Kaposi's sarcoma (KS) is a multicentric vascular tumor caused by herpesvirus type 8. It can occur in classic, AIDS-associated, endemic, and iatrogenic forms. Diagnosis is by biopsy. Treatment for indolent superficial lesions involves cryotherapy, electrocoagulation, excision, or electron beam radiation therapy. Radiation therapy is used for more extensive disease. In the AIDS-associated form, antiretrovirals provide the most improvement.

KS originates from endothelial cells in response to infection by human herpesvirus 8 (HHV-8). Immunosuppression (particularly by AIDS and drugs for organ transplant recipients) markedly increases the likelihood of KS in HHV-8–infected patients. The tumor cells have a spindle shape, resembling smooth muscle cells, fibroblasts, and myofibroblasts.

Kaposi's Sarcoma (Classic)

Classification

Classic KS: This form occurs most often in older (> 60 yr) men of Italian, Jewish, or Eastern European ancestry. The course is indolent, and the disease is usually confined to a small number of lesions on the skin of the lower extremities; visceral involvement occurs in < 10%. This form is usually not fatal.

Kaposi's Sarcoma (AIDS-Associated)

AIDS-associated (epidemic) KS: This form is the most common AIDS-associated cancer and is more aggressive than classic KS. Multiple cutaneous lesions are typically present, often involving the face and trunk. Mucosal, lymph node, and GI involvement is common. Sometimes KS is the first manifestation of AIDS.

Endemic KS: This form occurs in Africa independent of HIV infection. There are 2 main types:

• Prepubertal lymphadenopathic form: It predominantly affects children; primary tumors involve lymph nodes, with or without skin lesions. The course is usually fulminant and fatal.
• Adult form: This form resembles classic KS.

Iatrogenic (immunosuppressive) KS: This form typically develops several years after organ transplantation. The course is more or less fulminant, depending on the degree of immunosuppression.

Symptoms and Signs

Cutaneous lesions are asymptomatic purple, pink, or red macules that may coalesce into blue-violet to black plaques and nodules. Some edema may be present. Occasionally, nodules fungate or penetrate soft tissue and invade bone. Mucosal lesions appear as bluish to violaceous macules, plaques, and tumors. GI lesions can bleed, sometimes extensively, but usually are asymptomatic.

Diagnosis

• Biopsy

Diagnosis is confirmed by punch biopsy. Patients with AIDS or immunosuppression require evaluation for visceral spread by CT of the chest and abdomen. If CT is negative but pulmonary or GI symptoms are present, bronchoscopy or GI endoscopy should be considered.

Treatment

• Surgical excision, cryotherapy, or electrocoagulation for superficial lesions
• Local radiation therapy for multiple lesions or lymph node disease
• Antiretroviral therapy or sometimes IV interferon alfa for AIDS-associated KS
• Reduction of immunosuppressants for iatrogenic KS

Indolent lesions often require no treatment. One or a few superficial lesions can be removed by excision, cryotherapy, or electrocoagulation. Intralesional vinblastineSome Trade Names
VELBAN
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or interferon alfa is also useful. Multiple lesions and lymph node disease are treated locally with 10 to 20 Gy of radiation therapy.

AIDS-associated KS responds markedly to highly active antiretroviral therapy (HAART), probably because CD4+ count improves and HIV viral load decreases; however, there is some evidence that protease inhibitors in this regimen may block angiogenesis. AIDS patients with indolent disease and CD4+ counts > 150 /μL and HIV RNA < 500 copies/mL can be treated with IV interferon alfa. Patients with more extensive or visceral disease can be given liposomal doxorubicinSome Trade Names
ADRIAMYCIN
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20 mg/m² IV q 2 to 3 wk. If this regimen fails, patients may receive paclitaxelSome Trade Names
TAXOL
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. Other agents being investigated as adjuncts include IL-12, desferrioxamine, and oral retinoids. Treatment of KS does not prolong life in most AIDS patients because infections dominate the clinical course.

Iatrogenic KS responds best to stopping immunosuppressants. In organ transplant patients, reduction of immunosuppressant dosage often results in reduction of KS lesions. If dosage reduction is not possible, conventional local and systemic therapies used in other forms of KS should be instituted. SirolimusSome Trade Names
RAPAMUNE
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may also improve iatrogenic KS.

Treatment of endemic KS is challenging and typically palliative.

Last full review/revision October 2008 by Gregory L. Wells, MD

Content last modified February 2012