Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease

Abstract

BackgroundEndemic Kaposi sarcoma (KS) was first described in African children over fifty years ago, but has recently been overshadowed by HIV-related disease. We aimed to evaluate the similarities and differences between endemic HIV-negative and epidemic HIV-positive pediatric KS in a KS-associated herpesvirus-endemic region of Africa.MethodsWe describe clinical characteristics of 20 HIV-negative children with endemic KS over a six-year period and compare findings with a historical control—an HIV-related pediatric KS cohort from Lilongwe, Malawi.ResultsThe HIV-negative endemic KS cohort was 70% male with a median age of 9.3 years. Lymph node involvement was present in 50%, hyperpigmented skin lesions in 45%, and woody edema in 40%. One patient (5%) presented with oral KS involvement and no patients presented initially with visceral KS. Significant anemia (hemoglobin < 8 g/dL) and thrombocytopenia (platelet count < 100 × 109/L) were found at time of original KS diagnosis in 45 and 40% respectively. In both HIV-negative and HIV-positive cohorts, lymphadenopathy was the most common presentation, prototypical skin lesions were often absent, severe cytopenias were a common clinical feature, and treatment outcomes were similar. Patients with endemic KS demonstrated less frequent oral involvement (5% versus 29%, P = 0.03) and a lower proportion of patients with visceral involvement (0% versus 16%, P = 0.06).ConclusionsThese data suggest clinical overlap between epidemiological variants. Treatment protocols for pediatric KS in sub-Saharan Africa should be devised to include both endemic HIV-negative and epidemic HIV-related disease to better define the clinical and biological comparison.Electronic supplementary materialThe online version of this article (10.1186/s13027-018-0207-4) contains supplementary material, which is available to authorized users.

Systemic Treatment Initiation in Classical and Endemic Kaposi’s Sarcoma: Risk Factors and Global Multi-State Modelling in a Monocentric Cohort Study

“…Endemic KS develops in younger patients and tends to be difficult to treat and have poor clinical outcome with high lymphedema rates [ 22 , 23 ]. To our knowledge, this is the first report of a large series of non-HIV related and non-iatrogenic KS, highlighting the endemic subtype clinical aggressiveness.…”

Use of Paclitaxel to Successfully Treat Children, Adolescents, and Young Adults with Kaposi Sarcoma in Southwestern Tanzania

“…With paclitaxel treatment, she achieved complete clinical remission. Her case highlights the important therapeutic role paclitaxel can play in the management of pediatric patients with endemic KS and the importance of ensuring patients with endemic KS can access paclitaxel treatment [ 29 ].…”

Epidemic, Endemic, or Stewart–Bluefarb? When Several Forms of Kaposi Seem to Dispute Paternity

“…Endemic KD affects adults as well as children. Its transmission is more probably horizontal (child-child) or vertical (mother-child and parents-children) [1,3,[6][7][8]. It is diagnosed most often between 30 and 40 years with a marked predominance with males.…”

“…Since the advent of the HIV-AIDS (human immunodeficiency virus-acquired immunodeficiency syndrome) pandemic, it has experienced an epidemiological outbreak. e risk of developing KD is higher in areas where there is a high prevalence of HHV8 infection and HIV infection: Central, West, and South Africa [4][5][6][7][8].…”