Chordoma - Pathology - Orthobullets


Introduction
A malignant tumor of primitive notochordal origin slow growing and frequently misdiagnosed as low back pain Epidemiology incidence most common primary malignant spinal tumor in adults demographics 3:1 male to female ratio usually in patients > 50 years location 50% occur in the sacrum and coccyx 35% in spheno-occiptal region 15% in mobile spine Pathoanatomy forms from malignant transformation in residual notochordal cells resulting in midline location Prognosis metastasis 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 survival 60% 5-years survival 25% long term survival local extension may be fatal

Symptoms

Presentation
- pain
  - insidious onset of pain
  - may be mistaken for low back or hip pain
- neurologic
  - often complain of bowel or bladder changes
  - sensory deficits rare due to distal nature of tumor
- gastrointestinal
  - constipation
  - fecal incontinence
Physical exam
- neurologic
  - motor deficits rare because most lesions at S1 or distal
  - bowel and bladder changes are common
- rectal exam
  - more than 50% of sacral chordomas are palpable on rectal exam

Imaging

Radiographs
- often difficult to see lesion due to overlying bowel gas
CT
- will show midline bone destruction and soft tissue mass
- calcifications often present within the soft tissue lesion
MRI
- bright on T2
- useful to evaluate soft tissue extension

Histology

Biopsy
- transrectal biopsy is contraindicated
Gross
- lobular and gelatinous
Histology
- findings
  - characterized by foamy, vacuolated, physaliferous cell
  - grows in distinct nodules
- histochemical staining
  - keratin positive
    - important to distinguish from chondrosarcoma, which is not keratin positive
  - weakly S100 positive

Treatment

Nonoperative
- radiation treatment
  - indications
    - inoperable tumors
Operative
- wide margin surgical resection +/- radiation
  - indications
    - standard of care in most patients
  - technique
    - must be willing to sacrifice sacral nerve roots to obtain adequate surgical margins
    - add radiation if margin not achieved
  - outcomes
    - long-term survival 25-50%
    - en bloc corpectomy has best chance of local control with spinal lesions

Complications

Local recurrence
- 50% local recurrence common
- some newer evidence that radiation with proton-photon beams may be beneficial for recurrence
Loss of bowel/bladder function postoperatively
- to preserve near normal bowel/bladder function
  - preserve bilateral S2 nerve roots (at least)
  - preserve unilateral S2, S3, S4 roots

Differentials & Groups

	Sacral lesions in older patients (1)	Keratin stain positive	Similar Appearance on Xray	Treated with wide-resection alone (2)
Chordoma	•	•	•	•
Chondrosarcoma	•		•	•
Metastatic disease	•	•	•
Lymphoma	•		•
Multiple Myeloma	•
MFH	•
Secondary sarcoma
Enchondroma of hand
Olliers
Maffucci's
Periosteal chondroma
Osteochondroma (MHE)
Parosteal osteosarcoma				•
Adamantinoma		•		•
Synovial sarcoma		•
Epitheloid sarcoma		•
Squamous cell (3)				•
ASSUMPTIONS: (1) Older patient is > 40 yrs; (2) assuming no impending fracture (3) assuming no metastatic disease

	Location	Xray	Xray	CT	B. Scan	MRI	MRI	Histo(1)
Case A	sacrum
Case B	sacrum
Case C	sacrum
Case D	cervical spine

(1) - histology does not always correspond to clinical case

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(OBQ08.56) 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? Review Topic | Tested Concept

QID: 442

FIGURES:

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Regional lymph node metastasis

(233/2838)

Liver metastasis

(53/2838)

Bone metastasis

13%

(378/2838)

Local recurrence

70%

(1990/2838)

Malignant transformation

(172/2838)

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