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For further information, see CMDT Part 6-42: Kaposi Sarcoma

Key Features

Essentials of Diagnosis

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

General Considerations

  • Classic Kaposi sarcoma

    • Occurs in older men

    • Has a chronic clinical course

    • Rarely fatal

  • Endemic Kaposi sarcoma occurs in an often aggressive form in young black men of equatorial Africa

  • Iatrogenic Kaposi sarcoma

    • Occurs in patients receiving immunosuppressive therapy

    • Improves upon decreasing immunosuppression

  • HIV-related Kaposi sarcoma

    • Prevalence has been reduced due to antiretroviral therapy

    • However, continues to occur in both well controlled HIV infection or AIDS

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

  • 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 the elderly

    • Palliative local therapy with intralesional chemotherapy 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 because in most cases this treatment alone is associated with improvement (see HIV Infection)

  • Other therapeutic options include cryotherapy or intralesional vinblastine (0.1–0.5 mg/mL) for cosmetically objectionable lesions

  • Systemic chemotherapy

    • Indicated for rapidly progressive skin disease (more than 10 new lesions per month), with edema or pain, and with symptomatic visceral disease or pulmonary disease

    • Liposomal doxorubicin

      • Highly effective in severe cases

      • May be used alone or in combination with bleomycin and vincristine

    • Other therapeutic options include

      • Paclitaxel

      • Etoposide

      • Bortezemib

      • Lenalidomide

    • Targeted immunotherapy with PD-1/PDL-1 blockade (nivolumab, pembrolizumab) has been successful in small series

Surgery

  • Laser surgery for certain intraoral and pharyngeal lesions

Therapeutic Procedures

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