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Updated: Jun 21 2021

Chordoma

Images
https://upload.orthobullets.com/topic/8032/images/Case A - pelvis - xray - parsons_moved.jpg
https://upload.orthobullets.com/topic/8032/images/Case A - pelvis - CT - parsons_moved.jpg
https://upload.orthobullets.com/topic/8032/images/Histology A - parsons_moved.jpg
https://upload.orthobullets.com/topic/8032/images/axialmri.jpg
https://upload.orthobullets.com/topic/8032/images/gross.jpg
  • summary
    • Chordomas are malignant tumors of primitive notochordal origin that most commonly occur in the sacrum and coccyx. The tumor typically presents in patients > 50 years of age with insidious low back pain with bowel or bladder dysfunction.
    • Diagnosis is made with biopsy showing foamy, vacuolated, physaliferous cells that are keratin positive on histochemical stains. 
    • Treatment is usually wide margin surgical resection with or without radiation.
  • Epidemiology
    • Incidence
      • most common primary malignant spinal tumor in adults
    • Demographics
      • 3:1 male to female ratio
      • usually in patients > 50 years
    • Anatomic location
      • 50% occur in the sacrum and coccyx
      • 35% in spheno-occiptal region
      • 15% in mobile spine
  • Etiology
    • Pathoanatomy
      • forms from malignant transformation in residual notochordal cells
        • resulting in midline location
  • 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
  • Differential
      • Differential of Chordomas
      • Sacral lesions in older patients
      • Keratin stain positive
      • Similar Appearance on Xray
      • Treated with wide-resection alone
      • Chordoma
      • o
      • o
      • o
      • o
      • Chondrosarcoma
      • o
      • o
      • o
      • Metastatic disease
      • o
      • o
      • o
      • Lymphoma
      • o
      • o
      • Multiple Myeloma
      • o
      • MFH
      • o
      • Secondary sarcoma
      • Enchondroma of hand
      • Ollier's
      • Maffucci's
      • Periosteal chondroma
      • Osteochondroma (MHE)
      • Parosteal osteosarcoma
      • o
      • Adamantinoma
      • o
      • o
      • Synovial Sarcoma
      • o
      • Epitheloid sarcoma
      • o
      • Squamous cell 
      • o
  • 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 S3 nerve roots
        • preserve unilateral S2, S3, S4 roots when possible
  • 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
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