Cure Alveolar Soft Part Sarcoma International (iCureASPS)

We discussed before that the presence of the primary tumor could be involved in less efficient antitumor activity of antiangiogenic agents. We were mainly talking about the TKI drugs that many of our patients are on with the limited/transient efficacy, and if the unresected primary tumor presence might reduce the efficacy of the TKI drugs (we have some ASPS patients with the unresected primary tumors when it was found at the already metastasized stage, we generally always advocate the primary resection as possible although our suggestions are based on the mostly anecdotal data when people with the primaries resected fare better). This subject is discussed all over diseases spectrum and is addressed in the new article publ. in Ann Surg Oncol. 2014 Jan 14.
" Bevacizumab Efficacy in Metastatic Colorectal Cancer is Dependent on Primary Tumor Resection."
http://www.ncbi.nlm.nih.gov/pubmed/24419756
The authors performed a retrospective analysis of factors associated with overall survival (OS) in a study cohort of 409 mCRC patients and it was found, that addition of bevacizumab to chemotherapy is associated with improvement of OS ONLY in patients with primary tumor resection.
If you find yourself in a situation when the oncologist is not supportive to an idea of resection of the primary tumor, this article might be used as one of the blocks of the logical ground for the primary tumor resection justification before you start any systemic treatment.

Dear Olga,
As always, thank you for thoughtfully sharing important researched and published information to help guide ASPS patients in making important treatment decisions. As you have said, we ( you, Ivan, I , and some others) have always advocated the resection of the primary tumor if possible based on our extensive anecdotal experience and observations that "the unresected primary tumor presence might reduce the efficacy of the TKI drugs" (and probably any systemic treatment), and that "people with the primaries resected fare better" in terms of increased, longer, and more sustained efficacy of the drug and overall survival (OS). Tragically, I am not personally aware of any ASPS patient who has had long term survival without the resection of their primary tumor which I attribute to the increased tumor burden that the primary tumor places on the patient's body which then reduces the body's immune system from being able to better fight this very challenging disease, while the primary tumor may continue to release tumor cells into the blood stream to metastasize throughout the body. Of course, this is just my personal theory based on extensive research and observation during our twelve and a half year ASPS journey, and as with everything with this extremely rare disease, there remain so many unknowns.
With ongoing deepest gratitude for your faithful sharing and with special caring thoughts and continued Hope,
Bonni