Cure Alveolar Soft Part Sarcoma International (iCureASPS)

I thought I would post this, as some ASPS patients are being offered XL880, an Exelixis drug that has both oral met inhibitor activity and anti VEGF. We applied to be included the the trial because of its promise in papillary CA (and the met data in ASPS from Sloan Kettering) and our knowledge that our daughter's ASPS primary was positive for both met and VEGF.

We ultimately were denied access at Dana Farber because John Goldberg had taken a job in Florida, and the peds onc that we were to inherit (all sarcomas were to go to her) was not interested in new therapies and was concerned about the risk of putting 'K' into an adult trial. There was an abstract about XL880 that said that one adverse reaction occurred in a patient had previously been on Sutent (I think it was hypertension) - and that patient left the trial. Our daughter had been on Sutent, so we thought, just as well she couldn't get in. Besides the commute (Seattle to Boston) would have been horrendous.

We subsequently read about Jonny May (an ASPS patient with a blog, also registered at alveolarspsarcoma.net) and his unsuccessful experience with XL880. He left the study after 6 months. His post: http://www.jonnymay.org/?p=86

Papillary CA and ASPS share an identical gene splice abnormality, but Papillary CA is a balanced translocation, whereas ASPS is not. That may mean ASPS is more unstable and prone to more genetic variation (why some tumors must be removed surgically, and not all tumors may respond to medical treatment).

Our oncologist speculated that the met inhibitor effects of XL880 are fairly weak - that it is more a VEGF inhibitor than met inhibitor, and he expressed his preference for ARQ197 over XL880 for that reason, but that is all speculative, I think. The data are too early to tell.

Hope this may help some of you. If XL880 and even Gemzar are discussed more commonly as possible treatments for stage IV ASPS patients, perhaps Olga you could give them their own New Topic rather than filed under Other Clinical Trials?

Dear 'F',
Thank you very much for sharing this important information. Unfortunately the information which you have found in your research seems to indicate that the results of the XL880 treatment for ASPS have been quite discouraging thus far, and this is certainly something which we will address and discuss with the oncologist who has recommended XL880 as a possible promising systemic treatment for Brittany. Once again, your gracious information sharing has illustrated the vital importance of this Web site and its participants in providing invaluable information to our ASPS Community so that we can make the best possible treatment choices and decisions. Thank you again 'F'. We are so very appreciative of your continued sharing.
With deepest gratitude, special caring thoughts, and continued Hope,
Bonni

We should be careful about concluding much from one case. In fact in the blog, this person talked about the decrease in PET activity and (I think) disappearance of some lesions on XL880, but then progression and new appearance of others. He also ran into the problem of being told his nodules were stable, but really it was growth that was under the 20% required for RECIST criteria. And who knows? Maybe nodules were growing on XL880, but not as fast as when he wasn't on any drug?

There may also be different steps when drugs are effective - perhaps he quit the drug because new small nodules were appearing or growing, but XL880 could have had some effect on the larger ones.

So not to muddy the waters too much, but it is still possible XL880 may be helpful for some people with ASPS.

Dear 'F',
Thank you for the additional clarification of the information which you provided. We of course understand that it is difficult to judge the apparent success or failure of a treatment on limited data or on just one individual's anecdotal experience, but it is still very helpful to know as much as possible about all treatment results when we are trying to research and gather information upon which to form the basis of important treatment decisions. Because ASPS is typically an indolent disease that even with no systemic treatment at all can sometimes exhibit incidences of unexplained tumor stabilization, shrinkage, and/or disappearance of tumors ( as when all except four of Brittany's fifty+ lung mets suddenly and inexplicably disappeared in 2002), it further complicates the problem of trying to determine if the stabilization or shrinkage of the tumors is due to the systemic treatment or just the nature of this unpredictable and poorly understood disease. However, with this disease we unfortunately don't have the luxury of being able to wait for several years for long term study results and data, so we must try to make the best treatment decisons based on the limited information that we do have. I have contacted our consulting oncologist at Dana Farber to try to obtain more data and input regarding the results of the XL880 treatment thus far for ASPS patients who have participated in the Clinical Trial, and I will update the Web site if and when he provides me with any new information. Hopefully he will have more promising data to share which will offer better encouragement to all of us who are so desperately seeking an effective treatment and ultimately a cure for this insidious disease.
With special caring thoughts and continued Hope,
Bonni