Kaposi sarcoma
Kaposi sarcoma is a human herpesvirus 8-associated vascular malignancy that presents as violaceous macules, papules, plaques, or tumors of the skin, mouth, gastrointestinal tract, or lungs, most often in HIV-associated, transplant-related, or classic elderly disease. Diagnosis is confirmed by biopsy. Every patient with HIV-associated Kaposi sarcoma starts or optimizes antiretroviral therapy, with liposomal doxorubicin 20 mg/m2 intravenously every 21 days added for extensive or visceral disease.
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What this article covers
- Overview
Virally driven vascular malignancy: Kaposi sarcoma is a vascular proliferative disease attributed to human herpesvirus 8 (HHV-8), also called Kaposi sarcoma-associated herpesvirus.
- Epidemiology
Acquired immunodeficiency syndrome (AIDS) defining cancer: Kaposi sarcoma is one of three cancers, along with non-Hodgkin lymphoma and invasive cervical cancer, designated as AIDS-defining.
- Risk factors
Immunosuppression: the principal risk factor is immunosuppression, including AIDS and corticosteroid use.
- Pathophysiology
HHV-8-driven proliferation: HHV-8 has been identified in tissue from patients with both epidemic and classic Kaposi sarcoma, supporting a direct viral role in the malignancy.
- Clinical features
Lesion evolution: physical examination shows purplish, reddish, or dark brown to black macules and papules that progress to patches, plaques, and then tumors.
- Differential diagnosis
Bacillary angiomatosis: caused by Bartonella infection and typically occurring in advanced HIV disease, it shows a neutrophilic infiltrate on biopsy, unlike the lymphocytic infiltrate of Kaposi…
- Diagnosis
Biopsy confirms the diagnosis: Kaposi sarcoma is diagnosed by history, clinical impression, and histology; biopsy is especially important to distinguish Kaposi sarcoma from bacillary angiomatosis…
- Management
First, for every patient with HIV-associated disease, start or optimize antiretroviral therapy: this is indicated for all HIV-associated Kaposi sarcoma and has been shown to decrease both the…
- Complications
Visceral spread: plaques can extend to the gastrointestinal tract and lung, which can cause bleeding or respiratory compromise depending on the site.
- Prognosis
Antiretroviral therapy improves outcomes: antiretroviral therapy decreases both the incidence and severity of HIV-associated Kaposi sarcoma, making adherence a central determinant of prognosis.
- Prevention
Antiretroviral adherence prevents progression: maintaining antiretroviral therapy adherence is the central preventive measure, since gaps in treatment are linked to Kaposi sarcoma progression and…
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