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Chordoma

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Topic updated on 03/22/16 10:23pm
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%
Complications
  • Local recurrence
    • 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)
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? Topic Review Topic
FIGURES: A   B        

1. Regional lymph node metastasis
2. Liver metastasis
3. Bone metastasis
4. Local recurrence
5. Malignant transformation

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