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For further information, see CMDT Part 6-13: Kaposi Sarcoma

KEY FEATURES

Essentials of Diagnosis

  • Human herpes virus 8 (HHV-8) or Kaposi sarcoma–associated herpes virus (KSHV), is universally present in all forms of Kaposi sarcoma

General Considerations

  • Occurs in five forms

    • Classic Kaposi sarcoma occurs in older men, has a chronic clinical course, and is rarely fatal

    • Endemic Kaposi sarcoma is an often aggressive form that occurs in young Black men of equatorial Africa

    • Iatrogenic Kaposi sarcoma occurs from immunosuppressive therapy

    • Epidemic Kaposi sarcoma is associated with HIV-associated immune deficiency

      • Incidence is diminishing

      • Can develop in patients with HIV infection, high CD4 counts, and low viral loads

      • Usually resembles the endemic form, being indolent and localized

      • Can be clinically aggressive

      • The presence of Kaposi sarcoma at the time of antiretroviral initiation is associated with Kaposi sarcoma–immune reconstitution inflammatory syndrome, which has an especially aggressive course in patients with visceral disease

    • Indolent Kaposi sarcoma occurs exclusively in men without HIV who have sex with men

Demographics

  • The most common HIV-related malignancy

CLINICAL FINDINGS

Symptoms and Signs

  • Red, purple, or dark plaques or nodules on cutaneous or mucosal surfaces

  • Marked edema may occur with few or no skin lesions

  • Commonly involves the gastrointestinal tract, but in asymptomatic patients these lesions are not sought or treated

  • Pulmonary Kaposi sarcoma

    • Can present with shortness of breath, cough, hemoptysis, or chest pain

    • May be asymptomatic, appearing only on chest radiograph

  • Chronic Kaposi sarcoma

    • Can develop in patients with HIV infection, high CD4 counts, and low viral loads

    • Usually resembles the endemic form, being indolent and localized

    • However, it can be clinically aggressive

Differential Diagnosis

  • Bacillary angiomatosis

  • Hemangioma

  • Vasculitis (palpable purpura)

  • Dermatofibroma

  • Pyogenic granuloma

  • Prurigo nodularis

  • Melanoma

DIAGNOSIS

Laboratory Tests

  • Diagnosis is based on appearance of skin lesions with confirmatory biopsy

Imaging Studies

  • Chest radiography

Diagnostic Procedures

  • Fecal occult blood testing in patients with gastrointestinal symptoms

  • Bronchoscopy in patients with pulmonary symptoms

TREATMENT

Medications

  • Kaposi sarcoma in older patients

    • Palliative local therapy with intralesional chemotherapy (vinblastine (0.1–0.5 mg/mL) or radiation is usually all that is required

  • In the setting of iatrogenic immunosuppression

    • The treatment is primarily reduction of doses of immunosuppressive medications

  • AIDS-associated Kaposi

    • The patient should first be given effective anti-HIV antiretrovirals

    • Intralesional vinblastine (0.1–0.5 mg/mL) for cosmetically objectionable lesions

    • Systemic therapy is indicated in patients with skin disease that is cosmetically unacceptable or in those with advanced cutaneous, oral visceral, or nodal disease

  • Antiretroviral therapy plus chemotherapy appears to be ...

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