Alveolar Soft Part Sarcoma (ASPS)

Overview

Alveolar soft part sarcoma (ASPS) is a rare, slow-growing but highly metastatic soft tissue sarcoma defined by the pathognomonic ASPSCR1-TFE3 fusion gene arising from the unbalanced translocation der(17)t(X;17)(p11;q25). It predominantly affects adolescents and young adults. ASPS is characteristically resistant to conventional cytotoxic chemotherapy; treatment increasingly relies on anti-angiogenic agents (sunitinib, cediranib) and, more recently, immune checkpoint inhibitors.

Cohorts in the corpus

  • mixed_pipseq_2017 — PIPseq pediatric pan-cancer cohort (Columbia University Medical Center), which includes an ASPS case among 101 high-risk pediatric patients PMID:28007021.

Recurrent alterations

  • PIPseq cohort: ASPSCR1-TFE3 fusion identified by RNA-seq as diagnostic of alveolar soft part sarcoma in a pediatric patient PMID:28007021.

Subtypes

  • Defined by ASPSCR1-TFE3 fusion; no recognized molecular subtypes beyond this defining event.

Therapeutic landscape

  • Anti-angiogenic agents (sunitinib, cediranib) show activity in metastatic ASPS.
  • Immune checkpoint inhibitors (atezolizumab) under investigation.
  • Conventional cytotoxic chemotherapy is largely ineffective.

Sources

  • PMID:28007021 — Oberg et al. PIPseq pediatric pan-cancer sequencing program (n=101).

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