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Angiogenesis-promoting gene patterns in alveolar soft part sarcoma.

Alexander J F Lazar¹, Parimal Das, Daniel Tuvin

¹Sarcoma Research Center, The University of Texas M. D. Anderson Cancer Center, and Department of Pathology, Texas Children's Hospital and Baylor College of Medicine, Houston, TX 77030, USA.

Clinical Cancer Research : an Official Journal of the American Association for Cancer Research

|December 21, 2007

Summary

Alveolar soft part sarcoma (ASPS) is a vascular tumor characterized by the ASPSCR1-TFE3 fusion transcript. Targeting angiogenesis-promoting proteins may offer new therapeutic strategies for this rare sarcoma.

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Area of Science:

Oncology
Molecular Biology
Genetics

Background:

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma.
ASPS is characterized by high vascularity and metastatic potential.
The ASPSCR1-TFE3 fusion transcript is a hallmark of ASPS.

Purpose of the Study:

To investigate the molecular determinants of angiogenesis in ASPS.
To identify potential therapeutic targets for ASPS.

Main Methods:

Retrospective review of 71 ASPS patients' records.
Analysis of 33 ASPS tumor samples for ASPSCR1-TFE3 fusion transcript using RT-PCR.
Angiogenesis oligomicroarrays and immunohistochemistry were performed on tumor samples.

Main Results:

ASPSCR1-TFE3 fusion transcripts were detected in 16 of 18 ASPS samples.
18 angiogenesis-related genes were upregulated in ASPS tumors compared to normal tissue.
Immunohistochemistry confirmed overexpression of jag-1, midkine, and angiogenin.

Conclusions:

ASPS exhibits a unique angiogenic signature compared to other sarcomas.
Despite frequent metastasis, ASPS has a favorable outcome, with mortality driven by resistant metastases.
Targeting overexpressed angiogenesis-promoting proteins presents a potential therapeutic avenue for ASPS patients.