Kaposi's sarcoma in childhood: An analysis of 100 cases from Uganda and relationship to HIV infection
✍ Scribed by John L. Ziegler; Edward Katongole-Mbidde
- Publisher
- John Wiley and Sons
- Year
- 1996
- Tongue
- French
- Weight
- 445 KB
- Volume
- 65
- Category
- Article
- ISSN
- 0020-7136
No coin nor oath required. For personal study only.
✦ Synopsis
We report 100 cases of Kaposi's sarcoma (KS) in children under I 5 years of age treated at the Uganda Cancer Institute in the 6-year period 1989-1994. The incidence of childhood KS has risen more than 40-fold in the era of AIDS, and 78% of 63 cases tested were seropositive for HIV-I. There were 63 boys and 37 girls. The median age was 4 years and the median age of onset was 33 months. Tumour distribution was lymphadenopathic and muco-cutaneous, with 2 major patterns: pattern I, oro-facial dominant (79%); and pattern II, inguinal-genital dominant (13%). A newly described herpes-like virus is implicated as the cause of KS (KSHV), and DNA sequences of this virus were present in all of 8 childhood cases tested. If KSHV is a direct cause of KS, this tumour distribution in children suggests mucosal routes of virus entry, possibly during birth or breast feeding. The dramatic increase of childhood KS implies that the prevalence of causative factors is rising in Uganda.
o 1996 Wiley-Liss, Inc.
Kaposi's sarcoma (KS) is now epidemic in countries where HIV is most prevalent. In Kyadondo County, Uganda (a peri-urban region that contains the majority of Kampala hospital referrals), KS accounts for half of the malignancies reported to the Kampala Cancer Registry in 1989-1991(Wabinga et al., 1993)). KS comprises about 5% of AIDSdefining diagnoses, and has an estimated prevalence of 7 to 10% among HIV-infected patients (AIDS Control Programme, 1992; Desmond-Hellman et al., 1991).
Prior to the HIV epidemic, KS was already endemic in Uganda, most common in older men from the western part of the country. Childhood KS was rare. Data from the Kampala Cancer Registry showed no cases recorded in 1954-1960 (Davies et al., 1965) and in 1964-1968 just 2 cases in boys, a crude incidence of 0.3 per 100,000 (Taylor et al., 1973). At the Uganda Cancer Institute, a major referral centre, only 12 cases were seen in 1968-1974 (Olweny et al., 1976). Children had lymphadenopathic KS and an aggressive clinical course. With the advent of AIDS, childhood KS has become more common, both in the West (Gutierrez-Ortega et al., 1989; Beral et al., 1990) and in Africa (Bouquety et al., 1989;Athale et al., 1995).
Epidemologic evidence in Europe and North America implicates a sexually transmissible agent in the aetiology of AIDS-associated KS (Beral et al., 1990). A herpes-like virus, discovered in KS tissues by molecular probe, has emerged as a potential causative agent, although the virus has not yet been isolated from KS tissues (Chang et al., 1994). The best explanatory model posits the existence of an infectious KS agent that is sexually or enterally transmitted, has a long latency, and becomes expressed in circumstances of immune dysfunction (Peterman et al., 1993). Biologic studies support a promotional role of paracrine cytokines, particularly the HIV Tat protein, that incite angiogenesis (Ensoli et al., 1994).
If KS is caused by a transmissible agent, study of childhood KS may reveal clues of maternal passage of a KS agent, as infants and children are unlikely to acquire HIV by the sexual route. We report a retrospective analysis of 100 cases of KS in Ugandan children in the 6-year period 1989-1994.