Pediatric Kaposi sarcoma in context of the HIV epidemic in sub-Saharan Africa: current perspectives

Abstract

The global experience with pediatric Kaposi sarcoma (KS) has evolved immensely since the onset of HIV (human immunodeficiency virus). In this review, current perspectives on childhood KS are discussed in the context of the HIV epidemic in sub-Saharan Africa. Endemic (HIV-unrelated) KS was first described over 50 years ago in central and eastern Africa, regions where human herpesvirus-8, the causative agent of KS, is endemic. With the alarming rise in HIV prevalence over the past few decades, KS has become not only the most common HIV-related malignancy in Africa, but also one of the most common overall childhood cancers throughout the central, eastern, and southern regions of the continent. The unique clinical features of pediatric KS that were described in those early endemic KS reports have been re-affirmed by the contemporary experience with HIV-related KS. These characteristics include a predilection for primary lymph node involvement, significant proportions of patients lacking prototypical cutaneous lesions, and the potential for fulminant disease progression. Other clinical features that distinguish childhood KS from adult disease include disease presentation with severe cytopenias, and the common occurrence of childhood KS without severe CD4 count suppression. Distinct clinical heterogeneity in disease presentation and treatment response have been demonstrated. Long-term complete remission and event-free survival can be achieved—especially in children with lymphadenopathic KS—utilizing treatment with antiretroviral therapy plus mild–moderate chemotherapy regimens that are well tolerated, even in low-income settings. A pediatric-specific staging classification and risk-stratification platform have been retrospectively validated, and may help guide therapeutic strategies. With expansion of the HIV treatment infrastructure throughout Africa, coupled with recent developments in establishing comprehensive pediatric oncology programs, there is great potential for improving outcomes for children with KS. Increased awareness of the unique clinical nuances and collaborative evaluations of pediatric-specific treatment paradigms are required to optimize survival for children with KS.

Keywords:

• Kaposi sarcoma
• pediatric KS
• HHV-8
• KSHV
• pediatric oncology
• global health

Acknowledgments

The authors express gratitude and admiration to the many patients and families battling against the injustice of poverty and the misfortune of severe illness. We thank the many brilliant individuals who have contributed to the development of the pediatric KS treatment programs across the clinical network of sites in Africa set up by the Baylor College of Medicine International Pediatric AIDS Initiative at Texas Children’s Hospital. Especially at the sites in Lilongwe, Malawi and Mbeya, Tanzania, we recognize the expert clinical care and guidance of William Kamiyango, Jimmy Villiera, and Jason Bacha, all of whom made immense contributions to developing the current treatment paradigm. We also gratefully acknowledge the external support of many individuals who have helped guide the clinical programs over the past decade, including Parth Mehta, Jeremy Slone, Carrie Cox, Carrie Kovarik, Michael Scheurer, Carl Allen, Joseph Lubega, Gordon Schutze, Mark Kline, David Poplack, Dirk Dittmer, Asulwisye Kapesa, Phoebe Nyasulu, Avni Bhalakia, Maria Kim, and Saeed Ahmed. We extend sincere gratitude to the many colleagues working at the Baylor Children’s Foundation Clinical Centres of Excellence in Lilongwe, Malawi and Mbeya, Tanzania, the Tingathe Outreach Program, and Kamuzu Central Hospital.

Disclosure

The authors report no conflicts of interest in this work.