Updated: 6/22/2021

Angiosarcoma

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https://upload.orthobullets.com/topic/8070/images/Case B - leg - clinical photo - parsons_moved.jpg
https://upload.orthobullets.com/topic/8070/images/Case B - leg - clinical photo 2 - parsons_moved.jpg
https://upload.orthobullets.com/topic/8070/images/Case D - dist tibia - CT - Parsons_moved.png
https://upload.orthobullets.com/topic/8070/images/Case A - pelvis invasion - parsons_moved.png
https://upload.orthobullets.com/topic/8070/images/Case B - leg - MRI - parsons_moved.jpg
https://upload.orthobullets.com/topic/8070/images/Histology A - parsons_moved.png
  • summary
    • Angiosarcomas are malignant, aggressive tumors that derive from the endothelium of blood vessels.
    • The condition is typically seen in elderly patients who present with regional pain and overlying skin changes.
    • Diagnosis is made with a biopsy showing malignant cells associated with vascular structures.
    • Treatment is usually wide surgical resection with radiation.
  • Epidemiology
    • Demographics
      • male > female
      • elderly
    • Anatomic location
      • osseous involvement in <10%
        • 60% long bone with osseous involvement
    • Risk factors
      • chronic vascular stasis
      • trauma
  • Symptoms
    • Symptoms
      • pain
      • symptoms often insidious
    • Physical exam
      • hallmarked with
        • overlying skin changes
  • Imaging
    • Radiograph
      • may see the 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
  • Prognosis
    • Poor prognosis
      • high local failure rate and amputation is often required
      • propensity for lymphatic spread
      • metastases to lung is common

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(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?

QID: 71
FIGURES:

Epithelioid sarcoma

19%

(463/2432)

Chondrosarcoma

4%

(102/2432)

Angiosarcoma

70%

(1697/2432)

Osteosarcoma

1%

(21/2432)

Synovial sarcoma

6%

(135/2432)

L 2 D

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