Notochordal and Vascular tumours Flashcards Preview

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Flashcards in Notochordal and Vascular tumours Deck (15):
1

What is a chordoma?

  • 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

2

What are the symtpoms of chordoma?

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

3

What is seen on radiographs of chordoma?

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

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4

What is seen on histology of chordoma?

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

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5

What is the tx of chordoma?

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

6

What is the long term survical of chordoma?

  • 25-50%

7

What are the complications of chordoma?

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

8

What is a haemangioma?

  • 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

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9

What are the radiofrpahic features of haemangioma?

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

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10

What is seen on histology of  haemangioma?

  • Cavenous lesions iwth numerous thin walled blodd vessels

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11

What is the tx of haemangiomas?

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

12

What is haemangioendothelioma/ haemangiosarcoma?

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

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13

What is seen in xrays of haemangioendothelioma/ haemangiosarcoma?

  • Lytic lesions with no reactive bone

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14

What is the tx of haemangioendothelioma/ haemangiosarcoma?

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

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