chordoma

09
Pathology
• This tumour type develops from tissue known as the notochord
(pronounced no-tow-cord). The notochord is required during
embryonic development to form the template of spinal tissue while
the baby is in the womb. Over time, this spinal tissue is replaced with
bone and there is no use for the notochord.
• Small areas of the notochord can remain into adulthood with no
effect. However, like any other cell type, these small areas of
remaining notochord are capable of being transformed into
cancerous cells which grow uncontrollably to form a chordoma
tumour
 TEORY
• Chordoma is a rare form of primary bone cancer, known to affect only 1
individual per 800,000 people every year

• Chordomas most frequently arise in the spine or the skull, and the
different locations of these tumour lead to varying symptoms

• Chordomas can occur at any age but are usually seen in adults (30-70
years). Those located in the spheno-occipital region most commonly occur
in patients 20-40 years of age, whereas sacrococcygeal chordomas are
typically seen in a slightly older age group (peak around 50 years 10). They
are commonly found in Caucasians
• Chordomas are uncommon malignant tumors of the axial skeleton
that account for 1% of intracranial tumors and 4% of all primary bone
tumors.
• They originate from embryonic remnants of the
primitive notochord (earliest fetal axial skeleton, extending from
the Rathke's pouch to the tip of the coccyx). Since chordomas arise in
bone, they are usually extradural and result in local bone destruction.
They are locally aggressive but uncommonly metastasize.
LOCATION
• Chordomas are found along the axial skeleton and a relatively evenly
distributed among three locations:
• sacrococcygeal: 30-50% 2-3
• spheno-occipital: 30-35%
• vertebral body: 15-30%
Sacrococcygeal cordoma
• Sacrococcygeal is the most common location, accounting for
approximately 30-50% 2-3 of all chordomas and commonly involving
the fourth and fifth sacral segments 2. In this location, a male
predilection has been reported (M:F ratio of 2:1), and the tumor may
be particularly large at presentation 1
Radiographic features
CT
centrally located
well-circumscribed
destructive lytic lesion, sometimes with marginal sclerosis
expansile soft-tissue mass
usually hyper-attenuating relative to adjacent brain; however, inhomogenous areas
may be seen due to necrosis or hemorrhage
soft-tissue mass is often disproportionately large relative to the bony destruction
irregular intratumoral calcifications (thought to represent sequestra of
normal bone rather than dystrophic calcifications)
moderate to marked enhancement
MRI
T1
intermediate to low-signal intensity
small foci of hyperintensity (intratumoral hemorrhage or a mucus pool)
T2: most exhibit very high signal
T1 C+ (Gd)
heterogeneous enhancement with a honeycomb appearance corresponding to low T1 signal areas
within the tumor
greater enhancement has been associated with poorer prognosis 11
SWI/GE: variable intralesional hemorrhage, suggested by the presence of blooming
artefact
DWI/ADC
conventional chordoma: 1474 ± 117 x 10-6 mm2/s 13
dedifferentiated chordoma: 875 ± 100 x 10-6 mm2/s 13