Abstract

Kaposi sarcoma (KS) is a malignancy associated with Kaposi's sarcoma-associated herpesvirus (KSHV), primarily affecting immunocompromised individuals, such as those with HIV or those receiving immunosuppressive treatments. Immunocompetent individuals may also be affected, illustrating the disease's heterogeneity. KS manifests in different forms-classic, endemic, epidemic, iatrogenic, and in men having sex with men-each with distinct clinical features depending on immune status and geographic area of origin. Although advances in treatment have improved disease control, effective management remains a challenge. This review focuses on the comprehensive approach to investigating and treating KS. It highlights the role of histology, immunohistochemistry, and staging in diagnosing KS and assessing disease extension, together with other KSHV diseases (multicentric Castelman disease, primary effusion lymphoma, and KS inflammatory cytokine syndrome). Treatment strategies are discussed, with emphasis on restoring immunity in immunocompromised patients, alongside conventional local therapies, and chemotherapy options like liposomal doxorubicin and paclitaxel for aggressive and extensive forms. Promising emerging therapies, including immunomodulatory agents, antiangiogenic therapies, and checkpoint inhibitors, are also explored. The review emphasizes the importance of personalized treatment based on the patient's underlying condition and KS subtype. It provides an in-depth look at the pathogenesis, diagnostic methods, and evolving therapeutic approaches, offering valuable insights into improving management and outcomes for KS patients.