• Rare Sarcoma
  • Typically seen in immunosuppressed (classically HIV)
  • Classification
    • Classic (sporadic): indolent disease in elderly (especially Mediterranean) men
    • Endemic: more aggressive disease seen in young African people
    • Transplant-related: rare; seen in pts on cyclosporine
    • Epidemic: aggressive disease seen in HIV patients
      • For some reason in those that acquired AIDS via sexual or transfusion transmission
  • Aetiology
  • Clinical
    • Papular (raised) brown / red nodules
    • Can be associated systemic symptoms (type B) – weight loss, fever, on-going infection
  • Pathology
    • Spindle-cell tumour thought to be of endothelial cell lineage
      • Express markers of both endothelial (vascular & lymphatic) & smooth muscle lineages
    • Usually found on skin, but may be in mouth / GI tract / respiratory tract
  • Investigation
    • Punch Bx
  • Management
    • Treating the immunosuppression can slow or stop the progression of KS
    • Surgery: For small superficial lesions If larger→ associated with high recurrence rate
    • Cryosurgery
    • Laser
    • Radiotherapy
    • If widespread: can consider systemic Rx with Interferon-α or Paclitaxel