Alveolar soft-part sarcoma (ASPS) is a rare, distinctive sarcoma, typically occurring in young
patients. Although it displays a relatively indolent clinical course, the ultimate prognosis is
poor and is often characterised by late metastases. Recently, our understanding of the
genetic events underlying the pathogenesis of ASPS has greatly increased. The historical,
histopathological, ultrastructural, immunohistochemical and genetic aspects of ASPS are
reviewed in this article.