# The der(17)t(X;17) of alveolar soft part sarcoma fuses TFE3 to ASPL

Source: https://onco.cc/key-papers/paper-ladanyi-aspl-tfe3-oncogene-2001/  
OnCo record `paper-ladanyi-aspl-tfe3-oncogene-2001` (Key paper). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

This study identified the ASPL-TFE3 gene fusion created by the characteristic chromosome translocation in alveolar soft part sarcoma, giving the disease a defining molecular marker and a diagnostic test.

## Summary

Molecular cloning of the unbalanced der(17)t(X;17)(p11;q25) translocation in alveolar soft part sarcoma showing fusion of the ASPL (ASPSCR1) gene on chromosome 17 to the TFE3 transcription factor gene on the X chromosome, producing a fusion transcription factor.

## Fields

- Kind: Key paper
- Last checked: 2026-09-17
- Journal: Oncogene
- Year: 2001
- DOI: 10.1038/sj.onc.1204074
- Authors: Ladanyi M, Lui MY, Antonescu CR, et al.
- Findings: ASPL-TFE3 fusion identified as the consistent alteration in alveolar soft part sarcoma.
- What it means: TFE3 immunostaining and ASPSCR1-TFE3 fusion testing confirm the diagnosis of alveolar soft part sarcoma and link it to a family of TFE3-rearranged tumours including a type of renal cell carcinoma.
- Caveats: No direct therapeutic target has followed from the fusion itself.

## Sources

- Oncogene 2001: https://doi.org/10.1038/sj.onc.1204074
- PubMed: https://pubmed.ncbi.nlm.nih.gov/11244503/

## Connected records

- cancers: [Alveolar soft part sarcoma](https://onco.cc/cancers/alveolar-soft-part-sarcoma/)
- journals: [Oncogene](https://onco.cc/journals/oncogene-journal/)

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