Kaposi's Sarcoma

Aka: Kaposi's Sarcoma, Kaposi Sarcoma, Multiple Idiopathic Hemorrhagic Sarcoma

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II. Epidemiology

  1. Highest frequency in HIV homosexual or bisexual men
  2. Middle aged to elderly white males
  3. Young Black patients in Africa
  4. Very uncommon in women and children
  5. Incidence
    1. Was more common (48%) early in the HIV epidemic
    2. Now only seen 18% of HIV cases in homosexual or bisexual men

III. Pathophysiology

  1. Endothelial tumor
  2. Usually multicentric
  3. Involves the skin and visceral organs
  4. Very rarely involves the brain
  5. May be caused by an enteric sexually transmitted agent
    1. Associated with Human Herpes Virus 8

IV. Risk Factors

  1. HIV Infection
    1. AIDS definining illness (less common with highly active Retroviral therapy)
  2. More common in those with oral or anal sexual contacts

V. Symptoms

  1. Lesions typically non-pruritic
  2. Initial: Painless
  3. Later: Lesions may become painful as they enlarge

VI. Signs: Cutaneous lesions

  1. Pigmented (violaceous)
    1. Red-blue/purple or bluish-brown Plaques, Papules and Nodules
    2. Flush with skin or raised strawberry-like Plaques
    3. Lesions enlarge, coalesce and may bleed
  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

VII. Signs: Intraoral disease

  1. Intraoral disease present at time of diagnosis: 50%
  2. Higher risk of gastrointestinal involvement

VIII. Signs: Gastrointestinal involvement (Usually asymptomatic)

  1. Dysphagia
  2. Gastric outlet obstruction
  3. Gallbladder

IX. 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

X. Labs: Biopsy

  1. Histology Findings
    1. Dermis with increased spindle cells and vascularization
    2. Significant plasma cell infiltration
  2. Histology differentiates from
    1. Bacillary Angiomatosis
    2. Cutaneous Cryptococcus

XI. Differential Diagnosis

  1. Dermatofibroma
  2. Bacillary Angiomatosis
  3. Hemosiderotic Hemangioma
  4. Fibrous histiocytoma
  5. Interstitial Granuloma Annulare
  6. Arteriovenous Malformation
  7. Pyogenic Granuloma

XII. Management: General

  1. Evaluate for underlying predisposing condition (esp. HIV Infection)
  2. No treatment is curative!
  3. Slow the progression of systemic disease
  4. Manage local or regional problems

XIII. 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

XIV. Resources

  1. Kaposi Sarcoma (Orphanet)
    1. https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=33276
  2. Kaposi Sarcoma (Stat Pearls)
    1. https://www.ncbi.nlm.nih.gov/books/NBK534839/

XV. References

  1. Helm (2023) Am Fam Physician 107(2): 191-2 [PubMed]

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