Alveolar soft‐part sarcoma: a review and update

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Alveolar soft‐part sarcoma: a review and update


Abstract

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.

Alveolar soft‐part sarcoma (ASPS) is a rare, distinctive sarcoma, typically occurring in young patients. Despite a relatively indolent clinical course, the prognosis is poor and is often characterised by late metastases. Since its formal description by Christopherson et al at Memorial Sloan Kettering Cancer Center, New York, USA,1 ASPS has been the subject of considerable interest for pathologists and clinicians, owing to its unique microscopic features, uncertain line of differentiation and unpredictable clinical behaviour. In the past several years, our understanding of the genetic events underlying the pathogenesis of ASPS has greatly increased. This article reviews the historical, histopathological, ultrastructural, immunohistochemical and genetic aspects of ASPS.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/