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

• It is a common infection in central Africa, is more common in Italy than in the United States, and is common in HIV-infected men who have sex with men and is rare in HIV-infected hemophiliacs

General Considerations

• Occurs endemically in an often aggressive form in young Black men of equatorial Africa, but it is rare in American Blacks

• The epidemiology of infection with HHV-8 or KSHV parallels the incidence of Kaposi sarcoma in various risk groups and geographic regions

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 the elderly

  • 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 because in most cases this treatment is associated with improvement (see HIV & AIDS)

• Other therapeutic options include cryotherapy or intralesional vinblastine for cosmetically objectionable lesions

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

• Antiretroviral therapy plus chemotherapy appears to be more effective than antiretroviral therapy alone

  • First-line systemic therapies include liposomal doxorubicin and paclitaxel

  • Other therapeutic options include

    • Pomalidomide

    • Etoposide

    • Gemcitabine

    • Imatinib

    • Interferon alpha-2b

    • Thalidomide

    • Vinorelbine

    • Bleomycin ...