Alveolar soft part sarcoma

Definition:

Alveolar soft part sarcoma (ASPS) is a rare, histologically distinctive soft-tissue sarcoma typically occurring in children and young adults. Although it displays a relatively indolent clinical course, the ultimate prognosis is poor and is often characterised by late metastases. ASPS is characterised by an unbalanced translocation: der(17)t(X:17)(p11;q25). This translocation causes the fusion of the TEF3 (transcription factor binding to IGHM enhancer 3) with a novel gene at 17q25, named ASPL. Translocation between chromosomes X and 17 is seen in all the tested cases, implicating transcriptional deregulation in the pathogenesis of this tumor.

References:

[1]. A L Folpe, et al. Alveolar soft-part sarcoma: a review and update. J Clin Pathol. 2006 Nov;59(11):1127-32. [Content Brief]

[2]. Amy Ruffle, et al. Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion. Pediatr Dev Pathol. 2019 Jul-Aug;22(4):391-395. [Content Brief]

[3]. G D Letson, et al. Genetic and molecular abnormalities in tumors of the bone and soft tissues. Cancer Control. 2001 May-Jun;8(3):239-51. [Content Brief]

[4]. Lee J Helman, et al. Mechanisms of sarcoma development. Nat Rev Cancer. 2003 Sep;3(9):685-94. [Content Brief]

[5]. Pedram Argani, et al. Aberrant nuclear immunoreactivity for TFE3 in neoplasms with TFE3 gene fusions: a sensitive and specific immunohistochemical assay. Am J Surg Pathol. 2003 Jun;27(6):750-61. [Content Brief]

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