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Updated: Jan 15, 2026
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Establishment of a Primary Culture of Patient-derived Soft Tissue Sarcoma
Published on: April 11, 2018
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Alveolar Soft Part Sarcoma: An Updated Review
Pedram Argani1,2, Cristina R Antonescu3
1Departments of Pathology.
Advances in Anatomic Pathology
|October 16, 2025
Summary
Alveolar soft part sarcoma (ASPS) is a rare cancer. Research has identified its genetic cause, the ASPSCR1::TFE3 gene fusion, clarifying its origin as a unique tumor type.
Area of Science:
- Oncology
- Genetics
- Pathology
Background:
- Alveolar soft part sarcoma (ASPS) was first described in 1952.
- The cell of origin for ASPS remained controversial for decades.
- Recent advances have elucidated the genetic underpinnings of ASPS.
Purpose of the Study:
- To review the clinical and pathologic features of alveolar soft part sarcoma.
- To discuss the current understanding of ASPS based on genetic discoveries.
- To highlight the significance of gene fusion-driven neoplasms.
Main Methods:
- Review of historical and recent scientific literature.
- Analysis of clinical case data.
- Genetic analysis of tumor samples.
Main Results:
- The der(17)t(X;17)(p11;q25) translocation and ASPSCR1::TFE3 gene fusion were identified in 2001.
- ASPS is now understood as a gene fusion-driven sarcoma.
- These tumors lack a normal cellular counterpart.
Conclusions:
- The identification of the ASPSCR1::TFE3 fusion has resolved the longstanding debate on ASPS origin.
- Alveolar soft part sarcomas are a distinct group of neoplasms characterized by specific genetic alterations.
- Understanding the molecular basis of ASPS is crucial for its diagnosis and potential therapeutic strategies.
