SCHMALE; Fiscal Year: 2006
    ..DNF), will be used to address basic questions in the pathogenesis of neurofibromas, malignant peripheral nerve sheath tumors and chromatophoromas...
  33. American Society for Neurochemistry: Annual Conference Grant
    George DeVries; Fiscal Year: 2005
    ..abstract_text> ..
  34. Arachidonic Acid in Alzheimer's Disease Pathogenesis
    RENE SANCHEZ MEJIA; Fiscal Year: 2006
    ..I will test whether inhibition of PLA2 will prevent and reverse AD-like morphological, molecular, and functional alterations in hAPP mice. [unreadable] [unreadable] [unreadable]..
  35. STUDY OF SAPOSINS' BIOLOGICAL FUNCTIONS
    Xiaoyang Qi; Fiscal Year: 2003
    ..abstract_text> ..
  36. Cellular and Molecular Mechanisms of Neural Development
    George DeVries; Fiscal Year: 2004
    ..abstract_text> ..
  37. Axonal Oligodendrocyte Signaling in Multiple Sclerosis
    George DeVries; Fiscal Year: 2006
    ..e. migration, proliferation, and survival of OPC cells), which eventually will enable researchers to pinpoint areas for therapeutic intervention. [unreadable] [unreadable]..