Kaposi's sarcoma and its management in AIDS patients. Recommendations from a Scandinavian Study Group.

KS is the most frequent malignancy in homo/bisexual male AIDS patients, affecting more than 30% of these patients. KS may present itself as a few innocent cutaneous lesions or may show progression resulting in severe morbidity and mortality. Approximately half of the patients may develop severe progressive disease. The prognosis of patients with progressive disease is poor, with a median survival of less than 6 months. There is no cure for AIDS-related KS, but several therapies are available for palliation. The treatment options may be applied locally or systemically. Radiotherapy is efficacious and safe, but only a few lesions may be treated at one time. For severe progressive KS, systemic therapy with various forms of chemotherapy is used. Three regimes in particular have been focused on, namely bleomycin/vincristine (BV), doxorubicin + BV (DBV), or liposomal daunorubicin (LD) administered every 2 weeks. The agents result in a clinically relevant response (in 50-80% of patients) 2-4 weeks after initiation, but few patients have complete remission of the KS (< 10%), and the tumour may relapse after 4-6 months despite continued therapy. BV is less effective but also less toxic compared with the other regimens. Time to response for DBV may be slightly better than for LD, but the overall efficacy of these 2 regimes is similar. LD treatment is associated with significantly fewer episodes of peripheral neuropathy and alopecia than treatment with DBV. Thus, the recommended order of use of chemotherapeutic agents is BV, LD and DBV. Alpha-interferon may have a role in the small percentage of patients with CD4 cell count > 200 mill/L. In conclusion, several therapeutic options are available for palliation of KS. All systemically applied therapies are associated with severe side-effects and the optimal choice of treatment is a careful balance between response and toxicity. The recent discovery of human herpes virus 8 as a putative causative agent for KS and new potent groups of anti-retroviral agents, may lead to the development of more effective treatments of KS.