Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello all,
I'm happy to have found this interesting Forum and love to join this community.
My Name is Saskia from Germany close to Frankfurt/Main, 23 years, female.
I was diagnosed ASPS with lung mets end of April after biopsy and pathology.
My primary is in my leg/femoral with a size of 12,5x7x3 cm and around 100 very little mets all over my lungs. I alsIo had a biopsy of 2 mets in may and therefore was able and accepted to a Master Study in Heidelberg.
I was described Cabozantinib 60mg and am on it since mid of May.
Since 2 weeks I have the imagination that the primary shrinks and sometimes is more soften and also drives away from my muscle and nerv in my leg.
I tried to change some more things in my life and habits to support some healing and progress with this fckng cncr….ups sorry.
Apart from healthy food, i aditionally take some alternative products in form of capsules. It is boswelia serrata and curcurma. I also try to digest (it is not yummy) up to 4 spoons of so called linseedoil which has a lot of omega3s combined with curd cheese which relates to some protein combination stuff figured by a biochemist doctor called Johanna Budwig.
For my mental support I figured that I really love to do the "Healing Code" by Alex Loyd and Ben Johnson.
Bless you all and keep living happy.
I'm looking forward to have informative chats and nice contacts here.
Let's help and support each other.

Saskia

Hello Saskia

My name is Debbie and I live here in the USA
My son , Josh was diagnosed ( dx’d) back in 2012 and is currently on immunotherapy and axitinib .
He had just turned 32 years old in 2012.
Here’s his story .

https://www.cureasps.org/forum/viewtopic.php?f=4&t=750
Your discovery of ASPS is not unusual , as is your anger is totally normal to having been dx’d . It simply sucks!

However there is a lot to be hopeful for as we have be so very fortunate to of had a multitude of people before us share all that they know about our so so very rare sarcoma . :)
Hang in there and let us know how you are doing !
Do your professors think you have bone invasion by prescribing cometriq?

Dear all and dear Debbie,

thanks for the reply.
There was no warning to invasion of bones by cabozantinib by my docs and is neither mentioned as side effects as far as I read on my description.
I'll crosscheck.

My next big day with checks will be on 1st of Aug !!! CT lungs for mets, MRI leg for primary shrinking - hopefully- and MRI Abdomen, because I have some stomach trouble sometimes and high liver values (108) at this time.
Keep thumbs up for this day.

Does anybody else has any experiences with cabozantinib and side effects? What dose do you take and for how long?

Saskia

Hi Saskia,

Welcome to the forum that unfortunately no one wants to be a part of, but I am glad you found us. My name is Nhi and I was diagnosed in 2016 with primary to my right gluteus and lungs Mets 4 months after. I started on Sutent which kept me stable for 2 years and recently had progression with lots of bone Mets to the spine, chest bone, iliac bone and skull bones. I am now on Votrient (another TKI drug similar to yours) plus an ICI (keytruda). There is a sub forum that people posted with their experience on certain drugs including ICIs and TKIs. The link is here: https://www.cureasps.org/forum/viewforum.php?f=66

You can also start your own topic there to post about your experience on the drug so others can benefit from seeing it.

It is common to have elevated liver function test on TKIs so make sure you monitor that. I had liver toxicity when I took Sutent and had to hold it for a few weeks and then decreased the dose. It would be good to get full scans including MRI or CT of abdomen/pelvis, bone scans and MRI brain to establish a baseline to ensure you don’t have other Mets.

Dear Nhi,

thanks a lot for your reply.
I pray for your treatment combination of TKI and ICI to work wonderful and will follow your posts for sure.
Maybe you wrote about it already and I didn't see it but why are you on TKI plus ICI? I didn't know this works.

Does anybody also know if it might be possible to take the TKI and additionally get radiation to smaller the primary tumor?
I'd so love to have my primary smaller and have a surgery to (Maybe only for meтtal reasons) get rid of it (my so called Alien).
Sometimes I also think that then maybe my TKI can concentrate on my blood flow and mets instead additionally on the tumor/primary.

The decision for Cabozantinib was done on the figures of pathology of biopsy leg and a VATS of my lung mets according to VEGFR2 and MET as far as I understood.

I already had a MRI brain which was clear and abdomen/pelvis as well. No mets found there.
I didn't have a MRI spine.

Side effects of Cabozantinib 60mg for now: sometimes few cramps in my tummy, getting white hair, pimples.
I can live with all that really well.
Liver will for sure be monitored and my heart as hypertension might occur.

I'm very excited for the checks on 1st of August.

Saskia

Hello all
Unfortunately the checks on 1st aug didnt go well. I have more lung mets and some of them grew as well. My primary didnt grow. I stopped taking Cabozantinib and am on Votrient 800mg daily now. Side effects are worse. So on some days I only take 400mg.
We pushed my health insurance a lot. Thanks to my onc. Approval is given now for ICI (Pembrolizumab and Axitinib) and I will start with it on 20th aug.
On 19th I will have another surgery for a biopsy for pathology on my leg and its primary as we found another professor additionally to the master study I'm inn who will do a different gene analysis and test trestments in vitro.
I am deeply thinking of everybody who is also searching fir the right treatment somehow.

Saskia

It is unfortunate that you did not respond to Cabozantinib but our other patients did not have much success with it either.
Re. Pembrolizumab and Axitinib. The success of this treatment is based on your immune system functioning properly so if you have lots of side effects from the Pazopanib that make you sick and weak, you might consider stopping it early to recover from the side effects. Also make sure you eat and sleep properly, more raw fruits (especially berries with the small seeds are good) and vegetables for the microbiome health.

Saskia ,

I’m sorry for the increase but glad to hear of stable primary .

I agree with Olga in stopping the pazopanib. Sooner than later as it will cause immune suppression and gut microbial issues. Also with the biopsy I’d worry about bleeding issues , while on an anti-vascular Med .

Was your abdomen scan performed as well ?
Glad to hear of the axitinib pembro being scheduled for the 20th :)

Hello Olga and Debbie,
surgery for biopsy went well. Drainage was removed after two days. The healing is slow of course because of the former treatment with pazopanib which I stopped in time as you suggested.
ICI then started on 22nd of august with Keytruda. Dose of 200mg. I was very tired on this day but really happy. With stopping TKI I am very hungry again which is wonderful. Of course I eat a lot of fruits and berries and vegetables for a good microbiom. Additionally I digest daily a spoon of natron in water with magnesium, a chlorophyll powder in water and fiber powder Bios7 (unicity) to get my body more alkaline and to support mikrobiom and my immunsystem.
My abdomen scan was clear.
Axitinib to support the ICI Keytruda will start on 9th of september. We wanted to wait with starting to let my wound heal a bit and to differ side effects between Keytruda and Axitinib. I hope to cope better with Axitinib than I did with Cabozantinib and Pazopanib which was worse.
Next week I will have my second treatment. There will be 6 in total and next scans I guess will be in beginning of december.


My prayers go to Jussis family. I was shocked. He was such a warrior.

Love
Saskia

Hello Saskia
I’m glad to hear you are feeling better and your appetite is returning . :)
In adding the axitinib , we are experiencing a little bit of an appetite reduction ( lost 10 lbs since middle of May ) and also mouth dryness . Overall compared to sutent , axitinib appears to be much more tolerable !

We are all in shock with the news of Jussi’s death.
Hugs coming your way .
Love ,

Saskia, thank you for the update, we all wish you well with the Keytruda response.
You posted that you are taking "natron in water with magnesium, a chlorophyll powder in water and fiber powder Bios7 (unicity) to get my body more alkaline and to support mikrobiom and my immunsystem."
I am not sure what is the reason you think these 3 supplements are going to help with Keytruda response? Have you found any proof studies/officially published articles that would support that? If you have can you share? Eating enough vegetables/fruits makes you body more alkaline already, and it is incorrect to assume that your own microbiome is not good. Also there is no such a thing that supporting immune system - it is very diverse and not all its components need to be supported. Why do you think you need an extra magnesium and not for example zinc? Anything you take may interfere with the K working. Its goal is to prevent some immune system signals to be used by cancer cells. There are other things that you can do to support the immune system - keep the weight stable, sleep well, exercise to keep the muscle dense, avoid stress, have fun as often as possible.
Re. Axitinib. There is no proof Keytruda works better in ASPS with TKI added versus K only, it was never tested alone in ASPS. There are other immune-checkpoint inhibitors that are tested in ASPS alone and they show high activity so far.
Deb, if Josh lost 10 pounds on the combination regimen, it is unacceptable as the stable or even higher weight is needed for the sustained response to immune checkpoint inhibitors, according to recent articles, with sufficient muscle mass to support the immune system functioning well.

Olga
Makes sense
Will research .
Saskia look into as well . :)
Maybe with your tumor load , there might be a need for axitinib ?
Olga what’s your thoughts ..

Love

I can not possibly have any thoughts about something that is not tested somehow - the clinical medicine is an applied science, i.e. we can assume and think whatever we want but until it is tested and the results are published we do not really know. I know that adding the radiation therapy to a tumor at the same time as immunotherapy is given (the same days) may improve the immune system tumor recognition because the results of the clinical trials show that.
Also wanted to add why it is unclear that non-specifically stimulating the immune system while on immune-checkpoint inhibitors treatment might be not a good idea - the immune related adverse effects are caused by some of the immune system components that are running unregulated and unrestricted while on PD-1 drugs. They can be severe and catastrophic so I would not risk trying to increase that. Yes we need the good functioning immune system in order to attack the tumors while the breaks are out, but we have no way in separating the adverse and positive effects of ICI.

Olga
It’s truly an emotional journey and should never be a Hail Mary, ( a reference to football last effort , when not needed) , effort that could turn the tables to our folks making a mistake ? Progression verses reduction . Maybe what you are saying ?

You’ve been with this ASPS journey for LOTS AND LOTS more years than myself .

Targeted therapies have their place ,but Saskia , let the scans make the difference , the choice .
Do not add axitinib until there is discussion to do so .
This is my non doctor opion.

Let us know your findings .

Thinking of you .

Love ,

Dear all

Sorry you haven't heard from me for a while.
I was kind of fed up with all the topics. Now I'm writing with my mum, or she does, to be more precise.
Hello my Name is Tanja, Saskia s mum.
Saskia wasn't well as she had a bad cold and we did all to not get antibiotics or any other medicine which could reduce leucocyts. That's what we learned by now, not to weaken the ICI therapy with keytruda. All went well but Saskia lost some more weight with the cold. She only had 46,5 kg and had 56 in April when all this started. Now she is at 49kg again. Juhu.
Additionally we had some trouble with her wound at her leg because it doesn't heal since last biopsy mid August. A second medical thread was still in but grew out now somehow. We hope it was the last one they forgot.

I can't remember all questions and discussions on our topics here and what is still up to date.
The magnesium she took was only against cramps. She stopped that.
Natron with lemons, boswelia serrata, chlorophyll and curcuma is still on her daily list to get the body more alkaline because tumor cells are more likely to grow in acidic environment.
Once weekly she has a alkaline intravenous infusion also and one hour on her primary with a ionen induction machine to increase tumor cells of 15mV because good cells have around 70mV. This should support the immunsystem to fight against the bad cells. The medical machine is called Papimi.

Saskia is still on her treatment with keytruda, every 3rd week and daily on axitinib pills. There is a clinical study with this combination. I'll ask Saskia to copy the link for you later.

Now comes a But!!!
We had the chance to find a professor who did a more specific analysis from her latest biopsy. He confirmed ASPSCR1-TFE3 fusion according to the sarcoma and a pathogenic substitution on her gene FGFR4.
He tested existing treatments and recommend pathological Pan-FGRF-Tki Erdafitinib.
This treatment is only available in the US at the moment.
Another oncologist in cologne is now discussing her cSe with our oncologist in Frankfurt how to continue or WHT to suggest could help best.
We will stay on the actual treatment till we know any news and will have checks beginning December anyway. Lungs CT, abdomen mrt etc.

What I guess, but am not sure, is that we all should consider if treatments like sutent, votrient, cabozantinib, axitinib and all Tkis from this row are absolutely the right decision for everybody with ASPS because they refer to the genetic pathway for VEGFA.
Do all of you have a specific genetic analysis and was the error named on VEGFA?
Because we now wonder if all this might help? Is there a connection somewhere to errors on FGFR by those Tkis as well?

I don't want to confuse anybody but this idea came into my mind and for sure it will discussed by our oncologists.
Does anybody have an idea here as well or are my thoughts just silly and without any medical. Knowledge - for sure :)

I hope you are all doing somehow well. I will start reading all your recently updated topics on your stories this weekend to be up to date again since it was a while.... Sorry... And all the best for you.

Hugs from a November Grey rainy Frankfurt from
Saskia and Tanja