# Classic Kaposi Sarcoma: Current Treatment Strategies and Emerging Therapeutic Approaches

## TL;DR

Classic Kaposi sarcoma is a rare cancer linked to a virus, and this review discusses current and new treatment options for managing it, especially in elderly patients.

## Contribution

The paper provides a comprehensive review of current and emerging therapies for classic Kaposi sarcoma, emphasizing the need for personalized treatment due to limited clinical trial data.

## Key findings

- Classic Kaposi sarcoma is rare and often managed with limited evidence from clinical trials.
- Treatment strategies include local and systemic therapies tailored to patient comorbidities and disease stage.
- Emerging therapies like immunotherapy and antiangiogenic agents show promise for future management.

## Abstract

Classic Kaposi sarcoma (CKS) is a rare vascular neoplasm associated with human herpesvirus-8 (HHV-8). It characteristically follows an indolent clinical course, typically manifesting in elderly populations of Mediterranean and Eastern European descent. While CKS often presents as localized cutaneous lesions, its management becomes increasingly complex when patients develop symptomatic systemic complications. Because of its rarity and the specific population it affects, robust prospective clinical trial data remain limited; consequently, current knowledge regarding standardized therapeutic strategies is often limited to retrospective analyses and expert consensus. Furthermore, the challenge of treating elderly patients with multiple comorbidities necessitates a personalized approach to minimize toxicity. However, the evolving understanding of tumor pathogenesis has paved the way for innovative interventions. This review provides a comprehensive, up-to-date overview of current and emerging therapeutic strategies for CKS. We discuss established treatment modalities and shift focus toward emerging approaches, including the promising roles of immunotherapy and antiangiogenic agents.

Classic Kaposi sarcoma (CKS) is a rare vascular neoplasm driven by infection with Kaposi sarcoma-associated herpesvirus (KSHV) and characterized by a heterogeneous geographic distribution. While the etiology of other Kaposi’s sarcoma (KS) variants is well established, the underlying mechanisms of CKS appear multifactorial, with several risk factors, among which advanced age and male sex are most significant. Due to the rarity of CKS and its often indolent clinical course, clinical trials are sparse, and current knowledge of therapeutic approaches remains limited. Most available clinical trials and practice guidelines focus on human immunodeficiency virus (HIV) -related Kaposi sarcoma. Therefore, specific recommendations for the classic form are restricted and rely on a low level of evidence, as categorized by the National Comprehensive Cancer Network (NCCN) Clinical Practice Guidelines, making disease management more challenging. Current approaches to treating CKS include both local and systemic therapies, selected based on disease stage, patient comorbidities, and individual preferences. Systemic treatment options may include immunotherapy, chemotherapy, or antiangiogenic agents. This review summarizes current management strategies and highlights emerging therapeutic approaches for CKS. It aims to support clinicians in optimizing therapeutic decision-making, including the use of novel and investigational therapies. Although several novel therapies are currently under investigation in clinical trials, significant gaps remain. Therefore, further research is needed to improve disease management.