What is endemic African Kaposi sarcoma (KS)?

Updated: Apr 11, 2019
  • Author: Jessica Katz, MD, PhD, FACP; Chief Editor: Edwin Choy, MD, PhD  more...
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Answer

This entity occurs primarily in men but also in women and children who are HIV seronegative and may have an indolent or aggressive course. It was relatively common before the AIDS epidemic. Since the advent of AIDS, its incidence has increased about 20-fold in the African countries of Malawi, Swaziland, Uganda, Zambia, and Zimbabwe. [36, 1]

In central Africa, Kaposi sarcoma has become the most prevalent form of cancer in men and the second most prevalent in women. [37] It represents about 9% of all cancers seen in Ugandan males. This form of the disease involves lymph nodes more commonly than the classic variant. Factors associated with risk in HHV-8 seropositive patients are not as well characterized.

Rarely or never wearing shoes is associated with an increase of endemic Kaposi sarcoma in rural areas with volcanic clay soils, possibly related to chronic lymphatic obstruction in the feet and legs from fine soil particles. [38, 39] Relative affluence in Ugandan patients is associated with increased risk for endemic Kaposi sarcoma, a finding similar to that of Kaposi sarcoma in AIDS patients. [35]

A lymphadenopathic form of Kaposi sarcoma is also seen in Africa, chiefly in HIV-seronegative children before the age of puberty. Generalized lymphadenopathy is seen with visceral involvement and carries a dismal prognosis, with a 100% fatality rate at 3 years.


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