Introduction A multifocal systemic tumor of endothelial cell origin. linked with human herpesvirus 8 infection Has Four clinical variants Responds to radiation and chemotherapy,
Classic KS Occurs in elderly males of eastern European heritage. Predominantly arises on the legs but also occurs in lymph nodes and abdominal viscera. slow progression and has good prognosis
African-Endemic KS bimodal age distribution young adults, mean age 35; and young children , mean age 3 years Males > females Has poor prognosis (young adults 5–8 years; young children 2–3 years) Four clinical patterns
African-Endemic KS patterns Nodular type : benign course and resembles classic KS . Florid or vegetating type : more aggressive. Nodular extend deeply into the subcutis , muscle, and bone . Infiltrative type : more aggressive has mucocutaneous and visceral . Lymphadenopathic type : children and young adults. confined to lymph nodes and viscera, also involves the skin and mucous membrane .
Iatrogenic Immunosuppression -Associated KS Rare . Most commonly in solid-organ transplant recipients chronic use of immunosuppressive drugs Resolves on cessation of immunosuppression .
HIV/AIDS-Associated KS AIDS increases risk of KS ( 20,000 times that those not infected with HIV) Common in homosexual men Rapid progression , extensive systemic involvement KS of the bowel and/or lungs is responsible for numerous deaths
Pathogenesis Initially polyclonal proliferation is due angiogenic factors. Later becomes monoclonal . KS lesions produce growth factors. It is not know how HHV8 promotes the process.
Clinical manifestation Mucocutaneous lesions are usually asymptomatic lower extremities - edema and moderate to severe pain Urethral or anal canal lesions- obstruction Pulmonary KS - bronchospasm , intractable coughing, shortness of breath, progressive respiratory failure.
Skin lesions The lesions are normally palpable even at the patch stage.
Management Radiotherapy Cryosurgery Photodynamic Therapy Excisional Surgery Intralesional Cytotoxic Chemotherapy Chemotherapy- single and combined chemotherapy