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Updated: Mar 31, 2026

A Mouse Model of Incompletely Resected Soft Tissue Sarcoma for Testing Neoadjuvant Therapies

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Alveolar Soft Part Sarcoma.

Omar I Jaber, Patricia A Kirby¹

¹From the Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City.

Archives of Pathology & Laboratory Medicine

|October 31, 2015

Summary

Alveolar soft part sarcoma (ASPS) is a rare cancer defined by a specific genetic translocation. Detecting the ASPSCR1-TFE3 fusion transcript offers a sensitive diagnostic method for this challenging neoplasm.

Area of Science:

Oncology
Genetics
Pathology

Background:

Alveolar soft part sarcoma (ASPS) is a rare neoplasm affecting soft tissues or head and neck regions.
ASPS typically presents as a slow-growing mass or metastatic disease.
A characteristic chromosomal translocation, der(17)t(X:17)(p11:q25), defines ASPS.

Purpose of the Study:

To highlight the diagnostic utility of the ASPSCR1-TFE3 fusion.
To discuss the role of TFE3 immunohistochemistry and molecular detection.
To explore emerging targeted therapies for ASPS.

Main Methods:

Immunohistochemistry for TFE3 detection.
Real-time polymerase chain reaction (PCR) for ASPSCR1-TFE3 fusion transcript detection in paraffin-embedded tissues.

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Analysis of the MET proto-oncogene signaling pathway.

Main Results:

The ASPSCR1-TFE3 fusion transcript is a specific diagnostic marker for ASPS.
Real-time PCR is more sensitive and specific than TFE3 immunohistochemistry for detecting the fusion.
Cathepsin K immunohistochemistry can assist in diagnosis.
The ASPSCR1-TFE3 fusion protein influences the MET pathway, suggesting therapeutic targets.

Conclusions:

The ASPSCR1-TFE3 fusion is a defining feature of ASPS, with molecular detection offering high diagnostic accuracy.
Targeting the MET pathway holds promise for novel therapeutic strategies in ASPS.