AIDS-related Kaposi's sarcoma (KS) is a tumour of vascular endothelium. whi
ch is seen predominately in men who have sex with men. The majority of affe
cted individuals have advanced immunosuppression at the time of the initial
KS diagnosis. The disease may present with cutaneous lesions, or with invo
lvement of visceral organs. of which the gastrointestinal tract is most com
mon. KS may also present with lymphoadenopathy or with isolated lymphoedema
, even in the absence of cutaneous lesions. Affected individuals are unifor
mly co-infected with HIV and with Human Herpesvirus type 8 (HHV8). HHVB is
present within KS tissues, and is aetiological in the pathogenesis of disea
se, along with aberrant cytokine expression, production of multiple angioge
nic peptides. and immune dysregulation. While not presently curable. multip
le treatment options exist and must be evaluated in terms of the specific n
eeds of the individual patient. Various local therapies are aimed at eradic
ating small lesions, while acknowledging that the KS in general, or its lik
elihood of recurring will be unaffected. Systemic chemotherapy is used to t
reat extensive visceral involvement, Knowledge of the pathogenesis of disea
se has led to the development of novel treatment strategies, aimed at HHV8
as the target of therapy. or at the inflammatory cytokine or angiogenic mil
ieu necessary for KS growth. Use of highly active anti-retroviral therapy,
aimed at controlling the underlying HIV infection, has been associated with
a dramatic decrease in the incidence of KS. and may also be useful in the
treatment of existing KS disease. (C) 2001 Elsevier Science Ltd. All rights
reserved.