Notochordal and Vascular tumours

Question 1

What is a chordoma?

Answer 1

• A malignant tumour of primative notochord origin
• slow growing and frequently misdiagnosed as low back pain
• **most common primary malignant spinal tumour in adults**
• males >females 3:1
• usually pts >50 yrs
• location
  
  • sacrum and coccyx 50%
• forms in malignant transformation in residual notochordal cells
  
  • results in midline location
• ​Metastatic disease in 30-50%
• occurs late in course of disease so long term fu required
• **25% long term survival, **60% 5 yr

Question 2

What are the symtpoms of chordoma?

Answer 2

• Pain
  
  • insidious onset
• Neurological
  
  • motor deficit rare as most lesions at S1 or distal
  • bowel & bladder changes common
  • PR- 50% papable
• GI
  
  • constipation/ incontinence

Question 3

What is seen on radiographs of chordoma?

Answer 3

• Difficult to see due to bowel gas
• MRI- bright on T2
• Ct will show midline bone destruction and soft tissue mass

Question 4

What is seen on histology of chordoma?

Answer 4

• Rectal Biopsy is CI
• Lobular and gelatinous- gross
• foamy, vaculoated physalifeous cell
• keratin postive
• weakly S100 positive

Question 5

What is the tx of chordoma?

Answer 5

Non operative

• Radiation tx
• for inoperative tumours

Operative

• wide margin surgical rescetion +/- radiation
  
  • most pts
  • must be willing to sacrifice sacral nerve roots to obtain adequate surgical resesction margins
  • add radiation if margins not achieved

Question 6

What is the long term survical of chordoma?

Answer 6

• 25-50%

Question 7

What are the complications of chordoma?

Answer 7

• Local reoccurance
  
  • 50% common
  • proton-photon beam maybe beneficial to reocurrance

Question 8

What is a haemangioma?

Answer 8

• A benign vascular tumour of bone that often occurs in the Vertebral bodies and cranio-facial bones
• maybe asymptomatic
• may present with Pain or pathological fx

Question 9

What are the radiofrpahic features of haemangioma?

Answer 9

• Lytic lesion cahracteristic vertical striations= honey comb/ jair bar appearance
• maybe multifocal
• bone scan warm- hot

Question 10

What is seen on histology of haemangioma?

Answer 10

• Cavenous lesions iwth numerous thin walled blodd vessels

Question 11

What is the tx of haemangiomas?

Answer 11

non operative

• observation is indicated if pt is asymptomatic

Operative

• Currettage and bone grafting
  
  • is lesion is symptomatic and accessible
• low dose radiation
  
  • is symptomatic but inaccessible

Question 12

What is haemangioendothelioma/ haemangiosarcoma?

Answer 12

• A rare malignant vascular tumour of bone
• found in all age groups
• 30% are multifocal
• symptoms= pain

Question 13

What is seen in xrays of haemangioendothelioma/ haemangiosarcoma?

Answer 13

• Lytic lesions with no reactive bone​

Question 14

What is the tx of haemangioendothelioma/ haemangiosarcoma?

Answer 14

• Non operative
  
  • radiation therapy alone
    
    • low grade lesions
• Operative
  
  • surgical resection +/- radiotherapy
    
    • high grade lesion