Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hey everyone, I am Nino, 24yo from Berlin/Germany and I was diagnosed with ASPS last year.

I was on holidays with some friends in Sweden in July 2022 when I suddenly experienced three seizures within a matter of a few days. MRI in a swedish hospital revealed two lesions in my brain (1cm and 0.5cm).

Back in Germany the larger lesion was resected and diagnosis of an ASPS met was done. I had further staging scans like PET-CT, CT and MRIs which revealed some pulmonary nodules, though no hint to a primary tumor. There was a large lesion about 3.7cm in the right lower lobe and a few small ones <0.7cm. At first they wanted to start me on pazopanib. After some research I found out that ASPS can be misdiagnosed as arteriovenous malformation which I had one resected (6cmx2cm) in March 2015 from my right calf, for which I was in and out of hospitals since 2012.
Pathology back then did not reveal any malignancy. I told that to my sarcoma oncologist and she requested the pathology report to be redone and indeed it revealed ASPS. After that the plan was to resect the other brain lesion and the largest nodule from my right lung. So another craniotomy was done and I received ~25gy radiation after resection over a course of two weeks to the resected areas. After that a UVATS was done to remove the large nodule in my left lung. Pathology revealed that it consisted of 4 mets, the largest being max. 3cmx2cmx1cm. A month after that decision was made to remove nodules on my right lung as well. 10 resections were made, but only 6 were confirmed to be ASPS, one was even noted as almost entirely regressed.

Anyways, on my next CT and MRIs at the start of February two new lesions were found in my brain. One 8mmx4mmx3mm, which in retrospective was already visible on scans in July 2022 (~1-1.5mm) and one small lesion which did not have any dimensions mentioned. On scans it does not look much larger than around 2mm. Other scans were clear. To both I received STS with around 27gy in a single fraction just two weeks ago.
I had also a separate MRI done to my calves as the primary was most likely not resected with negative margins back in 2015. MRI revealed a small lesion in the Tibia, around 9mmx6mm. Pathology report said it might be an AVM or residual disease/recurrence of ASPS. My oncologist says it is unlikely that it is a recurrence, especially after that long time, but they will keep an eye on it. The scan in February did note that the measurements stayed the same.

Because of the dynamics in new brain lesions (which I received STS to) they want to start me on systemic therapy as soon as possible. They requested Pembro/Axitinib combination at my health insurance and that might take some more weeks to get approved.

I am unsure about going that route right away. I have done a lot of research within the past 6 months regarding ASPS, systemic treatment and also treatment in malignant melanoma and ccRCC. I do not see a reason to go both TKI and Immunotherapy just yet (though that might change if the next scans show many new lesions). As some published expert opinions on RCC state that on NED disease with TKI/Imm combination one might encounter unnecessary resistance to both drugs and unnecessary toxicity and that it might impact sequential treatment lines.
If the next scan would not show any new lesions, which we know might happen with the course of this disease - maybe mono-immunotherapy might be enough for now -> looking at KEYNOTE studies done on RCC, which showed no benefit in using TKIs as adjuvant treatment, though in the KEYNOTE 564 study adjuvant pembrolizumab showed great benefit in the M1 NED group, although both drug and placebo group were rather small to draw definite conclusions.
Also I am cautious about using TKIs on brain mets as quite some reports on ASPS brain mets treated with TKIs mention cranial hemorrhaging and seizures.

Hi Nino
Welcome .

Wow what a whirlwind of procedures you’ve been thru in a years time ! How are you feeling?

Before you embark on an immune therapy I’d research the
bolstering of your immune system as you’ve been through a myriad of immune suppressive procedures. That is your most important duty to attend to make sure you get the best possible outcome ie immune response from the immune therapy ,to combat your ASPS .
Waiting for your next scan is a really good idea.

Surgeries eliminate the tumors but suppress a persons immune response so the micro- Mets are able to proliferate and evade our immune systems response to destroy them.

How big was your primary in 2015?

Here’s a really good post from Ivan and Olga , 2 of the administrators of cureasps .

https://cureasps.org/forum/viewtopic.php?f=1&t=1701

Hey Debbie, thanks for your reply. Actually I am doing quite well, physically and mentally. Since my thoracotomy I have been doing lots of active recovery but also took a rest when it became necessary.
I am also following almost all of the advice already, eating clean, walking and stretching daily, only need to get back into the gym, I did not want to overwork my body too fast after all the procedures.
I am also doing Mistletoe injections since December to kind of activate my immune system metronomically, so that might help with getting it back to normal. (I got that tip from another german ASPS patient, she had brain+lung recurrence in 2004 - 10 years after her primary - which was treated with chemo and metastasectomies, started mistletoe injections afterwards and has been NED to this day)

And as stupid it may sound I am also kind of glad that after all those procedures the scans only showed these two new lesions and everything else came back clear.

My primary was 6cmx2cm.

Nino.

And as stupid it may sound I am also kind of glad that after all those procedures the scans only showed these two new lesions and everything else came back clear.

It’s really a good sign that your growth of the brain tumor that was a little less than 2mms, now 8mm, grew like 1mm a month I believe ? In spite of all the surgeries and ablations.
The clean living as well as not having the tumor load , seems to be very beneficial to you in my opinion .☺️

So you were 16 when your primary was removed ?
Did you know of the primary prior to its removal?
I’m curious if it could of been evident 2-5 years prior ?

That’s awesome that you are in touch with another ASPS patient. Having that support is invaluable to our mental health which is just as important as all the meds and procedures combined.

I agree with you, taking it as a good sign.

Yes, I have been 16 when it was removed, one month before my 17th birthday.
We noticed it first when I was around 13 years old, as my shin started to hurt when running for longer periods of time. Scans later revealed that the tibia was fractured a bit due to the pulsation of the tumor. Also it was well visible that my shin was (and still is) kind of curved towards the outside. In 2012 I was sent from a local hospital to a sarcoma centre because they suspected a sarcoma. Unfortunately that sarcoma centre diagnosed me with benign hemangioma and treated the tumor with embolisation, which - after some short follow up scan - did not work to stop the blood flow. Finally in 2015, in a different hospital, the diagnosis of an AVM was made and the tumor removed.

Nino
Looks like historically your ASPS chemical makeup lends itself to a slow growth ? It was 6cm at 13? Or was it half that size say 3cms?

I just checked the first report I received about the tumor in 2012. On the sonography report the tumor size was noted as 6cmx3cmx3cm (also that report mentions that I first noticed the bump at my shin in 2011 already). So it seems like it did not grew too much until 2015 or maybe just in one dimension, as the surgery report of 2015 only notes 6cmx2cm (so maybe 3rd dimension was around 6cm as well?). But I am also not too sure on how accurate sonography measurements are compared to MRI or other scans 🤔

EDIT: I just checked an MRI scan from end of 2014 and did measurements by myself, although measuring is kind of difficult as the tumors shape is somewhat irregular. I think the surgery report measurements are rather inaccurate. I measured ~5.9cmx3.2cmx4.2cm.

Hey Nino
Thank you for being forthwith on the information.
You’ve kept really good records and that’s excellent news and should be very very helpful for your future care !

It’s my understanding that the earlier that a patients asps begins to grow , that it appears to take on a different response compared with older patients?
I’m certainly not a doctor but I read alot and did spend the first 5 years of our sons dx’d on the computer trying to begin to understand ASPS.
This forum , cureasps.org has been a life saver for our family.

Here’s a link to a sarcoma website referred to as Libby Shriver . The parents had a child diagnosed with a sarcoma. They ( husband and wife ) set up this foundation to cater to information and services for sarcoma patients, as sarcomas are 1% of cancers and rare so consequently aren’t as well funded through grants etc.


http://sarcomahelp.org/asps.html

Yes, that is what I found too. I think that old clinicopathological study from 1989 already noted a link between age and outcomes, though there is no definite explanation to that. Also the study from 2006 by Kayton et al. noted favorable outcomes in young adolescents, although that might be correlated to the use of metastasectomies. I think most studies hypothesize that age correlates with smaller tumor size (because of a higher incidence of head and neck site) and thus a less likelihood of spread. But of course we know that ASPS spreads very early so not sure if that really makes a lot of sense. I think there was even an old study that actually had patients with tumors <5cm have worse outcomes than patients with larger primaries.
Maybe it also as to do with the immune system and age, as immune function wears off as we grow older and the younger the patient the better the immune system is able to fight the disease on its own. But this is still just a lot of guessing.

I agree, that forum has definitely been a great resource to learn from other patients experiences and I am very grateful for that as well. It definitely summarizes much more data than you could find in any databases or published studies regarding all the possibilities of treatment in every course of this disease.

Hi Nino
I have to compliment you on a very good understanding of the disease and studies done, you are a quick learner.
Re. Deb advice about preparing for the immunotherapy - read on microbiome and add non soluble fibre to feed it.
There is a lot we have written about.
We recently added the psyllium husk every morning over the cereal and yogourt .

As for the systemic therapy, at the time, we decided against the TKI and Ivan had pembrolizumab only. I do not see any indications that the combo works better, it increases the response rate insignificantly at the expense of the higher toxicity but does not increase the survival/response time. Perhaps because there are side effects and the immune system is affected as a result, i.e. counterproductive.
And you are correct about the scars propensity to bleed under the TKI.
Also to let you know, if any of the brain mets scars inflame or bleed you can have the second radiosurgery again to burn it a bit or the microsurgery if accessible. We had the patients to go trough it.

Hi Olga,
thank you, this forum and all the members contributions have made it much easier to dive into the important points of the disease.
Yes, I am aware of it, also psyllium husk looks like a great addition. Judging by a quick search it looks like it is increasing those bacteria cultures that are associated with response to PD1 therapy. I will definitely add it to my breakfast, so far I only added flax seeds.

Regarding PD1/TKI combination that is exactly the same way I see it. But for now it is really just waiting for the next scan and also waiting for insurance approval and after that I will discuss it with my oncologist again.

Also good to know regarding treatment for inflammation or bleeding, thanks!

Hi all,
I have had discussions with my oncologist the last few weeks regarding the possibility of pembro monotherapy and the side effects of axi/pembro therapy. She understood my concerns and consulted again with the head of the sarcoma center. However, she also concludes that combination therapy is the better choice in my case. They understand that ASPS can have a very unpredictable and quite rapidly progressive course and they assume that my disease is rather active at the moment and they would achieve the best tumor control with combination therapy.
In addition, it is also very unlikely that my insurance company would approve axi additionally at a later date if I wanted to add it to pembro.

So in the end I agreed to combination treatment and I am going to receive my first infusion in about 3 weeks after my next scans.

Hello Nino,

Thank you for the update .
Will you start with axitinib, then introduce Keytruda weeks after ?
How are the brain tumors doing ?

Do let us know how you are doing.
Wishing you the best ❣️
Love,

Hey Debbie,
thank you for your wishes :)
My oncologist told me I should recover from my cold that I have right now and just start axitinib after that.
I can't tell you anything about the brain tumors, as I did not have any scans for them so far, they decided to just go with my regular schedule and check them then. But so far I did not have any side effects like nausea or headaches 😅
Otherwise I am doing really good, I had a great time on my vacation in Prague and I also started going to work again last week. It is just a bit of a pain that I can't do a lot of sports right now as I contracted covid about two months ago and caught a cold last week