Vascular Tissue- Soft tissue tumours

Question 1

What is an Haemangioma?

Answer 1

• Benign vascular neoplasm in soft tissue
• may be cutaneous, subcutaneous, intramuscular
• capillary ( superificial )or cavernous ( deep)
• M=F
• <1% All benign vascular tumours
• common pts <30 yrs
• location
  
  • intramuscular is deep in lower extremities
  • often isolated lesions except diffuse haemangioma (involves a single limb in childhood)
  • commonly in the hand

Question 2

What is the pathophysiology of haemangiomas?

Answer 2

• Caused by error in the new formation of a segment of the vascular system
• 20% assoc with trauma

Question 3

What is the prognosis of haemangiomas?

Answer 3

• Infantile haemangiomas involute by age 7
• no incidence of malignant transformation

Question 4

How do haemangiomas present?

Answer 4

• Symptoms
  
  • Pain variable
  • if large-
    
    • aching
    • heaviness
    • swelling

O/E

• mass of variable size depending on activity level- see pic

Question 5

What is seen on imaging of haemangiomas?

Answer 5

• Xrays
  
  • small phleboliths inside the lesion- see pic
• MRI w gadolinium
  
  • increased signal on T1 & T2

Question 6

What is the tx of haemangiomas?

Answer 6

non operative

• Observation, NSAIDS, VAscular stockings & activity modification
  
  • first line tx
  • childhood lesions
• Sclerotherapy or emobilisation
  
  • by inteventional radiologist
  • large , painful lesions fail nsaids/stocking

Operative

• Marginal excision
  
  • small lesions in hand
• Wide surgical resection
  
  • for those lesions resistant to non op mx
  • high incidence of local recurrence
    *

Question 7

What is the complication of haemangiomas?

Answer 7

• Kasabach-Meritt Syndrome
  
  • rare complx caused by entrapped platelets -> possibly fatal coagulopathy

Question 8

What is an angiosarcoma?

Answer 8

• Rare Malignant & very aggressive tumour that derived from endothelium of blood vessels
• M>F
• elderly pts
• locations
  
  • osseous invovlement <10% ( 60% long bones)
• Risk factors
  
  • trauma
  • chronic vascular stasis

Question 9

What is the prognosis of Angiosarcoma?

Answer 9

• Poor
• High failure rate & amputation required
• Propensity for lymphatic spread
• Mets to lung common

Question 10

What are the symptoms of Angiosarcoma?

Answer 10

• Pain
• symptoms often insidious
• hallmark with overlying skin changes

Question 11

What is seen on imaging of Angiosarcoma?

Answer 11

• xray - invasion into bone
• MRI-to evaulate soft tissue mass
• cd31 in histology is sensitive marker for Angiosarcoma

Question 12

What is the tx of Angiosarcoma?

Answer 12

Operation

• Wide surgical resection
  
  • tx of choice
  • Insensitive to chemo/radiation
• Amputation
  
  • for agressive tumour
  • to gain local control