Chondroid chordoma: subtype of conventional chordoma with large areas of matrix that mimics hyaline cartilaginous tumors
Dedifferentiated chordoma: biphasic appearance with conventional chordoma and high-grade sarcoma
Poorly differentiated chordoma: poorly differentiated neoplasm with notochordal differentiation
IMAGING
General Features
Imaging Recommendations
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Akinduro OO et al: Targeted therapy for chordoma: key molecular signaling pathways and the role of multimodal therapy. Target Oncol. 16(3):325-37, 2021
Tirabosco R et al: Benign notochordal cell tumour. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 449-50, 2020
Tirabosco R et al: Conventional chordoma. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 451-3, 2020
Zuckerman SL et al: Predictive value of preoperative magnetic resonance imaging findings for survival and local recurrence in patients undergoing en bloc resection of sacral chordomas. Neurosurgery. 85(6):834-42, 2019
Stacchiotti S et al: Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group. Ann Oncol. 28(6):1230-42, 2017
Lebellec L et al: Molecular targeted therapies in advanced or metastatic chordoma patients: facts and hypotheses. Crit Rev Oncol Hematol. 95(1):125-31, 2015
Meng T et al: Clinical features and prognostic factors of patients with chordoma in the spine: a retrospective analysis of 153 patients in a single center. Neuro Oncol. 17(5):725-32, 2015
Stacchiotti S et al: Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol. 16(2):e71-83, 2015
Varga PP et al: Surgical treatment of sacral chordoma: prognostic variables for local recurrence and overall survival. Eur Spine J. 24(5):1092-101, 2015
Kayani B et al: A review of the surgical management of sacral chordoma. Eur J Surg Oncol. 40(11):1412-20, 2014
Kayani B et al: Prognostic factors in the operative management of dedifferentiated sacral chordomas. Neurosurgery. 75(3):269-75; discussion 275, 2014
Orguc S et al: Primary tumors of the spine. Semin Musculoskelet Radiol. 18(3):280-99, 2014
Wang T et al: Role of apparent diffusion coefficients with diffusion-weighted magnetic resonance imaging in differentiating between benign and malignant bone tumors. World J Surg Oncol. 12:365, 2014
Bernard SA et al: Primary osseous tumors of the spine. Semin Musculoskelet Radiol. 17(2):203-20, 2013
Si MJ et al: Differentiation of primary chordoma, giant cell tumor and schwannoma of the sacrum by CT and MRI. Eur J Radiol. 82(12):2309-15, 2013
Chou WC et al: De novo dedifferentiated chordoma of the sacrum: a case report and review of the literature. Chang Gung Med J. 32(3):330-5, 2009
Farsad K et al: Sacral chordoma. Radiographics. 29(5):1525-30, 2009
Martin MP et al: Intradural drop metastasis of a clival chordoma. J Clin Neurosci. 16(8):1105-7, 2009
Presneau N et al: Potential therapeutic targets for chordoma: PI3K/AKT/TSC1/TSC2/mTOR pathway. Br J Cancer. 100(9):1406-14, 2009
Torres MA et al: Optimal treatment planning for skull base chordoma: photons, protons, or a combination of both? Int J Radiat Oncol Biol Phys. 74(4):1033-9, 2009
Gerber S et al: Imaging of sacral tumours. Skeletal Radiol. 37(4):277-89, 2008
Hanna SA et al: Dedifferentiated chordoma: a report of four cases arising 'de novo'. J Bone Joint Surg Br. 90(5):652-6, 2008
Hanna SA et al: Sacral chordoma: can local recurrence after sacrectomy be predicted? Clin Orthop Relat Res. 466(9):2217-23, 2008
Nguyen QN et al: Emerging role of proton beam radiation therapy for chordoma and chondrosarcoma of the skull base. Curr Oncol Rep. 10(4):338-43, 2008
Park SA et al: F-18 FDG PET/CT evaluation of sacrococcygeal chordoma. Clin Nucl Med. 33(12):906-8, 2008
Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
Casali PG et al: Chordoma. Curr Opin Oncol. 19(4):367-70, 2007
Soudack M et al: Imaging features of posterior mediastinal chordoma in a child. Pediatr Radiol. 37(5):492-7, 2007
Teichgräber V et al: Percutaneous MR-guided radiofrequency ablation of recurrent sacrococcygeal chordomas. AJR Am J Roentgenol. 187(2):571-4, 2006
Anis N et al: Use of radio-frequency ablation for the palliative treatment of sacral chordoma. AJNR Am J Neuroradiol. 25(9):1589-91, 2004
Neeman Z et al: Percutaneous radiofrequency ablation of chordoma. AJR Am J Roentgenol. 179(5):1330-2, 2002
Resnick D: Diagnosis of Bone and Joint Disorders. 4th ed. WB Saunders. 4014-23, 2002
Chondroid chordoma: subtype of conventional chordoma with large areas of matrix that mimics hyaline cartilaginous tumors
Dedifferentiated chordoma: biphasic appearance with conventional chordoma and high-grade sarcoma
Poorly differentiated chordoma: poorly differentiated neoplasm with notochordal differentiation
IMAGING
General Features
Imaging Recommendations
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Akinduro OO et al: Targeted therapy for chordoma: key molecular signaling pathways and the role of multimodal therapy. Target Oncol. 16(3):325-37, 2021
Tirabosco R et al: Benign notochordal cell tumour. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 449-50, 2020
Tirabosco R et al: Conventional chordoma. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 451-3, 2020
Zuckerman SL et al: Predictive value of preoperative magnetic resonance imaging findings for survival and local recurrence in patients undergoing en bloc resection of sacral chordomas. Neurosurgery. 85(6):834-42, 2019
Stacchiotti S et al: Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group. Ann Oncol. 28(6):1230-42, 2017
Lebellec L et al: Molecular targeted therapies in advanced or metastatic chordoma patients: facts and hypotheses. Crit Rev Oncol Hematol. 95(1):125-31, 2015
Meng T et al: Clinical features and prognostic factors of patients with chordoma in the spine: a retrospective analysis of 153 patients in a single center. Neuro Oncol. 17(5):725-32, 2015
Stacchiotti S et al: Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol. 16(2):e71-83, 2015
Varga PP et al: Surgical treatment of sacral chordoma: prognostic variables for local recurrence and overall survival. Eur Spine J. 24(5):1092-101, 2015
Kayani B et al: A review of the surgical management of sacral chordoma. Eur J Surg Oncol. 40(11):1412-20, 2014
Kayani B et al: Prognostic factors in the operative management of dedifferentiated sacral chordomas. Neurosurgery. 75(3):269-75; discussion 275, 2014
Orguc S et al: Primary tumors of the spine. Semin Musculoskelet Radiol. 18(3):280-99, 2014
Wang T et al: Role of apparent diffusion coefficients with diffusion-weighted magnetic resonance imaging in differentiating between benign and malignant bone tumors. World J Surg Oncol. 12:365, 2014
Bernard SA et al: Primary osseous tumors of the spine. Semin Musculoskelet Radiol. 17(2):203-20, 2013
Si MJ et al: Differentiation of primary chordoma, giant cell tumor and schwannoma of the sacrum by CT and MRI. Eur J Radiol. 82(12):2309-15, 2013
Chou WC et al: De novo dedifferentiated chordoma of the sacrum: a case report and review of the literature. Chang Gung Med J. 32(3):330-5, 2009
Farsad K et al: Sacral chordoma. Radiographics. 29(5):1525-30, 2009
Martin MP et al: Intradural drop metastasis of a clival chordoma. J Clin Neurosci. 16(8):1105-7, 2009
Presneau N et al: Potential therapeutic targets for chordoma: PI3K/AKT/TSC1/TSC2/mTOR pathway. Br J Cancer. 100(9):1406-14, 2009
Torres MA et al: Optimal treatment planning for skull base chordoma: photons, protons, or a combination of both? Int J Radiat Oncol Biol Phys. 74(4):1033-9, 2009
Gerber S et al: Imaging of sacral tumours. Skeletal Radiol. 37(4):277-89, 2008
Hanna SA et al: Dedifferentiated chordoma: a report of four cases arising 'de novo'. J Bone Joint Surg Br. 90(5):652-6, 2008
Hanna SA et al: Sacral chordoma: can local recurrence after sacrectomy be predicted? Clin Orthop Relat Res. 466(9):2217-23, 2008
Nguyen QN et al: Emerging role of proton beam radiation therapy for chordoma and chondrosarcoma of the skull base. Curr Oncol Rep. 10(4):338-43, 2008
Park SA et al: F-18 FDG PET/CT evaluation of sacrococcygeal chordoma. Clin Nucl Med. 33(12):906-8, 2008
Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
Casali PG et al: Chordoma. Curr Opin Oncol. 19(4):367-70, 2007
Soudack M et al: Imaging features of posterior mediastinal chordoma in a child. Pediatr Radiol. 37(5):492-7, 2007
Teichgräber V et al: Percutaneous MR-guided radiofrequency ablation of recurrent sacrococcygeal chordomas. AJR Am J Roentgenol. 187(2):571-4, 2006
Anis N et al: Use of radio-frequency ablation for the palliative treatment of sacral chordoma. AJNR Am J Neuroradiol. 25(9):1589-91, 2004
Neeman Z et al: Percutaneous radiofrequency ablation of chordoma. AJR Am J Roentgenol. 179(5):1330-2, 2002
Resnick D: Diagnosis of Bone and Joint Disorders. 4th ed. WB Saunders. 4014-23, 2002
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