Clinical presentation and outcome of epidemic Kaposi sarcoma in Ugandan children
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Published on: August 25, 2017
Soren Gantt1, Abel Kakuru, Anna Wald
1Seattle Children's Hospital, Seattle, WA, USA. sgantt@uw.edu
Insights
Epidemic Kaposi sarcoma (KS) in children in Uganda often presents with lymph node involvement, occurring at younger ages and higher CD4 counts. Most children responded well to treatment, highlighting the need for further research into optimal management strategies.
Area of Science:
- Pediatric Oncology
- Infectious Diseases
- Epidemiology
Background:
- Kaposi sarcoma (KS) is a prevalent pediatric cancer in sub-Saharan Africa.
- Limited data exist on the clinical presentation and treatment response of epidemic (HIV-associated) KS in children.
Purpose of the Study:
- To describe the clinical characteristics of epidemic KS in children in Uganda.
- To evaluate treatment response in this pediatric population.
Main Methods:
- Retrospective review of medical records of children with KS and HIV infection at Uganda Cancer Institute (2004-2007).
- Data abstracted included demographics, KS lesion location, CD4 T-cell counts, and treatment outcomes.
Main Results:
- 73 children with epidemic KS were identified; 60% presented with lymph node (LN) involvement.
- Children with lymphadenopathic KS were younger and had higher CD4 T-cell counts compared to those without LN involvement.
- A complete response to chemotherapy and/or antiretroviral therapy was observed in 62.5% of patients with available outcome data.
Conclusions:
- Lymph node involvement in epidemic KS in children occurs at younger ages and higher CD4 levels compared to cutaneous involvement.
- This presentation may suggest recent human herpesvirus 8 infection with rapid progression.
- Favorable treatment responses were noted, but prospective studies are needed for optimal management.
Background:
Kaposi sarcoma (KS) is one of the most common pediatric cancers in sub-Saharan Africa. Few data are available about the clinical presentation or response to treatment of children with epidemic (HIV-associated) KS.
Methods:
Medical records of all children with KS and HIV infection referred to the Uganda Cancer Institute in Kampala, Uganda from October 2004 to June 2007 were reviewed. Charts were abstracted for age, sex, location of KS lesions at presentation, biopsy results, CD4 T-cell count and percentage, and KS treatment and outcome.
Results:
Seventy-three children with epidemic KS were identified, 37 males and 36 females. The median age was 10.1 years (range 2-18). KS presented with lymph node (LN) involvement in 60% of cases. The median absolute and percentage CD4 T-cells at presentation were 210 cells/microl and 7.4%, respectively. Those children with lymphadenopathic KS were younger (mean difference 3.7 years; P = 0.01) and had higher CD4 T-cell counts (mean difference 242 cells/microl; P = 0.03) than those without LN involvement. Of 32 patients for whom outcome data were available, a complete response to chemotherapy and/or antiretroviral therapy was documented in 20 (62.5%) patients.
Conclusions:
In comparison to cutaneous involvement, LN involvement of epidemic KS occurs at younger ages and at higher CD4 levels. This clinical presentation may reflect recent infection with human herpesvirus 8 followed by a rapid progression to malignancy. Favorable response to treatment was observed in the majority of cases, but prospective studies are needed to determine optimal management.
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