What is the global prevalence of Kaposi sarcoma (KS)?

Updated: Mar 30, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Four groups are predisposed to Kaposi sarcoma including (1) older men of Mediterranean and Jewish lineage; (2) Africans from areas including Uganda, the Congo Republic, Congo (Brazzaville), and Zambia; (3) persons who are iatrogenically immunosuppressed; and (4) men who are homosexual. Kaposi sarcoma traditionally is an uncommon disease in middle-aged and elderly European men of Mediterranean or Jewish lineage.

A similar focus of Kaposi sarcoma exists in the same age and sex groups in Africa. If a crudely calculated incidence of 28 cases of Kaposi sarcoma per 100,000 in the Arabian population is correct, Kaposi sarcoma may be more common among Arabians than among Mediterranean people. A previously unrecognized genetic predisposition for Kaposi sarcoma among Arabians has been suggested. The incidence of Kaposi sarcoma among renal transplant recipients may be as high as 3.5% or higher in regions endemic for Kaposi sarcoma, which is significantly higher than the 0.4% incidence renal transplant recipients in the United States and Western Europe.

Endemic African Kaposi sarcoma has accounted for 10% of cancers and has been seen in a male-to-female ratio of 15:1. The Kampala Cancer Registry, one of the continent's first and foremost, has shown a significant alteration in the incidence of Kaposi sarcoma in the era of AIDS. In Uganda, Kaposi sarcoma has caused almost one half (48.9%) of cancer cases in men and 17.9% in women. The incidence in men (30.1 cases per 100,000) represents a more than 10-fold increase in men since the 1950s and is approximately 3 times the incidence found in women (11 cases per 100,000). The incidence in boys and girls was approximately the same in childhood (birth to 14 y), with small peaks in girls younger than 5 years and boys aged 5-9 years. Subsequently, a progressive rise in incidence peaked in women aged 25-29 years and in men aged 35-39 years. Lymphadenopathic Kaposi sarcoma affected 12% of total cases; 42% of childhood cases were of this type.

Of 73 Ugandan children with epidemic Kaposi sarcoma from the Uganda Cancer Institute in Kampala (from 2004-2007), 37 were boys and 36 were girls, with a median age of 10.1 years (range 2-18 y). [22]

In neighboring Zambia, the disorder was particularly aggressive among children, more than 80% of whom were HIV seropositive. Kaposi sarcoma was found to represent as much as 25% of childhood cancers. The average male-to-female ratio was 1.76:1, with male predominance higher in children older than 5 years (2.5:1 ratio) than in children younger than 5 years (1.4:1 ratio). The prevalence of HIV-related Kaposi sarcoma seems to be increasing in Nigeria, probably owing to more females having HIV disease. [23]

In Italy, KS-AIDS has produced notable epidemiologic changes. A doubling of Kaposi sarcoma incidence rates was noted in Italian men younger than 50 years from 1976-1984 to 1985-1990; however, no change, or possibly a decline, was observed in older men. The incidence of Kaposi sarcoma was estimated in the region around Venice, Italy, with rates higher in the coast and alpine valleys; in the latter, there was an excess of cases for both sexes combined (SIR = 191.1; CI = 113.2-302.0). [12] The standardized incidence rate for Kaposi sarcoma in Italy declined markedly in the highly active antiretroviral treatment (HAART) period. [24]

Classic Kaposi sarcoma in Greece seems to have an older age of onset; lower male-to-female ratio; endemic clustering; and disseminated skin disease at diagnosis, often accompanied by lymphedema and not unusual visceral or lymph node involvement. [25, 26, 27] A clustering was noted, with a high proportion of the patients being born in Peloponnesos (42.42%) and residing in Athens (51.51%) or in Peloponnesos (24.24%).

The incidence rates of classic Kaposi sarcoma in Italy after the spread of AIDS was evaluated. [13] The rates were 1 case per 100,000 population in men and 0.4 case per 100,000 population in women, varying from 0.3 cases per 100,000 population for men in Umbria and 4.7 cases per 100,000 population for men in Sassari in men and from 0.1 case per 100,000 population for women in Parma and 1.7 cases per 100,000 population for women in Sassari.

The rate of classic Kaposi sarcoma in southern Sardinia (Italy) from 1998-2002 was found to be 2.49 cases per 100 000 population per year (standardized), which was the highest rate recorded in the island. [28]

A Finnish nationwide registry–based analysis of cancer clustering detected a strong familial occurrence of Kaposi sarcoma. This study found Kaposi sarcoma had a very high cluster score, with Kaposi sarcoma patients forming clusters of close relatives. [29] One family was documented with 5 affected individuals in 2 generations. Several other families had 2 first-degree relatives with Kaposi sarcoma.

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