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malignant tumor of primitive notochordal origin
slow growing and frequently misdiagnosed as low back pain
most common primary malignant spinal tumor in adults
3:1 male to female ratio
usually in patients > 50 years
50% occur in the sacrum and coccyx
35% in spheno-occiptal region
15% in mobile spine
forms from malignant transformation in residual
metastatic disease in 30-50%
occurs late in the course of the disease so long term follow up required
may spread to lung and rarely to bone
60% 5-years survival
25% long term survival
local extension may be fatal
insidious onset of pain
may be mistaken for low back
or hip pain
often complain of
bowel or bladder changes
sensory deficits rare due to distal nature of tumor
motor deficits rare because most lesions at S1 or distal
bowel and bladder changes are common
more than 50% of sacral chordomas are
palpable on rectal exam
difficult to see
lesion due to overlying bowel gas
midline bone destruction
and soft tissue mass
calcifications often present within the soft tissue lesion
bright on T2
useful to evaluate soft tissue extension
transrectal biopsy is contraindicated
lobular and gelatinous
grows in distinct nodules
important to distinguish from chondrosarcoma, which is not keratin positive
weakly S100 positive
wide margin surgical resection +/- radiation
standard of care in most patients
must be willing to sacrifice sacral nerve roots to
obtain adequate surgical margins
if margin not achieved
long-term survival 25-50%
50% local recurrence common
some newer evidence that radiation with proton-photon beams may be beneficial for recurrence
Differentials & Groups
Sacral lesions in older patients (1)
Keratin stain positive
Similar Appearance on Xray
Treated with wide-resection alone (2)
Enchondroma of hand
Squamous cell (3)
ASSUMPTIONS: (1) Older patient is > 40 yrs; (2) assuming no impending fracture (3) assuming no metastatic disease
(1) - histology does not always correspond to clinical case
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Qbank (1 Questions)
A 55-year-old man complaining of increasing problems with constipation undergoes a biopsy of a mass after a digital rectal exams reveals a fullness. The radiograph and micrograph of the biopsy specimen are shown in Figures A and B, respectively. Immunohistochemistry is positive for keratin and S-100. Following wide surgical resection, oncologic surveillance is necessary due to the high rate of which of the following?
Regional lymph node metastasis
Select Answer to see Preferred Response
PREFERRED RESPONSE ▶
The clinical presentation is consistent with a sacral chordoma. These tumors have a very high local recurrence rate, and therefore close oncologic surveillance is necessary.
Chordomas are notoriously slow growing tumors that often cause insidious symptoms of low back pain, constipation, and problems with sitting due to mass effect. Treatment is wide surgical resection, which is difficult due to the sacral location. Local recurrence is dependent on the surgical margins.
Hulen et al showed in a restrospective review (Level IV) that 12/16 patients had recurrent disease, 15/16 had some bladder/bowel issue post operatively, 3/16 were able to walk without an assistive device.
Guo et al reviewed the clinical outcomes (Level IV) of 50 patients who underwent sacrectomy for various reasons and found an association between S-3 nerve root resection and bowel and bladder issues.
Figure A show an AP pelvis with large lytic area in sacrum. Figure B is a biopsy specimen shows physaliferous cells that appear soap-like. Illustration V is a video which shows the surgical resection of a chordoma from the sacrum.
Oncologic and functional outcome following sacrectomy for sacral chordoma.
Hulen CA, Temple HT, Fox WP, Sama AA, Green BA, Eismont FJ
J Bone Joint Surg Am. 2006 Jul;88(7):1532-9.
PMID: 16818979 (Link to Abstract)
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Bowel and bladder continence, wound healing, and functional outcomes in patients who underwent sacrectomy.
Guo Y, Palmer JL, Shen L, Kaur G, Willey J, Zhang T, Bruera E, Wolinsky JP, Gokaslan ZL.
J Neurosurg Spine. 2005 Aug;3(2):106-10.
PMID: 16370299 (Link to Abstract)
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Jan Szatkowski MD
John Badylak MD
Patrick McCulloch MD
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Chordoma - Histology Rounds
This video reviews the characteristic histology of a chordoma?
Sacral Chordoma Resection and Cryosurgery - Dr. James C. Wittig
Dr. James C. Wittig provides step-by-step procedure of Sacral Chordoma Cryosurge...
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