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Case of the Month

Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO

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June 2019
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Intracranial Chordoma

  • Background/Demographics​
    • ​Chordomas are rare tumors derived from primitive notochordal remnants, usually trapped in the midline skull base and spinal column.
    • As such, chordomas arise in the sacrococcygeal spine (50%)>spheno-occipital region (35%)>mobile spine (15%).
    • When arising in the skull base, they tend to be midline; in distinction to chondrosarcomas, which arise off midline (unusual off-midline appearance in this case).
  • Imaging
    • ​CT: Hypodense mass, +/- amorphous calcifications (calcifications more common in chondrosarcoma as chondroid matrix), lytic osseous destruction.
    • MRI: Always markedly T2 hyperintense, almost always reported as enhancing either mildly or avidly (unusual to not enhance as in this case).
    • MRS: Few to no specific case reports. In this case, the presence of a choline peak is a clue to a malignant neoplasm, as opposed to an epidermoid cyst.
  • Differential Diagnosis
    • ​Chondrosarcoma (extremely rare in children).
    • Epidermoid cyst (could appear similar but this case is very large, choline peak argues against).
    • Ecchordosis physaliphora (also rare, no reports could be identified of a case this size).
  • Treatment/Prognosis
    • ​Surgery (complete resection if possible) and adjuvant radiotherapy.
    • Local recurrence is more likely than distant metastasis.
    • Progression free survival at 5 years 50-65%.

Suggested Reading

  1. Erdem E, Angtuaco EC, Van Hemert R, et al. Comprehensive review of intracranial chordoma. Radiographics 2003;23:995-1009, 
    10.1148/rg.234025176.
  2. Cho YH, Kim JH, Khang SK, et al. Chordomas and chondrosarcomas of the skull base: comparative analysis of clinical results in 30 patients. Neurosurgical Review 2008;31:35-43, 10.1007/s10143-007-0099-z. 

Current Issue

American Journal of Neuroradiology: 42 (2)
American Journal of Neuroradiology
Vol. 42, Issue 2
1 Feb 2021
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