Kaposi's
sarcoma is a multicentric
vascular tumor caused by herpesvirus type 8. It can occur in classic, AIDS-associated, endemic, and iatrogenic forms. The tumor cells have a spindle shape, resembling
smooth muscle cells, fibroblasts, and myofibroblasts.
Classification
Classic Kaposi sarcoma: This occurs most often in older (> 60 yr). 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.
AIDS-associated (epidemic) Kaposi sarcoma: This is the most common AIDS-associated malignancy. Multiple cutaneous lesions are typically present, often involving the face and trunk. Mucosal, lymph node, and GI involvement is common.
Endemic Kaposi sarcoma: 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.
Iatrogenic (immunosuppressive): This typically develops several years after organ transplantation.
Symptoms and Signs
- Cutaneous lesions are asymptomatic purple, pink, or red macules that may coalesce into blue-violet to black plaques and nodules.
- Occasionally, nodules fungate or penetrate soft tissue and invade bone
- Mucosal lesions appear as bluish to violaceous macules, plaques, and tumors
Diagnosis
- Diagnosis is confirmed by punch biopsy
- Patients with AIDS or Immunosuppression require evaluation for visceral spread by CT of the chest and abdomen
Treatment
- Surgical excision, cryotherapy, or electro coagulation for superficial lesions
- Local radiation therapy for multiple lesions or lymph node disease
- Antiretroviral therapy or sometimes IV interferon-α for AIDS-associated Kaposi sarcoma
- Reduction of immunosuppressant for iatrogenic Kaposi sarcoma.