Kaposi sarcoma (KS) is among the three most common childhood malignancies in regions of Africa where human herpesvirus-8 (HHV-8)/KS-associated herpesvirus are endemic. 1-5 In contrast to adult disease, pediatric KS staging classifications, risk-stratification systems, and treatment paradigms remain poorly defined. 6-12

Prior studies suggest that the clinical features associated with survival in childhood KS are distinct from those in adults. 6-14 In our retrospective study of factors associated with event-free survival (EFS) and overall survival (OS) in pediatric KS, multivariable analysis demonstrated that visceral disease and disseminated skin/oral presentation (defined as≥ 20 hyperpigmented lesions in a widespread distribution) were independent risk factors for death and inability to achieve EFS with a minimally myelosuppressive regimen that contains bleomycin and vincristine (BV), which is commonly available even in low-income settings. 12 Patients with woody edema had low EFS but did not experience increased mortality. 12 Lymphadenopathic involvement in children was associated with the highest rates of long-term complete remission (CR). 12