Updated: 11/12/2019

Chordoma

0%
Topic
Review Topic
0
0
N/A
N/A
Questions
6
0
0
0%
0%
Evidence
6
0
0
0%
0%
Videos / Pods
3
Topic
https://upload.orthobullets.com/topic/8032/images/Case A - pelvis - xray - parsons_moved.jpg
https://upload.orthobullets.com/topic/8032/images/Histology A - parsons_moved.jpg
https://upload.orthobullets.com/topic/8032/images/Case A - pelvis - CT - parsons_moved.jpg
https://upload.orthobullets.com/topic/8032/images/axialmri.jpg
https://upload.orthobullets.com/topic/8032/images/gross.jpg


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

Please rate topic.

Average 4.1 of 15 Ratings

Questions (6)

(OBQ18.86) A 58-year-old male is referred to your clinic for a sacral mass found on a lumbar spine MRI. He brought a pathology report from a biopsy that was performed at an outside hospital that states “primary malignant bone tumor of notochordal remnant cells.” Staging work-up showed no distant disease. Which of the following describes the best treatment strategy for this patient? Tested Concept

QID: 212982
1

Neo-adjuvant chemotherapy, surgical excision, maintenance chemotherapy

21%

(292/1380)

2

Wide surgical excision

64%

(880/1380)

3

Tumor debulking, chemotherapy

6%

(83/1380)

4

Radiation therapy

7%

(96/1380)

5

Observation with serial imaging

1%

(20/1380)

L 3 A

Select Answer to see Preferred Response

(SBQ18SP.48) A 47-year-old man presents with 6 months of progressive back pain, especially while sitting in his recliner at home. He's also noted some recent bowel incontinence. Diagnostic tests are performed and shown in Figures A and B. All of the following are true with respect to this diagnosis EXCEPT? Tested Concept

QID: 211630
FIGURES:
1

The primitive pathological cells are similar to those that derive the nucleus pulposis

9%

(151/1646)

2

Metastatic disease is common at the time of presentation

33%

(549/1646)

3

MRI studies are in most cases sufficient for diagnosis without the need for formal biopsy

32%

(530/1646)

4

The primary location of these lesions generally does not lead to lower extremity motor deficits

10%

(160/1646)

5

Treatment consists of surgical resection with or without radiation

14%

(230/1646)

L 5 A

Select Answer to see Preferred Response

Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(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? Tested Concept

QID: 442
FIGURES:
1

Regional lymph node metastasis

8%

(264/3206)

2

Liver metastasis

2%

(66/3206)

3

Bone metastasis

14%

(442/3206)

4

Local recurrence

69%

(2219/3206)

5

Malignant transformation

6%

(201/3206)

L 2 D

Select Answer to see Preferred Response

Evidence (13)
VIDEOS & PODCASTS (4)
EXPERT COMMENTS (9)
Private Note