Angiosarcoma

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Topic updated on 05/20/13 12:14pm
Introduction
  • Rare malignant and very aggressive tumor that derives from endothelium of blood vessels
  • Epidemiology
    • demographics
      • male>female
      • elderly
    • location
      • osseous involvement in <10%
        • 60% long bone with osseous involvement
    • risk factors
      • chronic vascular stasis
      • trauma
  • Prognosis
    • poor
    • high local failure rate and amputation is often required
    • propensity for lymphatic spread
    • metastases to lung is common
Symptoms
  • Symptoms
    • pain
    • symptoms often insidious
  • Physical exam
    • hallmarked with
      • overlying skin changes
Imaging
  • Radiograph
    • may see invasion of bone 
  • MRI
    • study of choice to evaluate soft tissue mass 
Studies
  • Histology
    • vascular channels
    • variable degree of anaplasia
    • malignant cells associated with vascular structures  
Treatment
  • Operative
    • wide surgical resection  
      • indications
        • treatment of choice
        • relatively insensitive to chemotherapy and radiation
    • amputation
      • indications
        • to achieve local control of disease
        • very aggressive tumor
Ibank
  Location
Clinical
Xray
CT
B. Scan
MRI
MRI
Histo(1)
Case A leg
   
Case B pelvis  
   
(1) - histology does not always correspond to case 


 

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Qbank (1 Questions)

TAG
(OBQ05.34) A 34-year-old male with an eleven-year history of hand exposure to polyvinyl chloride presents with hand pain, but no constitutional symptoms. Physical exam shows lesions involving the nail-folds of the thumb, long, and ring finger as well as several soft-tissue masses in the palm. A radiograph is shown in Figure A and an axial T2 MRI is shown in Figure B. Angiography shows diffuse involvement of multiple lesions in the hand. A CT scan of the chest shows metastasis to the lungs. He undergoes an open biopsy and a representative histology image is shown in Figure C. The biopsy specimen stains positive for CD31 endothelial marker. What is the most likely diagnosis? Topic Review Topic
FIGURES: A   B   C      

1. Epithelioid sarcoma
2. Chondrosarcoma
3. Angiosarcoma
4. Osteosarcoma
5. Synovial sarcoma

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