A Comprehensive Review of Intracranial Chordomas
Eren Erdem, M.D., Edgardo J.C. Angtuaco, M.D., Muhammad Husain, M.D., Ossama Al-Mefty, M.D.

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Introduction
Cranial chordomas are relatively rare, aggressive, and locally destructive tumors of the skull base. 35% of all chordomas occur along the vicinity of the clivus (sphenooccipital bones). They represent 1% of intracranial and 4% of all primary bone tumors. Chordomas may occur at any age but are usually seen in adults with a peak incidence in the 4th decade of life with a male predominance of 2:1. Although generally slow growing, the tumors’ intimate relation to critical structures and extremely high local recurrence rate has led to most of the patients dying from the primary intracranial disease. Recent advances in skull base surgery and radiation therapy provides an opportunity for cure. This can be achieved with precise knowledge of tumor extent and its relationship to vital neural and vascular structures provided by advanced imaging capabilities of computed tomography (CT) and magnetic resonance imaging (MRI).

Histopathology
Chordomas are neoplasms of vestigial notochord tissue. Remnants of the notochord may remain in the midline, usually entrapped within bone along the ends of the spinal column. Clivus and coccyx serve as the main site of origin of chordomas. The tumor cells tend to be arranged in cords set in a pale matrix of mucopolysaccharide with characteristic physaliferous appearance.

• Imaging
  • Characteristic Imaging Features
  • Location
  • CT
  • MRI
    • T1-Weighted
    • T2-Weighted
    • Enhanced Studies
  • Magnetic Resonance Angiography
  • Angiography
• Tumor Spread
  • Anterior
  • Lateral
  • Posterior
  • Inferior
  • Superior
• Treatment
• Recurrence
  • Local
  • Surgical Pathway
  • Distant Metatasis
• References

Conclusion
Intracranial chordomas are rare, midline tumors of clival origin. MRI and CT imaging are the modality of choice for diagnosis, treatment planning, and follow up. Bone destruction and the presence of intratumoral calcification is visualized on CT, and MR imaging delineates soft tissue extension and the relation of the tumor to vital structures and intracranial vessels. Radical surgical resection followed by proton beam radiation therapy achieves the best results. Local recurrence is not uncommon with an unfavorable prognosis.

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