Kaposi's sarcoma (KS) occurs in Europe and the Mediterranean countries (cla
ssic KS) and Africa (endemic KS), immunosuppressed patients (iatrogenic or
post-transplant KS) and those with acquired immune deficiency syndrome (AID
S), especially among those who acquired human immunodeficiency virus sexual
ly (AIDS-KS). KS-associated herpesvirus (KSHV or HHV-8) is unusual among he
rpesviruses in having a restricted geographical distribution. Like KS, whic
h it induces in immunosuppressed or elderly people, the virus is prevalent
in Africa, in Mediterranean countries, among Jews and Arabs and certain Ame
rindians. Distinct KSHV genotypes occur in different I,arts of the world, b
ut have not been identified as having a differential pathogenesis. KSHV is
aetiologically linked to three distinct neoplasms: (i) KS, (ii) primary eff
usion lymphoma, and (iii) plasmablastic multicentric Castleman's disease. T
he histogenesis, clonality and pathology of the tumours are described, toge
ther with tile epidemiology and possible modes of transmission of the virus
.