Case of the Month: September 2000

Discussion: Kaposi sarcoma (KS) is observed predominantly among homosexual or bisexual men infected with HIV. In fact, the observation in 1981 of KS among homosexual men in New York City and California was an early clue to the existence of the syndrome that came to be known as AIDS. This tumor is rarely diagnosed among U.S. children, with the occasional exception of Haitian children with vertical HIV infection or older adolescents. Kaposi sarcoma is observed more commonly among HIV-infected children in some other geographic locales, including parts of Africa (e.g., Zambia and Uganda) and Romania.

Kaposi sarcoma has been linked to infection with a novel herpesvirus, now known as human herpesvirus 8 (HHV-8) or Kaposi sarcoma-associated virus (KSV). This virus is present in greater than 90 percent of all KS lesions in adults with AIDS. Only a few KS specimens from children have been studied for the presence of HHV-8, but the virus was present in all of them.

Both mucocutaneous and lymphadenopathic forms of KS have been described in the setting of AIDS. Cutaneous lesions can occur anywhere, including the eyelids, nose, or ears. Lesions may be present in the oral cavity. Lesions often are purple or brown in color, and can be flat, raised, or nodular. Dissemination to lungs, liver, and brain can occur. Chest radiograph may reveal multiple pulmonary nodules.

There is limited information on the treatment of KS in children. Remission of disease in association with initiation of highly active antiretroviral therapy has been observed. Such was the case with the Romanian girl pictured here.