Hematology and Oncology Book

Lymphatic Disorders

Sarcomas

  • Kaposi's Sarcoma

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Kaposi's SarcomaAka: Multiple Idiopathic Hemorrhagic Sarcoma

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  1. Epidemiology
    1. Highest frequency in HIV homosexual or bisexual men
    2. Middle aged to elderly white males
    3. Young Black patients in Africa
    4. Extremely uncommon in women and children
    5. Incidence
      1. Was more common (48%) early in the epidemic
      2. Now only seen 18% of cases (homosexual/bisexual men
  2. Pathophysiology
    1. Endothelial tumor
    2. Usually multicentric
    3. Involves the skin and visceral organs
    4. May be caused by an enteric sexually transmitted agent
    5. Very rarely involves the brain
  3. Risks
    1. More common in those with oral or anal sexual contacts
  4. Symptoms
    1. General: Lesions pruritic
    2. Initial: Painless
    3. Later: lesions can become large and painful
  5. Signs Cutaneous lesions
    1. Pigmented (violaceous)
      1. Red-blue or bluish-brown Plaques and Nodules
    2. Can involve any area of the skin
      1. Especially common on the soles of the feet
      2. Rarely affects the palms of the hands
    3. Biopsy differentiates from
      1. Bacillary Angiomatosis
      2. Cutaneous cryptococcus
  6. Signs Intraoral disease
    1. Intraoral disease present at time of diagnosis: 50%
    2. Higher risk of gastrointestinal involvement
  7. Signs gastrointestinal involvement (Usually asymptomatic)
    1. Dysphagia
    2. Gastric outlet obstruction
    3. GIB
  8. Signs Pulmonary involvement
    1. Rapidly fatal if not treated
    2. Usually symptomatic
      1. Non-productive cough
      2. Bronchospasm
      3. Dyspnea
    3. Pleural Effusion in 20-50% cases at diagnosis
      1. Pleural Fluid negative for Kaposi's Sarcoma
      2. Transudative or exudative
    4. Chest XRay
      1. Reticulonodular Pulmonary Infiltrates
      2. Much coarser than with Pneumocystis carinii
    5. Normal gallium scan
    6. Bronchoscopy
      1. Characteristic endobronchial lesions
      2. Biopsy usually avoided secondary to bleeding
  9. Management: General
    1. No treatment is curative!
    2. Slow the progression of systemic disease
    3. Manage local or regional problems
  10. Management: Medications
    1. Chemotherapy
      1. Cytotoxic drugs (Indicated for widespread disease)
        1. Doxorubicin
        2. Vinblastine
        3. Vincristine
        4. Bleomycin
      2. Interferon alpha
        1. Better with Less extensive cutaneous lesions
        2. Better if no edema or visceral disease
    2. Radiation Therapy
      1. Control Lymphedema
      2. Control painful or necrotic bulky tumors
    3. Surgical Resection
      1. Indicated for local complications
    4. Cryotherapy or Liquid Nitrogen
      1. Indicated to ablate single lesions

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