Chordoma¶

Definition¶

Chordoma is a rare, slow-growing malignant tumor arising from remnants of the embryonic notochord. It occurs exclusively along the axial skeleton, most commonly in the sacrococcygeal region (50–60%) and the clivus/skull base (25–35%), with the mobile spine accounting for a small minority of cases. Despite its slow growth, chordoma is locally aggressive with a high recurrence rate.

Imaging Findings¶

MRI¶

T1-weighted — Low to intermediate signal, lobulated mass
T2-weighted — Very high signal (characteristic) due to high water content and myxoid matrix. Internal septations or honeycomb pattern may be present.
Enhancement — Heterogeneous, typically moderate enhancement
Location — Midline, centered on the vertebral body. In the sacrum, large presacral soft tissue component is typical.
Bone destruction — Extensive lytic destruction of the vertebral body or sacrum

CT¶

Lytic, destructive midline mass centered on the vertebral body or sacrum
Amorphous calcifications or bone fragments within the mass (present in 30–70%)
Large soft tissue component, often extending anteriorly (presacral mass)
Well-defined lobulated margins

Clinical Pearl

The combination of a midline, sacral lytic mass with very high T2 signal and a large presacral soft tissue component is highly suggestive of chordoma. The key differential is giant cell tumor (which also occurs in the sacrum but tends to be more eccentric) and metastasis. Chordomas characteristically cross disc spaces, distinguishing them from infections which also cross disc spaces but have different clinical contexts.

Management¶

En bloc surgical resection — The treatment of choice; wide margins are critical to reduce recurrence
Radiation therapy — Proton beam or carbon ion therapy for unresectable or residual disease (conventional radiation has limited efficacy)
High recurrence rate even after complete resection

Key Points¶

Chordoma arises from notochord remnants and is exclusively midline/axial
Sacrococcygeal region is the most common location in the spine
Very high T2 signal is characteristic
Midline destructive mass with presacral soft tissue extension is the classic presentation
En bloc resection with wide margins is the treatment of choice
High recurrence rate despite treatment

References¶

Tenny S, Varacallo MA. Chordoma. In: StatPearls. Treasure Island (FL): StatPearls Publishing; updated 2023 Aug 7. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430846/
Chordoma. Radiopaedia. Available from: https://radiopaedia.org/articles/chordoma
Lee SH, Kwok KY, Wong SM, Chan CXJ, Wong YT, Tsang ML. Chordoma at the skull base, spine, and sacrum: a pictorial essay. J Clin Imaging Sci. 2022;12:44. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC9479632/
Farsad K, Kattapuram SV, Sacknoff R, Ono J, Nielsen GP. Sacral chordoma. RadioGraphics. 2009;29(5):1525-30. Available from: https://pubs.rsna.org/doi/full/10.1148/rg.295085215
Erdem E, Angtuaco EC, Van Hemert R, Park JS, Al-Mefty O. Comprehensive review of intracranial chordoma. RadioGraphics. 2003;23(4):995-1009. Available from: https://pubs.rsna.org/doi/full/10.1148/rg.234025176
Colangeli S, Muratori F, Bettini L, Frenos F, Totti F, D'Arienzo A, et al. Surgical treatment of sacral chordoma: en bloc resection with negative margins is a determinant of the long-term outcome. Surg Technol Int. 2018;33:343-8. Available from: https://pubmed.ncbi.nlm.nih.gov/30204925/
Denaro L, Berton A, Ciuffreda M, Loppini M, Candela V, Brandi ML, et al. Surgical management of chordoma: a systematic review. J Spinal Cord Med. 2020;43(6):797-812. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC7808319/