Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Ivan
So burn or remove with margins?
Thanks in advance
Debbie

Small ones (I think under 4mm) are ablated in-place. The scar can be bigger (6mm with margins) and can become even bigger due to inflammation later (say 8mm). Then it can stay with that size for years. I think I have some like that in my lungs right now.

Bad news. A met of 1*1*1.3cm was found in the brain MRI on Aug 7th. Didn't have a chance to take a rest before taking up the gun again...

We went to see 3 neurosurgeons. None of them think neurosurgery is an option considering there are some other mets in the brain although they were treated by Gama Knife. So we had Gama Knife again on Aug 9th.

The doctor suggest us start taking temozolomide asap. Does anyone know any ASPS experience with temozolomide on the brain mets? Should I combine it with some TKI that may work on ASPS, like Sutent, Pazuopanib, or everolimus (as reported to work on 3 ASPS patient by the post in the forum: http://www.cureasps.org/forum/viewtopic.php?f=2&t=530)?

The doctors can only offer Chemo and whole brain radiation, but Bonnie says this dosen't work on ASPS. And George had a Chemo in 2009 but didn't work. We're in a really tough condition now and not sure what to do. Can anyone please give us some opinions?

PS: George has recovered from the laser surgery very well and he's in very good condition now.

Any inputs are highly welcome here.

Thanks,
Lynette

Dear Lynette,
I don't have an answer for you in reference to the situation! But, i am sure you will be getting a reply from some of the other posters soon!

I do agree 100% no whole brain radiation!

I am happy that George is feeling better an i will keep you both in my thoughts and prayers!

Lynette - can George simply get MRI scans more often so the mets if found are found on a smaller size like 5 mm? Is GammaKnife treatment easy to get for him? I have seen an article that the number of the brain mets that could be treated with Gamma Knife might be bigger than 10 as long as the volume of the mets is small, with the good oncological outcome. How long ago he had the previous brain met found and treated.
The oncologist suggest temozolomide (Temodar in US) because this drug crosses blood-brain barrier. Other meds most probably not. I have no information about expected activity of the temozolomide in ASPS.

Hi Amanda and Olga, thank you so much for sharing your ideas.

We agree with Amanda that no whole brain radiation.

Currently, George has MRI every 2 ~2.5 month. We're also thinking of having MRI done more frequently, like once a month. But the problem is can he have Gama Knife once a month if some small mets are found? Is there any limite--maximum times to the Gama knife?

Last time when he had previous brain met found and treated was on April 1st. The one that found this time was suspicious in the MRI on May 21. All the MRI was done in 3.5mm interval. We will ask the Doctor to do it in 1mm interval next time.

But I think the primary is to find somthing working on the brain mets, right? And we can't know if anything will work without trying it...

As I know, Cediranib and pazuopanib can also crosses blood-brain barrier. But what's the difference of Cediranib and pazuopanib and temozolomide on the brain mets? Different drug concentration in the brain?

Thank you everyone for sharing you ideas.

Dear Lynette,
I am so sorry that George was diagnosed with another brain met, but am grateful that it was able to be so quickly treated with Gamma Knife, and that George has recovered well from the procedure. As you know, based on my 12 years of extensive ASPS research, networking, and observations of other ASPS patients, I strongly disagree with the doctors' recommendation for Whole Brain Radiation (WBR) since ASPS is so notoriously radiation resistant, and I am not persoanlly aware of any ASPS patients who have had successful results and a positive outcome with WBR. Also, unless the chemo that the doctors are recommending can cross the blood brain barriar, it would unfortunately not be effective in preventing the development of new brain mets. Temozolomide (Temodar) can cross the blood brain barriar, but when it was recommended to us for Brittany nine years ago when she was initially diagnosed with brain mets, the radiation oncologist could not provide us with any data regarding its possible effectiveness for ASPS so we declined the Temodar recommendation as well as WBR for the same reason. TKI's such as Cediranib are thought to be able to cross the blood brain barriar, but cannot be used if there are active brain mets due to the risk of cerebral hemmorhage. However, since George's brain mets have now been Gamma Knifed and Hopefully successfully destroyed, I personally think that systemic treatment with a TKI like Cediranib would be a good treatment choice to try to prevent any new brain mets. My concern about the TKI Pazopanib (Votrient) is that it has unfortunately not proven to be very effective for most ASPS patients, and I am personally aware of one ASPS patient who heartbreakingly developed brain mets despite being on Pazopanib. I encourage you to do as much research as possible and to request documented data from the doctors as to the potential success of any systemic treatment that George receives. I know that Cediranib is probably not available in China, but there is currently a Cediranib Clinical Trial in England and also one in the United States if it is financially and logistically possible for you to pursue that treatment option. Also, Cabozantinib,(Cometriq) is another promising new TKI that may be a possible treatment option that may be available in China since it has received FDA approval.
My most caring thoughts and very best wishes are with George and you, and I will be anxiously awaiting your next update. Take care Lynette and keep in touch with the Board as you are able.
Reaching out to share my special caring, healing wishes for George, and continued Hope,
Bonni

Lynette
I am so sorry that the tumor was found to be what you suspected

In your journey to rid Jorge of this met please remember what Bonnie said about treatment with certain TKI's:

' they cannot be used if there are active brain mets due to the risk of cerebral hemmorhage. '

It sounds like you have a great doctor on your side helping you fight this awlful disease
Stay strong and let us know when is good for you
Love
Debbie

You have very good availability of MRI scanning in China. There is no way any of our patients would get it more often than once in 3 months here in US or Canada - and the insurance would not approve the more frequent scanning than that - and they are probably right as there is a minimal size for the mets to be reliably covered by the radiation like 5-7 mm. How big was the met when it was found suspicious by the MRI on May 21? I am trying to figure out the rate (speed) of growth.

I've done some reading on the current information avail. on the strategy of the the treatment for the multiple brain mets - doing Gamma Knife as they appear versus combining it with the WBRT:

1. There is an article re. Gamma Knife treatment being equally effective in setting of the multiple (5-10 versus few (2-4) brain mets:
http://www.ncbi.nlm.nih.gov/pubmed/23839414

2. This is an article from the Gamma Knife Center, Karolinska University Hospital, Stockholm, Sweden and Gamma Knife Centre, Bupa Cromwell Hospital, London, United Kingdom:
Stereotactic radiosurgery in the treatment of brain metastases: The current evidence.
http://www.ncbi.nlm.nih.gov/pubmed/23810288
that shows that WBRT does very little for preventing new brain mets versus simply doing Gamma Knife as they appear:
"New remote brain metastases are reported in 33-42% after WBRT and in 39-52% after radiosurgery " - at first it seems that there is some benefit from WBRT but we have to correct the number by the types of cancers that are very radiosensitive (ASPS is not). They conclude:
"Larger metastases (>8-10cc) should be removed surgically, but for smaller metastases Gamma Knife radiosurgery appears to be equally effective as surgical tumor resection (level I evidence). Radiosurgery avoids the impairments in cognition and quality of life that can be a consequence of WBRT (level I evidence)."

3. There is also a recent study done in a lung cancer setting:
Multiple gamma knife radiosurgery for multiple metachronous brain metastases associated with lung cancer : survival time.
http://www.ncbi.nlm.nih.gov/pubmed/23133721
that shows that multiple GKRS for multiple metachronous brain metastases (they appear with an interval not at once) produces better survival than a single GKRS plus WBRT. They conclude:
"Our result supports that multiple GKRS is of value in extending the survival time in patients with multiple metachronous brain metastases, and that the number of the lesions and the frequency of development of new lesions are not an obstacle in treating patients with GKRS."

Thank you Olga for the important shared information regarding the documented ineffectiveness of WBRT ( Whole Brain Radiation Treatment) for preventing new brain mets. This is critically important information for every ASPS patient with brain mets to know as too often doctors recommend WBRT for patients with multiple and/or increasing brain mets. As shown in the studies which you shared, WBRT unfortunately has not been shown to be successful in preventing new brain mets in patients with cancers like ASPS that are radioresistant, and heartbreakingly WBRT can cause very debilitating cognitive problems with permanent short term memory loss which very negatively affects the quality of Life. It is essential to be as well researched and as knowledgeable as possible before undergoing any type of treatment which the doctor may recommend because the consequences to some treatments can be extremely serious and sometimes fatal.
Lynette, you may want to research and discuss with George's doctors the possibility of adding Rapamune ( an M-Tor inhibitor) to a TKI regimen because Olga has posted information on this Board in the TKI topic regarding the successful results of this regimen that was experienced by a Sarcoma patient who had developed disease progression while taking Pazopanib (Votrient).
With special caring thoughts and continued Hope,
Bonni

Thanks everyone for your care and comprehensive inputs There are so much info to learn and digest...

Now it's very clear no WBRT now. The brain will be supervised more frequently--MRI once a month.
The answer to Olga's question, the suspecious met found on May 21 was about 3mm. The radiologist considered it as vessel at that time. Actually, there are also 2 suspecious spots found in the positioning scan of the Gama Knife. The Gama Knife doctor (which I think is better in reading the scans than the radiologist) can't be sure about them, so they're left as vessel.

However, fingding an effective TKI to control the brain mets is the top priority now. To be honest, we don't rely on the doctors here any longer, they can't know more than you guys or me about this disease. I know what they would say if I ask them about the TKI, they will tell us to try because they don't know too many cases.

The first VEGFR inhibitor George had in the last 3 month is effective, so we can assume other VEGFR inhibitor Cediranib, Sutent, Pazopanib will also work, right? I think I get this idea from Olga on Josh or Kelvin's topic?
Then I can adding some TKI of another target M-Tor--everolimus, as there is sucessful report on it and I have ordered it already. The availability of the drugs is also an issue.
What do you think?

I will do more research on Cabozantinib, Rapamune, Axitini and see how to obtain them later.

Thank you again for your help!

The Gama Knife doctor (which I think is better in reading the scans than the radiologist) can't be sure about them, so they're left as vessel.

What was the resolution for the MRI? If they do it at 1mm, it should be very possible to compare to previous scans. Something that is 3mm couldn't be missed with that slice size.

What about the lesions that have been treated, do they look like they are resolving on the follow up scans?

The MRI have been done in ~3.5mm slice, which is not enough for the doctor to diagnose.

The other treated mets are stable, but the doctor re-do one (7mm) of them. I guess the radiation dose was not big enough. And the central dose is increased to 40Gy compared to last time.

Jorge, they use CT scan during the procedure - not MRI, correct? I guess from now on with 1mm slice MRI it will be easier to get the nodules diagnosed in a timely manner.

How are you tolerating the gamma knife procedure? Are you experiencing any neurological side-effects during your recovery?

Hi Ivan,
they use MRI to locate the mets.
The procedure is very easy, no pain. But the side effects will come later in a week to ~3 months, like headache, nausea, eyesight degrading. All these symptoms are caused by the radiactive swelling if it's serious enough. But for mets in mm, usually you may not feel anything.