J Clin Pathol 59(11): 1127-1132

Alveolar soft‐part sarcoma: a review and update

A L Folpe, Department of Laboratory Medicine and Pathology, Mayo Clinic Rochester, Minnesota, USA

A T Deyrup, Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, Georgia, USA

Correspondence to: A L Folpe
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA; folpe.andrew@mayo.edu

A L Folpe, Department of Laboratory Medicine and Pathology, Mayo Clinic Rochester, Minnesota, USAA T Deyrup, Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, Georgia, USACorrespondence to: A L Folpe
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA; folpe.andrew@mayo.edu

Accepted 2006 Jan 25.

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.

Abstract

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.

Abbreviations

ASPS - alveolar soft‐part sarcoma

TFE3 - transcription factor 3

Abbreviations

Footnotes

Competing interests: None declared.

Footnotes

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