Introduction: Kaposi's sarcoma (KS) is an angioproliferative disease that occurs in four clinical-epidemiological forms sharing the same immunological and histopathological features, suggesting common etiological and pathogenic factors. Infection with the human herpesvirus 8, cytokine- and angiogenic factor-induced growth together with an immuno-dysregulated state represent fundamental conditions for the development of this tumor. Despite the recent improvements in KS management, it remains an incurable disease.
Areas covered: The growing knowledge of KS biology provides multiple opportunities for the development of rational, molecularly targeted therapies. The present review summarizes the current management of KS, including local and systemic conventional therapies, and thoroughly describes the results obtained with new pathogenesis-based anti-KS treatments.
Expert opinion: Kaposi's sarcoma represents a paradigm of how the elucidation of disease pathogenesis can drive the development of molecularly targeted treatments. The multifactorial pathogenesis of KS has led to the evaluation of many experimental agents targeting one or more specific factors or pathways involved in the development or progression of the disease. Although targeted therapy so far represents investigational treatment, clinical evaluation of several of these agents is yielding promising results.