chapter 20

Chordoma

CLINICAL SUMMARY
Chordoma is a slow-growing malignancy with a strong predisposition for the sacrum and skull base.
Because of its location, sacrococcygeal chordoma is often discovered after it has attained a large size.
Resection of large tumors can be associated with a disabling loss of sacral nerve root function, while local
recurrence is common due to the difficulty of obtaining wide margins. Metastasis is usually a late event.

DIAGNOSTIC FEATURES
History Unusual younger than age 40
Vague lower spinal pain; constipation is a late symptom
Symptoms often present for more than 1 year
Up to 30% of patients may have radiculopathy

Location Almost exclusively found at the two ends of the spine

Anterior sacrococcygeal (50% to 60%), with S3/S4 most common; spheno-occipital less
common (25% to 40%)

Margins Sacrococcygeal chordoma is often missed on radiographs

CT demonstrates aggressive bone destruction, often large soft tissue mass

Matrix Stippled calcification seen in majority of cases

IMAGIN G

AP radiograph of a
sacrococcygeal chordoma
showing calcification and
partial destruction of the lower
sacrum (left).
Lateral radiograph shows
subtle destructive process of
the distal sacrum (right).

124

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 Chordoma 125

A small chordoma in the distal

sacrum is seen on CT (left) and
sagittal T2-weighted MRI
(right). Note the extraosseous
mass (arrows).

Chordomas often present late,

as the mass can be quite large
before symptoms arise.

CT scan shows an anterior soft

tissue mass with areas of
mineralization causing extrinsic
compression of the rectum
(left).
In this patient, CT shows low-
attenuation anterior and
posterior soft tissue masses
with calcifications (right).

Sagittal T1 MRI (left)

demonstrates areas of high
signal corresponding to
hemorrhage (arrow). This helps
to differentiate chordomas from
primary chondrosarcomas,
which are rare in the sacrum.
Sagittal T2 MRI shows high
signal, reflecting the high water
content of these lesions (right).

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126 VISUAL GUIDE TO MUSCULOSKELETAL TUMORS: A CLINICAL - RADIOLOGIC - HISTOLOGIC APPROACH

Contrast-enhanced T1 sagittal
(left) and axial (right) MRI
images show areas of
nonenhancing hemorrhage
(arrows) amidst diffuse mild
enhancement.

HISTOLOGY

Sagittal gross specimen (left)

and macrosection (right) reveal
anterior and posterior soft
tissue components, a lobular
growth pattern, and areas of
hemorrhage. A small remnant
of normal sacrum is seen
superiorly.

Grossly, the growth pattern in

chordoma is lobular, as in
chondrosarcoma, but has a
more “fleshy” appearance
(left).
Histologically (right), the
tissues demonstrate myxoid
and cartilaginous regions with
epithelioid nests (arrow) and
delicate vascularity.

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 Chordoma 127

Note epithelial cells (star) and

typical physalliferous cells
(arrow), distinguished by their
bubbly cytoplasm (left).
Cytokeratin staining is strongly
positive, indicating epithelial
expression (right). In contrast,
chondrosarcoma does not
stain with cytokeratin,
although both tumors stain
positively with S100.

TREATMENT
Primary treatment is surgical excision.
Achieving negative margins can be very difficult in large lesions; therefore, local recurrence is com-
mon (30% to 60%). Loss of sacral integrity and nerve roots can be very disabling.
Adjuvant radiation can reduce the risk of local recurrence, but very high doses are required. There is
no useful role for chemotherapy in primary treatment.
Survival at 10 years is only 20% to 30% due to late metastasis.

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