title: Clinical presentation and outcome of epidemic Kaposi sarcoma in Ugandan children.
abstract: 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.
authors.affiliation: Seattle Children's Hospital, Seattle, WA, USA. sgantt@uw.edu
authors.forename: Soren
authors.initials: S
authors.lastname: Gantt
authors.forename: Abel
authors.initials: A
authors.lastname: Kakuru
authors.forename: Anna
authors.initials: A
authors.lastname: Wald
authors.forename: Victoria
authors.initials: V
authors.lastname: Walusansa
authors.forename: Lawrence
authors.initials: L
authors.lastname: Corey
authors.forename: Corey
authors.initials: C
authors.lastname: Casper
authors.forename: Jackson
authors.initials: J
authors.lastname: Orem
mesh_terms: D000293:Adolescent; D044966:Anti-Retroviral Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D018791:CD4 Lymphocyte Count; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D015658:HIV Infections / Q000188:drug therapy / Q000453:epidemiology*; D019288:Herpesvirus 8, Human; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D012514:Sarcoma, Kaposi / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology; D016896:Treatment Outcome; D014454:Uganda / Q000453:epidemiology
chemical_list: D044966:Anti-Retroviral Agents