chordoma

Summary

Summary: A malignant tumor arising from the embryonic remains of the notochord. It is also called chordocarcinoma, chordoepithelioma, and notochordoma. (Dorland, 27th ed)

Top Publications

  1. ncbi Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas
    Fortios Tzortzidis
    Department of Neurosurgery, University of Washington, Seattle, Washington, USA
    Neurosurgery 59:230-7; discussion 230-7. 2006
  2. ncbi Chordoma of the spine: clinicopathological features, diagnosis, and treatment
    Panayiotis J Papagelopoulos
    Dept of Orthopedics, Athens University Medical School, 4 Christovassili St, 154 51 Neo Psychikon, Athens, Greece
    Orthopedics 27:1256-63; quiz 1264-5. 2004
  3. ncbi Chordoma
    Paolo G Casali
    Adult Sarcoma Medical Oncology Unit, Istituto Nazionale dei Tumori, Milano, Italy
    Curr Opin Oncol 19:367-70. 2007
  4. pmc Recurrent chromosomal copy number alterations in sporadic chordomas
    Long Phi Le
    Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, United States of America
    PLoS ONE 6:e18846. 2011
  5. ncbi An integrated functional genomics approach identifies the regulatory network directed by brachyury (T) in chordoma
    Andrew C Nelson
    Randall Division of Cell and Molecular Biophysics, New Hunt s House, King s College London, Guy s Campus, London, SE1 1UL, UK
    J Pathol 228:274-85. 2012
  6. ncbi Chordoma: current concepts, management, and future directions
    Brian P Walcott
    Department of Neurosurgery, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA
    Lancet Oncol 13:e69-76. 2012
  7. ncbi Chordoma: the nonsarcoma primary bone tumor
    Rashmi Chugh
    Department of Internal Medicine, Division of Hematology Oncology, 24 Frank Lloyd Wright Drive, A3400, P O Box 483, Ann Arbor, Michigan 48106, USA
    Oncologist 12:1344-50. 2007
  8. pmc T (brachyury) gene duplication confers major susceptibility to familial chordoma
    Xiaohong R Yang
    Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, MD, USA
    Nat Genet 41:1176-8. 2009
  9. ncbi Phase II study of imatinib in advanced chordoma
    Silvia Stacchiotti
    Fondazione Istitutodi Ricovero e Cura a Carattere Scientifico Istituto Nazionale Tumori, Milan, Italy
    J Clin Oncol 30:914-20. 2012
  10. ncbi Genome-wide analysis of sixteen chordomas by comparative genomic hybridization and cytogenetics of the first human chordoma cell line, U-CH1
    S Scheil
    Institute of Pathology, University Hospitals of Ulm, Ulm, Germany
    Genes Chromosomes Cancer 32:203-11. 2001

Research Grants

  1. Genetic Analysis of Chordoma
    Michael J Kelley; Fiscal Year: 2012
  2. mTOR signaling and sporadic chordomas
    Sangyeul Han; Fiscal Year: 2010
  3. Third International Chordoma Research Workshop: Catalyzing New Advances in Chordo
    EDWARD LES; Fiscal Year: 2011
  4. Brachyury-downstream gene networks in the notochord
    Anna Di Gregorio; Fiscal Year: 2013
  5. Rare Cancer Genetics Registry
    Dianne M Finkelstein; Fiscal Year: 2013
  6. Fourth International Chordoma Research Workshop (ICRW)
    Patricia Cogswell; Fiscal Year: 2013
  7. Phase I/II Trial of ZD1839 and Celecoxib in Ex-Smokers
    Michael Kelley; Fiscal Year: 2006
  8. Measurement of Hypoxia in Non Small Cell Lung Carcinoma
    Michael Kelley; Fiscal Year: 2003
  9. GENETIC ANALYSIS OF HEREDITARY MACROTHROMBOCYTOPENIAS
    Michael Kelley; Fiscal Year: 2002
  10. Hypopituitarism after Moderate and Severe Head Injury
    Daniel Kelly; Fiscal Year: 2008

Detail Information

Publications255 found, 100 shown here

  1. ncbi Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas
    Fortios Tzortzidis
    Department of Neurosurgery, University of Washington, Seattle, Washington, USA
    Neurosurgery 59:230-7; discussion 230-7. 2006
    ..In this study, we evaluated patients' clinical outcome and recurrence rates at long-term follow-up after aggressive microsurgical resection of cranial base chordomas...
  2. ncbi Chordoma of the spine: clinicopathological features, diagnosis, and treatment
    Panayiotis J Papagelopoulos
    Dept of Orthopedics, Athens University Medical School, 4 Christovassili St, 154 51 Neo Psychikon, Athens, Greece
    Orthopedics 27:1256-63; quiz 1264-5. 2004
  3. ncbi Chordoma
    Paolo G Casali
    Adult Sarcoma Medical Oncology Unit, Istituto Nazionale dei Tumori, Milano, Italy
    Curr Opin Oncol 19:367-70. 2007
    To review developments in chordoma treatment.
  4. pmc Recurrent chromosomal copy number alterations in sporadic chordomas
    Long Phi Le
    Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, United States of America
    PLoS ONE 6:e18846. 2011
    The molecular events in chordoma pathogenesis have not been fully delineated, particularly with respect to copy number changes...
  5. ncbi An integrated functional genomics approach identifies the regulatory network directed by brachyury (T) in chordoma
    Andrew C Nelson
    Randall Division of Cell and Molecular Biophysics, New Hunt s House, King s College London, Guy s Campus, London, SE1 1UL, UK
    J Pathol 228:274-85. 2012
    b>Chordoma is a rare malignant tumour of bone, the molecular marker of which is the expression of the transcription factor, brachyury...
  6. ncbi Chordoma: current concepts, management, and future directions
    Brian P Walcott
    Department of Neurosurgery, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA
    Lancet Oncol 13:e69-76. 2012
    b>Chordoma is a rare bone cancer that is aggressive, locally invasive, and has a poor prognosis...
  7. ncbi Chordoma: the nonsarcoma primary bone tumor
    Rashmi Chugh
    Department of Internal Medicine, Division of Hematology Oncology, 24 Frank Lloyd Wright Drive, A3400, P O Box 483, Ann Arbor, Michigan 48106, USA
    Oncologist 12:1344-50. 2007
    ..Radiation therapy may also salvage some patients with local recurrence. One series reported a 2-year actuarial local control rate of 33% for patients treated with proton beam irradiation...
  8. pmc T (brachyury) gene duplication confers major susceptibility to familial chordoma
    Xiaohong R Yang
    Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, MD, USA
    Nat Genet 41:1176-8. 2009
    ..we identified unique duplications of a region on 6q27 in four multiplex families with at least three cases of chordoma, a cancer of presumed notochordal origin...
  9. ncbi Phase II study of imatinib in advanced chordoma
    Silvia Stacchiotti
    Fondazione Istitutodi Ricovero e Cura a Carattere Scientifico Istituto Nazionale Tumori, Milan, Italy
    J Clin Oncol 30:914-20. 2012
    ..To explore the antitumor activity of imatinib in patients with advanced platelet-derived growth factor β (PDGFB)/PDGF receptor β (PDGFRB)-positive chordomas...
  10. ncbi Genome-wide analysis of sixteen chordomas by comparative genomic hybridization and cytogenetics of the first human chordoma cell line, U-CH1
    S Scheil
    Institute of Pathology, University Hospitals of Ulm, Ulm, Germany
    Genes Chromosomes Cancer 32:203-11. 2001
    ..Therefore, the loss of 3p might be an early event in chordoma genesis. The most common gains involved 7q (69%), 20 (50%), 5q (38%), and 12q (38%)...
  11. ncbi Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas
    S Vujovic
    Wolfson Institute for Biomedical Research, University College London, and Department of Oral Pathology, King s College London Dental Institute at Guy s Hospital, WC1E 6BT, UK
    J Pathol 209:157-65. 2006
    ..These data provide compelling evidence that chordomas derive from notochord and demonstrate that brachyury is a specific marker for the notochord and notochord-derived tumours...
  12. ncbi Revisiting chordoma with brachyury, a "new age" marker: analysis of a validation study on 51 cases
    Nirmala A Jambhekar
    Department of Pathology, Tata Memorial Hospital, Parel, Mumbai, India
    Arch Pathol Lab Med 134:1181-7. 2010
    b>Chordoma is a rare, notochordal tumor with a characteristic histomorphology and immunohistochemical profile. At times, it presents a diagnostic challenge, especially in small biopsies...
  13. ncbi Establishment and detailed functional and molecular genetic characterisation of a novel sacral chordoma cell line, MUG-Chor1
    Beate Rinner
    Center for Medical Research, Medical University of Graz, Austria
    Int J Oncol 40:443-51. 2012
    ..To date, only two chordoma cell lines exist world-wide...
  14. ncbi Differential expression of microRNA (miRNA) in chordoma reveals a role for miRNA-1 in Met expression
    Zhenfeng Duan
    Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital, Boston, Massachusetts 02114, USA
    J Orthop Res 28:746-52. 2010
    ..may have important diagnostic, prognostic, and therapeutic value, but there is no data on miRNA expression in chordoma. The purpose of this study was to identify the role of miRNAs in human chordoma...
  15. ncbi Role of the transcription factor T (brachyury) in the pathogenesis of sporadic chordoma: a genetic and functional-based study
    Nadege Presneau
    UCL Cancer Institute, 72 Huntley Street, London WC1 6BT, UK
    J Pathol 223:327-35. 2011
    ..The U-CH1 cell line was validated as representing chordoma by the generation of xenografts, which showed typical chordoma morphology and immunohistochemistry in the NOD/..
  16. ncbi Chordoma: incidence and survival patterns in the United States, 1973-1995
    M L McMaster
    Genetic Epidemiollogy Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
    Cancer Causes Control 12:1-11. 2001
    b>Chordoma, a rare tumor arising from notochordal remnants, has been described to date only by single-institution case series or small population-based surveys.
  17. pmc Identification of nucleus pulposus precursor cells and notochordal remnants in the mouse: implications for disk degeneration and chordoma formation
    Kyung Suk Choi
    Department of Molecular Genetics, University of Florida, College of Medicine, Gainesville, Florida 32610, USA
    Dev Dyn 237:3953-8. 2008
    ..remnants," which in humans have been proposed to give rise to a rare type of late-onset cancer called chordoma. Previously, this cell type had not been identified in the mouse model system...
  18. pmc Carbon ion radiotherapy for sacral chordoma
    R Imai
    Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Anagawa 4 9 1, Inage Ku, Chiba, Japan
    Br J Radiol 84:S48-54. 2011
    ..Here, we present a retrospective analysis of CIRT for sacral chordoma. The study included 95 patients with medically unresectable sacral chordomas treated between 1996 and 2007...
  19. ncbi Generation of chordoma cell line JHC7 and the identification of Brachyury as a novel molecular target
    Wesley Hsu
    Department of Neurosurgery and Oncology, Brain Tumor Stem Cell Laboratory, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21231, USA
    J Neurosurg 115:760-9. 2011
    b>Chordoma is a malignant bone neoplasm hypothesized to arise from notochordal remnants along the length of the neuraxis...
  20. pmc Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review
    Simon G Launay
    Department of Medical Oncology, Institut Paoli Calmettes, 232 Bd Ste Marguerite, Marseille, 13009, France
    BMC Cancer 11:423. 2011
    ..They are characterized by slow growth and long history with frequent local relapses, and sometimes metastases. While chemotherapy is not efficient, imatinib has shown antitumor activity...
  21. ncbi Combination of PI3K/mTOR inhibition demonstrates efficacy in human chordoma
    Joseph Schwab
    Department of Orthopedic Surgery, Section of Orthopedic Oncology, Sarcoma Research Laboratory, Massachusetts General Hospital, Boston, MA 02114, U S A
    Anticancer Res 29:1867-71. 2009
    ..PI-103 is a inhibitor of PI3K/AKT and mTOR activation. This study aims to determine whether the PI3K/mTOR pathway was active in chordomas and whether their inhibition could lead to decreased proliferation and increased apoptosis...
  22. ncbi Skull base chordomas: efficacy of surgery followed by carbon ion radiotherapy
    Satoshi Takahashi
    Department of Neurosurgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku ku, Tokyo 160 8582, Japan
    Acta Neurochir (Wien) 151:759-69. 2009
    ..We report the effectiveness of combined radical skull base surgery with carbon ion radiotherapy for treating skull base chordomas...
  23. ncbi Current clinical evidence for proton therapy
    Michael Brada
    The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, Sutton, Surrey, UK
    Cancer J 15:319-24. 2009
    ..Any complex and expensive technology, including proton therapy, should not be employed on the basis of belief alone and requires testing to avoid inappropriate use of potential detriment to future patients...
  24. ncbi Intraosseous benign notochordal cell tumor
    Hoda Zeinab M Amer
    Department of Pathology and Laboratory Medicine, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, 150 Bergen St, UH E155, Newark, NJ 07103, USA
    Arch Pathol Lab Med 134:283-8. 2010
    ..and described as giant vertebral notochordal rest, giant notochordal hamartoma of intraosseous origin, and benign chordoma ...
  25. ncbi Novel therapeutic targets in chordoma
    Mohamad Bydon
    The Johns Hopkins Hospital, Department of Neurological Surgery, 600 N Wolfe St, Meyer 7 109, Baltimore, MD 21287, USA
    Expert Opin Ther Targets 16:1139-43. 2012
    ..Chordomas are malignant bone tumors arising from notochordal remnants. They most commonly occur at the sacrum, skull base, and spine. The gold standard treatment for these tumors is a combination of en-bloc resection and radiation therapy...
  26. pmc High-resolution whole-genome analysis of skull base chordomas implicates FHIT loss in chordoma pathogenesis
    Roberto Jose Diaz
    Arthur and Sonia Labatt Brain Tumor Research Centre, The Hospital for Sick Children, Toronto, Ontario, Canada
    Neoplasia 14:788-98. 2012
    b>Chordoma is a rare tumor arising in the sacrum, clivus, or vertebrae. It is often not completely resectable and shows a high incidence of recurrence and progression with shortened patient survival and impaired quality of life...
  27. pmc From notochord formation to hereditary chordoma: the many roles of Brachyury
    Yutaka Nibu
    Department of Cell and Developmental Biology, Weill Medical College of Cornell University, New York, NY 10065, USA
    Biomed Res Int 2013:826435. 2013
    b>Chordoma is a rare, but often malignant, bone cancer that preferentially affects the axial skeleton and the skull base...
  28. ncbi Phase II study on lapatinib in advanced EGFR-positive chordoma
    S Stacchiotti
    Sarcoma Unit, Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale Tumori, Via Venezian 1, 20133 Milan, Italy
    Ann Oncol 24:1931-6. 2013
    To report on a prospective, investigator-driven, phase II study on lapatinib in epidermal growth factor receptor (EGFR)-positive advanced chordoma patients.
  29. pmc Establishment and characterization of a primary human chordoma xenograft model
    I Mei Siu
    Department of Neurosurgery, The Johns Hopkins University, Baltimore, MD, USA
    J Neurosurg 116:801-9. 2012
    ..Currently, there are only a few established chordoma cell lines and no primary xenograft model...
  30. ncbi Characterization of cancer stem-like cells in chordoma
    Esra Aydemir
    Department of Genetics and Bioengineering, Yeditepe University, Istanbul, Turkey
    J Neurosurg 116:810-20. 2012
    ..b>Chordoma cells and cancer stem-like cells share similar characteristics, including self-renewal, differentiation, and ..
  31. ncbi A common single-nucleotide variant in T is strongly associated with chordoma
    Nischalan Pillay
    Cancer Institute, University College London, UK
    Nat Genet 44:1185-7. 2012
    b>Chordoma is a rare malignant bone tumor that expresses the transcription factor T. We conducted an association study of 40 individuals with chordoma and 358 ancestry-matched controls, with replication in an independent cohort...
  32. ncbi Chordoma: review of clinicoradiological features and factors affecting survival
    M Y Soo
    Department of Radiology, Westmead Hospital, Westmead, New South Wales, Australia
    Australas Radiol 45:427-34. 2001
    ..Except for complete surgical excision followed by radiotherapy in the subset of patients with sacrospinal tumours, none of the other clinical indicators show a statistical significant influence on survival...
  33. pmc Randomised trial of proton vs. carbon ion radiation therapy in patients with chordoma of the skull base, clinical phase III study HIT-1-Study
    Anna V Nikoghosyan
    Dept of Clinical Radiology, University of Heidelberg, INF 400, 69120 Heidelberg, Germany
    BMC Cancer 10:607. 2010
    ..Proton therapy is the gold standard in the treatment of skull base chordomas. However, high-LET beams such as carbon ions theoretically offer biologic advantages by enhanced biologic effectiveness in slow-growing tumors...
  34. pmc Analysis of receptor tyrosine kinases (RTKs) and downstream pathways in chordomas
    Elena Tamborini
    Experimental Molecular Pathology, Department of Pathology, Fondazione IRCCS Istituto Tumori Milano, Via G Venezian 1, 20133 Milano, Italy
    Neuro Oncol 12:776-89. 2010
    ..families; (ii) support the idea that a combination of upstream antagonists and mTOR inhibitors enhances the control of tumor growth; and (iii) indicate that the 4E-BP1/eIF4E pathway is a major regulator of protein synthesis in chordoma.
  35. ncbi Effectiveness of cetuximab/gefitinib in the therapy of a sacral chordoma
    Holger Hof
    Department of Radiation Oncology, University of Heidelberg, Heidelberg, Germany
    Onkologie 29:572-4. 2006
    ..Chordomas are rare tumors of the skull base and the spine. Treatment is difficult since conventional radiotherapy or chemotherapy have only limited effects...
  36. pmc Potential therapeutic targets for chordoma: PI3K/AKT/TSC1/TSC2/mTOR pathway
    N Presneau
    UCL Cancer Institute, University College London, 72 Huntley Street, London WC1E 6BT, UK
    Br J Cancer 100:1406-14. 2009
    ..The aim of this study was to identify potential molecular targets for the treatment of chordoma. In view of the reported association of chordoma and tuberous sclerosis complex syndrome, and the available ..
  37. ncbi Effectiveness and safety of spot scanning proton radiation therapy for chordomas and chondrosarcomas of the skull base: first long-term report
    Carmen Ares
    Center for Proton Radiation Therapy, Paul Scherrer Institute, Villigen, Switzerland
    Int J Radiat Oncol Biol Phys 75:1111-8. 2009
    ..To evaluate effectiveness and safety of spot-scanning-based proton radiotherapy (PT) in skull-base chordomas and chondrosarcomas...
  38. ncbi Proton radiation therapy for chordomas and chondrosarcomas of the skull base
    E B Hug
    Department of Radiation Medicine, Loma Linda University Medical Center, California 92354, USA
    J Neurosurg 91:432-9. 1999
    ....
  39. ncbi Epidermal growth factor receptor (EGFR) status in chordoma
    Konrad Ptaszynski
    Department of Pathology, Maria Skłodowska Curie Memorial Cancer Center and Institute of Oncology, Warsaw
    Pol J Pathol 60:81-7. 2009
    b>Chordoma is a rare tumour arising from the embryonal remnants of a notochord occurring most commonly in the sacrococcygeal as well as head and neck locations. Current treatment includes surgery and/or proton beam radiotherapy...
  40. ncbi Clinical outcome of sacral chordoma with carbon ion radiotherapy compared with surgery
    Yoshihiro Nishida
    Department of Orthopaedic Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan
    Int J Radiat Oncol Biol Phys 79:110-6. 2011
    To evaluate the efficacy, post-treatment function, toxicity, and complications of carbon ion radiotherapy (RT) for sacral chordoma compared with surgery.
  41. ncbi 'The chicken or the egg?' dilemma strikes back for the controlling mechanism in chordoma(#)
    Karoly Szuhai
    Department of Molecular Cell Biology, Leiden University Medical Center, Einthovenweg 20, 2330 RC, Leiden, The Netherlands
    J Pathol 228:261-5. 2012
    b>Chordoma is a rare malignant tumour of bone showing notochordal differentiation with characteristic expression of the transcription factor brachyury (T)...
  42. ncbi Diagnosing an extra-axial chordoma of the proximal tibia with the help of brachyury, a molecule required for notochordal differentiation
    Roberto Tirabosco
    Department of Radiology, Royal National Orthopaedic Hospital, Stanmore, Middlesex, HA7 4LP, UK
    Skeletal Radiol 36:59-65. 2007
    ..In contrast, making a robust diagnosis of an extra-axial chordoma has been difficult in the absence of specific markers for chordomas...
  43. ncbi Differential proteomic profiling of chordomas and analysis of prognostic factors
    Hua Zhou
    Department of Orthopedics, Peking University Third Hospital, Beijing, China
    J Surg Oncol 102:720-7. 2010
    The recurrence rate of chordoma is high, and the prognosis is poor.
  44. ncbi Reconstruction of extensive partial or total sacrectomy defects with a transabdominal vertical rectus abdominis myocutaneous flap
    Brian S Glatt
    Division of Plastic and Reconstructive Surgery, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY 10021, USA
    Ann Plast Surg 56:526-30; discussion 530-1. 2006
    ..It has a low incidence of complications, low morbidity, and is easy to perform with a high success rate...
  45. ncbi A treatment planning comparison of combined photon-proton beams versus proton beams-only for the treatment of skull base tumors
    Loic Feuvret
    Institut Curie, Centre de protonthérapie d Orsay, Orsay Cedex, France
    Int J Radiat Oncol Biol Phys 69:944-54. 2007
    ..To compare treatment planning between combined photon-proton planning (CP) and proton planning (PP) for skull base tumors, so as to assess the potential limitations of CP for these tumors...
  46. ncbi Current comprehensive management of cranial base chordomas: 10-year meta-analysis of observational studies
    Salvatore Di Maio
    Department of Neurological Surgery, University of Washington, Harborview Medical Center, Seattle, Washington 98104, USA
    J Neurosurg 115:1094-105. 2011
    ..This meta-analysis measures the relationship of complete resection and type of adjuvant radiation therapy to 5-year progression-free survival (PFS) and overall survival (OS) of cranial base chordomas...
  47. ncbi Systemic therapy options for unresectable and metastatic chordomas
    Silvia Stacchiotti
    Sarcoma Unit, Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale Tumori, Via Venezian 1, 20133 Milan, Italy
    Curr Oncol Rep 13:323-30. 2011
    b>Chordoma is an exceedingly rare tumor, marked by a slow growth rate. Surgery is the treatment of choice, but the most frequent sites of origin (spine and skull base) make treatment of primary disease challenging...
  48. pmc Identification of repurposed small molecule drugs for chordoma therapy
    Menghang Xia
    NIH Chemical Genomics Center, National Center for Advancing Translational Sciences, National Institutes of Health, Bethesda, MD, USA
    Cancer Biol Ther 14:638-47. 2013
    b>Chordoma is a rare, slow growing malignant tumor arising from remnants of the fetal notochord. Surgery is the first choice for chordoma treatment, followed by radiotherapy, although postoperative complications remain significant...
  49. pmc Wide resection of sacral chordoma via a posterior approach
    Apichat Asavamongkolkul
    Department of Orthopaedic Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, 10700, Thailand
    Int Orthop 36:607-12. 2012
    The study was carried out to report the results of wide resection in sacral chordoma using a posterior approach and gauze packing technique.
  50. ncbi The role of epidermal growth factor receptor in chordoma pathogenesis: a potential therapeutic target
    Asem Shalaby
    UCL Cancer Institute, 72 Huntley Street, London WC1 6BT, UK
    J Pathol 223:336-46. 2011
    b>Chordoma, the molecular hallmark of which is T (brachyury), is a rare malignant bone tumour with a high risk of local recurrence and a tumour from which metastatic disease is a common late event...
  51. pmc Frequent deletion of the CDKN2A locus in chordoma: analysis of chromosomal imbalances using array comparative genomic hybridisation
    K H Hallor
    Department of Clinical Genetics, Lund University Hospital, Lund SE 221 85, Sweden
    Br J Cancer 98:434-42. 2008
    The initiating somatic genetic events in chordoma development have not yet been identified...
  52. ncbi Operative management of sacral chordoma
    Bruno Fuchs
    Departments of Orthopedics and Surgical Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA
    J Bone Joint Surg Am 87:2211-6. 2005
    Sacrococcygeal chordoma presents a difficult diagnostic and therapeutic problem, with a high rate of local recurrence. The purpose of this report is to define the importance of adequate surgical treatment for optimum outcome and survival.
  53. ncbi Stereotactic radiosurgery for chordoma: a report from the North American Gamma Knife Consortium
    Hideyuki Kano
    Department of Neurological Surgery, and Radiation Oncology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA
    Neurosurgery 68:379-89. 2011
    ..Although considered slow-growing, low-grade malignancies, chordomas are locally aggressive and destructive tumors with high recurrence rates...
  54. ncbi Reconstruction of extensive defects from posterior en bloc resection of sacral tumors with human acellular dermal matrix and gluteus maximus myocutaneous flaps
    Hormuzdiyar H Dasenbrock
    School of Medicine, Johns Hopkins University, Baltimore, Maryland 21287, USA
    Neurosurgery 69:1240-7. 2011
    ..However, reconstruction of the resultant extensive soft-tissue defects is challenging because a vertical rectus abdominis myocutaneous flap is not harvested...
  55. ncbi Analysis of prognostic factors for patients with chordoma with use of the California Cancer Registry
    Joe Lee
    Department of Orthopaedic Surgery, University of California, Irvine, Irvine, California, USA
    J Bone Joint Surg Am 94:356-63. 2012
    b>Chordoma is the most common primary malignant tumor of the spine. It is extremely rare and has been studied primarily in single-institution case series...
  56. ncbi Proton therapy in chordoma of the base of the skull: a systematic review
    Maurizio Amichetti
    ATreP Provincial Agency for Proton Therapy, Via F lli Perini, 181, 38100 Trento, Italy
    Neurosurg Rev 32:403-16. 2009
    b>Chordoma is a rare, slow-growing, locally aggressive, primary bone tumor that arises from the skull base region in approximately 25-35% of cases...
  57. pmc Chordoma and chondrosarcoma gene profile: implications for immunotherapy
    Joseph H Schwab
    Department of Surgery, Orthopedic Service, Memorial Sloan Kettering Cancer Center, New York, NY 10021, USA
    Cancer Immunol Immunother 58:339-49. 2009
    b>Chordoma and chondrosarcoma are malignant bone tumors characterized by the abundant production of extracellular matrix...
  58. ncbi Emerging role of proton beam radiation therapy for chordoma and chondrosarcoma of the skull base
    Quynh Nhu Nguyen
    Department of Radiation Oncology, The University of Texas M D Anderson Cancer Center, Unit 97, 1515 Holcombe Boulevard, Houston, TX 77030, USA
    Curr Oncol Rep 10:338-43. 2008
    ..Compared with other forms of radiation therapy, proton beam therapy has been used to increase the dose delivered to the tumor while elegantly sparing dosing to adjacent critical normal structures...
  59. ncbi Establishment and characterization of a novel chordoma cell line: CH22
    Xianzhe Liu
    Department of Orthopaedic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA
    J Orthop Res 30:1666-73. 2012
    b>Chordoma is a rare primary malignant bone tumor and there exist only a few established human chordoma cell lines. The scarcity of robust chordoma cell lines has limited the ability to study this tumor...
  60. pmc Characterization and analysis of human chordoma cell lines
    Cao Yang
    Department of Orthopaedic Surgery, Sarcoma Biology Laboratory, Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA
    Spine (Phila Pa 1976) 35:1257-64. 2010
    An experimental study to investigate the characterization of 3 chordoma cell lines.
  61. ncbi Clinical results of proton beam therapy for skull base chordoma
    Hiroshi Igaki
    Proton Medical Research Center, University Hospital, University of Tsukuba, Ibaraki, Japan
    Int J Radiat Oncol Biol Phys 60:1120-6. 2004
    To evaluate clinical results of proton beam therapy for patients with skull base chordoma.
  62. ncbi On the cost-effectiveness of Carbon ion radiation therapy for skull base chordoma
    Oliver Jäkel
    Department of Medical Physics in Radiation Oncology, Heidelberg, Germany
    Radiother Oncol 83:133-8. 2007
    The cost-effectiveness of Carbon ion radiotherapy (RT) for patients with skull base chordoma is analyzed.
  63. ncbi The gluteus maximus muscle flap for reconstruction of sacral chordoma defects
    Poh Koon Koh
    Department of Plastic Surgery, Singapore General Hospital, Singapore
    Ann Plast Surg 53:44-9. 2004
    Four patients diagnosed with sacral chordoma underwent reconstruction with the gluteus maximus flap using an approach based on available muscle remnants and their residual blood supply...
  64. ncbi Optimal treatment planning for skull base chordoma: photons, protons, or a combination of both?
    Mylin A Torres
    Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
    Int J Radiat Oncol Biol Phys 74:1033-9. 2009
    ..We compared dosimetry of proton (PR), intensity modulated radiation therapy (IMRT) photon (PH), and combined PR and IMRT PH (PP) irradiation of skull base chordomas to determine the most optimal technique...
  65. ncbi Differential expression of epidermal growth factor receptor, c-Met, and HER2/neu in chordoma compared with 17 other malignancies
    Paul M Weinberger
    Section of Otolaryngology, Yale University, New Haven, CT 06520 8041, USA
    Arch Otolaryngol Head Neck Surg 131:707-11. 2005
    ..To examine the expression of c-Met, c-Erb-b2 (HER2/neu), and epidermal growth factor (EGFR) in a cohort of 12 chordomas, based on the current and future availability of targeted molecular inhibitors...
  66. ncbi Carbon ion radiotherapy for chordomas and low-grade chondrosarcomas of the skull base. Results in 67 patients
    Daniela Schulz-Ertner
    Department of Clinical Radiology, University of Heidelberg, Germany
    Strahlenther Onkol 179:598-605. 2003
    ..To prospectively evaluate outcome and toxicity after carbon ion radiotherapy (RT) in chordomas and low-grade chondrosarcomas...
  67. ncbi A case of coccygeal chondroid chordoma in a cat: morphological and immunohistochemical features
    Antonio Carminato
    Department of Histopathology, Istituto Zooprofilattico Sperimentale delle Venezie, Legnaro, PD, Italy
    J Vet Diagn Invest 20:679-81. 2008
    ..A diagnosis of chondroid chordoma was made based on histomorphological features and immunohistochemical results...
  68. ncbi Coccygeal chordoma in a dog
    John S Munday
    Athens Diagnostic Laboratory, Department of Veterinary Pathology, College of Veterinary Medicine, University of Georgia, Athens, GA 30602, USA
    J Vet Diagn Invest 15:285-8. 2003
    ..The histology and immunohistochemistry are consistent with that reported for chordoma. Although 4 chordomas have previously been reported in dogs, this is the first case in which immunohistochemistry ..
  69. ncbi Suspected metastatic coccygeal chordoma in a ferret (Mustela putorius furo)
    John S Munday
    Athens Diagnostic Laboratory and Department of Veterinary Pathology, College of Veterinary Medicine, University of Georgia, Athens, GA 30602, USA
    J Vet Diagn Invest 16:454-8. 2004
    A chordoma was removed from the tail base of a 6.5-year-old ferret (Mustela putorius furo). A nodule was observed in the area of tumor development when the ferret was purchased at 3 months of age...
  70. ncbi Radiographic, computed tomographic and histopathologic appearance of a presumed spinal chordoma in a dog
    Anthony P Pease
    Affiliated Veterinary Specialists P A, Maitland, FL 32751, USA
    Vet Radiol Ultrasound 43:338-42. 2002
    ..stellate in appearance with vacuolated cytoplasm (physaliferous cells) and mucinous background consistent with a chordoma. Chordoma is a rare, skeletal neoplasm that originates from mesoderm-derived notochord and has been reported in ..
  71. ncbi Chordoma of the spinal column
    Daniel M Sciubba
    Department of Neurological Surgery, Johns Hopkins University, Meyer 7 109, 600 North Wolfe Street, Baltimore, MD 21287, USA
    Neurosurg Clin N Am 19:5-15. 2008
    ....
  72. ncbi Immunohistochemical analysis of receptor tyrosine kinase signal transduction activity in chordoma
    J H Fasig
    Department of Pathology, Vanderbilt University Medical Center, Nashville, TN 37232, USA
    Neuropathol Appl Neurobiol 34:95-104. 2008
    Currently, there are no effective chemotherapeutic protocols for chordoma. Reports of receptor tyrosine kinase (RTK) expression in chordoma suggest that these tumours may respond to kinase inhibitor therapy...
  73. ncbi Response to erlotinib in a patient with treatment refractory chordoma
    Nimit Singhal
    Adelaide Cancer Centre, Ashford, South Australia, Australia
    Anticancer Drugs 20:953-5. 2009
    ..We report a case where treatment with erlotinib, an epidermal growth factor receptor tyrosine kinase inhibitor, induced symptomatic and radiological response in a patient with disease refractory to imatinib and vascular disrupting agent...
  74. ncbi Effect of carbon ion radiotherapy for sacral chordoma: results of Phase I-II and Phase II clinical trials
    Reiko Imai
    Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba, Japan
    Int J Radiat Oncol Biol Phys 77:1470-6. 2010
    To summarize the results of treatment for sacral chordoma in Phase I-II and Phase II carbon ion radiotherapy trials for bone and soft-tissue sarcomas.
  75. ncbi Analysis of the fibroblastic growth factor receptor-RAS/RAF/MEK/ERK-ETS2/brachyury signalling pathway in chordomas
    Asem A E Shalaby
    UCL Cancer Institute, London, UK
    Mod Pathol 22:996-1005. 2009
    ..The key genetic events responsible for the initiation and progression of chordomas remain to be discovered...
  76. pmc Familial chordoma, a tumor of notochordal remnants, is linked to chromosome 7q33
    M J Kelley
    Department of Medicine, Duke University, and Durham Veterans Affairs Hospital, Durham, NC, USA
    Am J Hum Genet 69:454-60. 2001
    b>Chordoma is a rare tumor originating from notochordal remnants that is usually diagnosed during midlife. We performed a genomewide analysis for linkage in a family with 10 individuals affected by chordoma...
  77. ncbi Chordoma and chondrosarcoma: similar, but quite different, skull base tumors
    Kaith Almefty
    Department of Neurosurgery, University of Arkansas for Medical Sciences, Little Rock, Arkansas 72205, USA
    Cancer 110:2457-67. 2007
    b>Chordoma and chondrosarcoma of the skull base are frequently amalgamated because of similar anatomic location, clinical presentation, and radiologic findings. The chondroid chordoma variant has been reported to carry a better prognosis...
  78. ncbi Metastatic disease from chordoma
    Gloria Vergara
    Servicio de Oncologia Radioterapica, Hospital Puerta de Hierro, Madrid, Spain
    Clin Transl Oncol 10:517-21. 2008
    ..When we analyzed our series with 35 chordomas studied and treated between 1975 and 2002, we found three patients that experienced a systemic dissemination...
  79. ncbi Intradural clival chordoma and ecchordosis physaliphora: a challenging differential diagnosis: case report
    Riccardo Ciarpaglini
    Center of Surgery for Pituitary Tumours and Endoscopic Skull Base Surgery, Neurosurgical Department, Bellaria Hospital, Bologna, Italy
    Neurosurgery 64:E387-8; discussion E388. 2009
    ..We describe these 2 entities and their differential diagnoses, clinical courses, and management. This is the first reported case to be treated using a neuroendoscopic technique...
  80. ncbi Clinical and histopathologic features of chordomas in children and young adults
    Robert V Ridenour
    Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Pediatr Dev Pathol 13:9-17. 2010
    b>Chordoma is a tumor of the axial skeleton that is distinctly uncommon in children and adolescents...
  81. ncbi Long-term control of clival chordoma with initial aggressive surgical resection and gamma knife radiosurgery for recurrence
    Eiji Ito
    Department of Neurosurgery, Fukushima Medical University, 1 Hikarigaoka, Fukushima 960 1295, Japan
    Acta Neurochir (Wien) 152:57-67; discussion 67. 2010
    ..This study analyzed long-term outcomes for clival chordomas after initial aggressive surgical resection and gamma knife radiosurgery for recurrence and investigated clinical factors predicting recurrence...
  82. ncbi Intradural drop metastasis of a clival chordoma
    Michael Patrick Martin
    The University of Queensland, Brisbane, Australia
    J Clin Neurosci 16:1105-7. 2009
    A 69-year-old man with a history of clival chordoma presented with cauda equina syndrome. A mass impinging on the cauda equina was detected on MRI scans and it was initially thought to be a sequestered disc...
  83. ncbi Notochordal remnant-derived mass: ecchordosis physaliphora or chordoma?
    Junji Takeyama
    Pathology 38:599-600. 2006
  84. ncbi Cervical chordoma in childhood without typical vertebral bony destruction: case report and review of the literature
    Hua Zhou
    Department of Orthopedics, Peking University Third Hospital, Beijing, China
    Spine (Phila Pa 1976) 34:E493-7. 2009
    ..Case report...
  85. ncbi Residual postoperative tumour volume predicts outcome after high-dose radiotherapy for chordoma and chondrosarcoma of the skull base and spine
    S Potluri
    Oncology Centre Box 193, Addenbrooke s Hospital, Hills Road, Cambridge, UK
    Clin Oncol (R Coll Radiol) 23:199-208. 2011
    ..This study reviewed our outcomes, in relation to residual tumour volume and radiation dose, in order to inform our future practice...
  86. ncbi Does insulin-like growth factor 1 receptor (IGF-1R) targeting provide new treatment options for chordomas? A retrospective clinical and immunohistochemical study
    Susanne Scheipl
    Department of Orthopaedics and Orthopaedic Surgery, Medical University of Graz, Graz, Austria
    Histopathology 60:999-1003. 2012
    The overall prognosis of chordoma is poor, and current treatment options are limited. The insulin-like growth factor 1 receptor (IGF-1R) pathway is important for cell signalling, and attractive for selective inhibition...
  87. pmc Endovascular occlusion of a lacerated primitive trigeminal artery during surgical resection of clival chordoma. a case report
    G Baltsavias
    Department of Neuroradiology, University Hospital of Zurich, Zurich, Switzerland
    Interv Neuroradiol 16:204-7. 2010
    We describe a case of a persistent primitive trigeminal artery (PPTA) coexistent with a clival chordoma. During surgery of the tumor, the partially incorporated PPTA was inadvertently traumatized and ruptured...
  88. ncbi Identifying mechanisms for therapeutic intervention in chordoma: c-Met oncoprotein
    Elena Ostroumov
    From the Centre for Bioengineering Research and Education, University of Calgary, Calgary, Alberta, Canada
    Spine (Phila Pa 1976) 33:2774-80. 2008
    A human sacral chordoma cell line, CCL3, was established and in vitro characterization of c-Met oncoprotein in chordoma cells was performed.
  89. ncbi Intradural spinal seeding of a clival chordoma
    S Asano
    Department of Neurosurgery, The University of Tokyo Hospital, Tokyo, Japan
    Acta Neurochir (Wien) 145:599-603; discussion 603. 2003
    Following recent progress in radiological and surgical techniques for treating skull base tumours, more attention should be paid to the relatively rare phenomenon of intradural spinal seeding in cases of clival chordoma.
  90. ncbi Hypofractionated stereotactic radiation therapy for skull base and upper cervical chordoma and chondrosarcoma: preliminary results
    Ho Shin Gwak
    Department of Neurosurgery, Korea Institute of Radiological and Medical Science, Seoul, Korea
    Stereotact Funct Neurosurg 83:233-43. 2005
    b>Chordoma and chondrosarcoma are rare tumors of the base of the skull and are difficult candidates for surgical treatment. They are also usually resistant to conventional radiation therapy...
  91. pmc Evidence for PDGFRA, PDGFRB and KIT deregulation in an NSCLC patient
    T Negri
    Br J Cancer 96:180-1. 2007
  92. ncbi Endonasal endoscopic approach for the resection of chondroid chordoma with skull base involvement
    Mustafa Sagit
    Department of Otorhinolaryngology, Kayseri Training and Research Hospital, Kayseri, Turkey
    J Craniofac Surg 22:1720-1. 2011
    b>Chordoma is an uncommon tumor of bone that is believed to develop from remnants of the notochord. The chondroid variant of chordomas contains elements possessing both chordoma and cartilaginous tissues...
  93. ncbi A comprehensive analysis of intracranial chordoma and survival: a systematic review
    Brian J Jian
    Department of Neurological Surgery, University of California at San Francisco, San Francisco, California, USA
    Br J Neurosurg 25:446-53. 2011
    Despite the published information on cranial chordoma, most of the data regarding survival in these patients has come from a single institution...
  94. ncbi The next step: innovative molecular targeted therapies for treatment of intracranial chordoma patients
    Jeffrey J Barry
    Department of Neurological Surgery, University of California at San Francisco, San Francisco, California 94117, USA
    Neurosurgery 68:231-40; discussion 240-1. 2011
    ..Surgery and radiotherapy are the treatment mainstays of chordoma, but the chance of local recurrence remains high...
  95. ncbi Derivation and characterization of an extra-axial chordoma cell line (EACH-1) from a scapular tumor
    Amalia M DeComas
    Ochsner Medical Center, New Orleans, LA 70121, USA
    J Bone Joint Surg Am 92:1231-40. 2010
    ..Few studies have been done on this neoplasm because of its rarity. In addition, there is a lack of a good in vitro model on which to perform more characterization...
  96. ncbi Methylthioadenosine phosphorylase and activated insulin-like growth factor-1 receptor/insulin receptor: potential therapeutic targets in chordoma
    Josh Sommer
    Chordoma Foundation, Greensboro, NC, USA, and Division of Medical Oncology, Department of Medicine, Duke University Medical Center and Durham Veterans Affairs Medical Center, Durham, NC, USA
    J Pathol 220:608-17. 2010
    Currently there is no effective chemotherapy for chordoma. Recent studies report co-expression of insulin-like growth factor-1 receptor (IGF1R) and its cognate ligand in chordoma, but it is unknown whether this receptor tyrosine kinase is ..
  97. ncbi Clival chordoma manifesting as nasal bleeding. A case report
    Ryuhei Kitai
    Department of Neurosurgery, Faculty of Medical Sciences, University of Fukui, 23 Shimoaizuki Matsuoka, Fukui, 9111193, Japan
    Neuroradiology 47:368-71. 2005
    b>Chordoma is a rare cartilaginous tumor, for which bleeding presentation is unusual. We report a case of rare hemorrhaged clival chordoma, which was diagnosed correctly by magnetic resonance imaging...
  98. ncbi Infections in surgery of primary tumors of the sacrum
    Pietro Ruggieri
    Department of Orthopedics, University of Bologna, Istituto Ortopedico Rizzoli, Bologna, Italy
    Spine (Phila Pa 1976) 37:420-8. 2012
    ..Retrospective case series...
  99. pmc Brachyury, SOX-9, and podoplanin, new markers in the skull base chordoma vs chondrosarcoma differential: a tissue microarray-based comparative analysis
    Gerard J Oakley
    Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA 15261, USA
    Mod Pathol 21:1461-9. 2008
    The distinction between chondrosarcoma and chordoma of the skull base/head and neck is prognostically important; however, both have sufficient morphologic overlap to make delineation difficult...
  100. ncbi Carbon ion radiotherapy for unresectable sacral chordomas
    Reiko Imai
    Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba
    Clin Cancer Res 10:5741-6. 2004
    ..The purpose is to evaluate the efficacy and toxicity of carbon ion radiotherapy for unresectable sacral chordomas...
  101. ncbi Treatment planning intercomparison for spinal chordomas using intensity-modulated photon radiation therapy (IMRT) and carbon ions
    D Schulz-Ertner
    Department of Radiation Oncology, University of Heidelberg, Heidelberg, Germany
    Phys Med Biol 48:2617-31. 2003
    ..A combination of carbon ions and photon IMRT might be preferable in order to profit by the physical advantages of carbon ions while ensuring a safe treatment...

Research Grants11

  1. Genetic Analysis of Chordoma
    Michael J Kelley; Fiscal Year: 2012
    ..In this proposal, we propose to complement our ongoing genetic analysis of familial chordoma, a rare cancer thought to arise from notochord, with approaches being applied to individualization of common ..
  2. mTOR signaling and sporadic chordomas
    Sangyeul Han; Fiscal Year: 2010
    ..Molecular mechanism of chordoma development remains unknown...
  3. Third International Chordoma Research Workshop: Catalyzing New Advances in Chordo
    EDWARD LES; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): This grant application requests partial support for the Third International Chordoma Research Workshop (ICRW) which will be coordinated and co-sponsored by the Chordoma Foundation, a 501(c)(3) ..
  4. Brachyury-downstream gene networks in the notochord
    Anna Di Gregorio; Fiscal Year: 2013
    ..studies have indicated a direct association between the duplication of the human Brachyury locus and familial chordoma, a malignant notochord-derived tumor...
  5. Rare Cancer Genetics Registry
    Dianne M Finkelstein; Fiscal Year: 2013
    ..Diagnoses of chordoma, uveal melanoma, Merkel cell carcinoma, vulvar cancer, and adenoid cystic carcinoma will be the focus of ..
  6. Fourth International Chordoma Research Workshop (ICRW)
    Patricia Cogswell; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): This grant application requests partial support for the Fourth International Chordoma Research Workshop (ICRW) being coordinated and co-sponsored by the Chordoma Foundation, a 501(c)(3) nonprofit ..
  7. Phase I/II Trial of ZD1839 and Celecoxib in Ex-Smokers
    Michael Kelley; Fiscal Year: 2006
    ..abstract_text> ..
  8. Measurement of Hypoxia in Non Small Cell Lung Carcinoma
    Michael Kelley; Fiscal Year: 2003
    ..We believe the data from this pilot study will be useful to design future study(ies) with clinical endpoints and to guide selection of subjects for novel hypoxia-directed therapies in patients with NSCLC...
  9. GENETIC ANALYSIS OF HEREDITARY MACROTHROMBOCYTOPENIAS
    Michael Kelley; Fiscal Year: 2002
    ..When the causative gene is identified, biochemical and physiological analysis will be pursued including development of a murine model of the disease. ..
  10. Hypopituitarism after Moderate and Severe Head Injury
    Daniel Kelly; Fiscal Year: 2008
    ..By diagnosing and treating both acute and chronic traumatic neuroendocrine deficiencies, this study may dramatically improvethe long-term prognosis of many TBI patients. ..
  11. Cognitive Studies of Temporal Lobe Function in Man
    Guila Glosser; Fiscal Year: 2003
    ..b) To assess the usefulness of presurgical fMRI activation measures during an allocentric spatial memory task for predicting memory performance following ATL. ..