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Navigating the heterogeneous landscape of pediatric Kaposi sarcoma

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Abstract

Vivid descriptions of Kaposi sarcoma (KS) occurring in children and adolescents from central and eastern Africa originated over 50 years ago. Unique clinical characteristics of pediatric KS in Africa were well described within these geographic regions that were eventually understood to be endemic for human herpesvirus-8/Kaposi sarcoma herpesvirus (HHV-8/KSHV) infection, the causative agent of KS. Having catapulted in incidence with the HIV epidemic, KS is currently among the top five most common childhood cancers in numerous countries throughout the region. The main feature that differentiates the childhood form of KS from adult disease is clinical presentation with primarily bulging lymphadenopathy. This group of patients represents the most common clinical subtype of pediatric KS in KSHV-endemic regions. Lymphadenopathic KS in children is associated with other distinct features, such as sparse occurrence of prototypical hyperpigmented cutaneous lesions, frequent presentation with severe cytopenias and a normal CD4 count, and a fulminant clinical course if untreated with chemotherapy. Increased awareness and improved recognition of lymphadenopathic KS are critically important, particularly because this subset of patients typically experiences a favorable response to chemotherapy characterized by durable complete remission. Clinical phenotypes typically observed in adult KS also occur in children—mild/moderate disease limited to cutaneous and oral involvement, woody edema, and visceral disease. This review summarizes the heterogeneous patterns of disease presentation and treatment response observed among the divergent clinical phenotypes of pediatric KS, highlights additional KSHV-related malignancies, and explores some of the potential biological drivers of such clinical phenomena.

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Acknowledgments

The authors express their utmost admiration and appreciation for our patients and their families. We would additionally like to thank our many colleagues from the Texas Children’s Cancer and Hematology Centers Global HOPE (Hematology-Oncology Pediatric Excellence) Program, Baylor Children’s Foundation Malawi in Lilongwe, the Tingathe Outreach Program, Baylor Children’s Foundation Tanzania in Mbeya, Kamuzu Central Hospital, the Baylor International Pediatric AIDS Initiative at Texas Children’s Hospital, the Pathology Laboratory at Kamuzu Central Hospital, Dr. Peter N. Kazembe, Dr. Carrie M. Cox, Dr. Parth S. Mehta, Dr. Dirk P. Dittmer and the University of North Carolina Vironomics Core Laboratory, Dr. Carrie L. Kovarik, Dr. Jason M. Bacha, and the many additional collaborating teams that have supported our work caring for children and adolescents with cancer in sub-Saharan Africa.

Funding

The National Cancer Institute at the National Institutes of Health (R21CA217137) and the Baylor-UT Houston Center for AIDS Research (through support from the National Institute of Allergy and Infectious Diseases, AI36211) have provided funding for research to the pediatric Kaposi sarcoma program at the Baylor College of Medicine Children’s Foundation Malawi in Lilongwe. The pediatric HIV-related malignancy clinical program in Lilongwe, Malawi, was also supported in part by a grant from the United States Agency for International Development through the Tingathe Program (674-A-00-10-00093-00), the Celgene Cancer Care Links grant program, and philanthropic contributions from ConocoPhillips, Abbvie, and the Bristol-Myers Squibb Foundation.

Author information

Author notes

  1. William Kamiyango and Jimmy Villiera contributed equally to this work.

Authors and Affiliations

  1. Baylor College of Medicine Children’s Foundation Malawi, Lilongwe, Malawi

    William Kamiyango, Jimmy Villiera & Allison Silverstein

  2. Texas Children’s Cancer and Hematology Centers Global HOPE Program, Lilongwe, Malawi

    William Kamiyango, Jimmy Villiera & Allison Silverstein

  3. Baylor College of Medicine, Houston, TX, USA

    Allison Silverstein, Erin Peckham-Gregory, Liane R. Campbell & Nader Kim El-Mallawany

  4. Global HOPE (Hematology-Oncology Pediatric Excellence), Texas Children’s Cancer and Hematology Centers, Baylor College of Medicine, 1102 Bates Street, Feigin Tower, Room 1025.16, Houston, TX, 77030, USA

    Erin Peckham-Gregory & Nader Kim El-Mallawany

  5. Baylor College of Medicine Children’s Foundation Tanzania, Mbeya, Tanzania

    Liane R. Campbell

Authors
  1. William Kamiyango
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  2. Jimmy Villiera
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  3. Allison Silverstein
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  4. Erin Peckham-Gregory
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  5. Liane R. Campbell
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  6. Nader Kim El-Mallawany
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Corresponding author

Correspondence to Nader Kim El-Mallawany.

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Kamiyango, W., Villiera, J., Silverstein, A. et al. Navigating the heterogeneous landscape of pediatric Kaposi sarcoma. Cancer Metastasis Rev 38, 749–758 (2019). https://doi.org/10.1007/s10555-019-09823-3

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