Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi there,
New to this forum. I was actually shocked to see such an active support channel exists for ASPS. It is comforting.
My partner has just recently been diagnosed with ASPS, aged 32. Primary tumor in his calf (approx 7cm x 4cm) and (5) 1cm nodules in his lungs. The lungs have not been biopsied but it is assumed they are associated with cancer. Prior to official diagnosis, the orthopedic oncologist had been talking about 5 weeks of radiation followed up surgery to remove the primary. After that, the lungs would be monitored. This seemed in line with treatment I had read about online.
Yesterday, we had another follow up appointment to discuss the treatment. The same oncologist has completely changed course after meeting with a board of other cancer specialists he's part of. He is now recommending my partner be treated with chemotherapy, stating that there have been cases where it seems to work. He has referred us to a medical oncologist that we will meet with today.
I don't know why chemo would be the first line of defense when treating this disease? Nearly everything I have read states chemotherapy is generally ineffective when it comes to ASPS, which goes directly against what the doctor stated. They also mentioned possibly not removing the primary tumor at all, unless it starts to cause my partner issues or pain. I can't imagine why you would leave a primary inside someone, when it's as accessible to remove as my partners? This has all been very confusing...
Has anyone else had treatment like this? Should we start looking at new doctors? The one we were sent to is highly recommended, so I don't know if it's worth spending the little time we have seeking out someone else. We have doctors in the family (although they are not cancer specialists in the slightest) that did not seem alarmed by this new plan. They said considering there have been cases where these particular sarcomas respond to chemo and the tumor is slow growing, it seems like an expected path forward. But I feel like something is just not right...

Hello ,

Welcome to the forum.
However I’m sorry to hear that your partner was dx’d with ASPS.

The discussion of chemo ,targeted chemo verses the old fashion incredibly harsh chemos , has been debated
almost since the beginning of time .😏
This sarcoma is so incredibly rare that most docs don’t see it in their life time of practicing .
Our family was lucky to of had our sons 6.5cm upper rt thigh tumor removed ,back in 2012 without a fight.

Ask for papers of chemo being more successful verses surgeries. Surgery has been proven to be successful in more cases with negative margins ,than chemo as I understand it.

How old is Ronald ?

The data out there looks very scary while being researched , but be assured there is lot of hope to be had.
😊
Sincerely ,

Hi Debbie,
Thank you for your reply. Things feel dim right now, so I will take the positivity where I can. I feel like I have been struggling with this diagnosis more than my partner. You're right about the research - everything I read seems terrifying and I usually feel very discouraged after reading it.
Brad is 32 years old, no other health problems to date. We really didn't see this coming, but from reading this forum, it sounds like no one does.
That's a good suggestion, asking for the papers re: chemo vs surgery. I will make sure to do that today when we see the medical oncologist that will be laying out the chemo schedule. I will also make sure to take note of the actual drug names and report back here. Perhaps that will help provide further context.
Glad to hear your son caught things early in 2012. <3
Naynay

Welcome here. To support what Deb posted - it is a general consensus re. metastatic ASPS that the surgery is the most proven way to prolong lives, with some cases even cured by the surgery alone.
Just to give you the general advice re. treatment management. Do not be intimidated by the seemingly qualified ppl opinions. The way a clinical medicine is set is an evidence based one, and their opinions are not the basis for the treatment unless they are proven by the studies. ASPS is a very rare but well studies disease. Ask for any proposed plan to be backed up by the studies and articles. If they do not have them, then propose your own and back it up by the studies, be polite but firmly state your own desire to learn and to proceed on the evidence based basis not on the general sarcoma guidelines or the limited experience by the given oncologist. The medical establishment is often irritated by the amateur attempts to make its own choice, but if they see that you and your partner are learning fast and are using the pier reviewed credible sources (PubMed) then you'd come to an even field. Try not to be the buffer between the relatives. doctors and your partner - he has to be involved himself as this is a life long disease and you can only work as a team to be successful. We welcome him to come here too.

Now back to the subject as how to proceed. Indeed the benefits of removal of the primary tumor is the established in ASPS as the part of aggressive surgical treatment plan overall and the lower tumor load was also shown to improve the response to immunotherapy treatments that are the most promising for now. 7*4 is a large tumor and is obviously immuniosupressive due to its overall health impact. I would insist on the primary surgery first, then the full recovery and to review the situation in the lungs again. If there are only few lung mets, they can be locally cryoablated with the great outcome, we have contact information for the very experienced doctors to do that.

Hi Olga,
Thank you very much for the reply. I have read a lot on Ivan's journey and was hoping you would weigh in.
Like you mention, I have also read that surgery is usually the way to go with this disease so it has me exceptionally concerned about the suggestion of chemotherapy. Because ASPS is so slow growing, I feel like it will be hard to know if the chemo is even working. Potentially, I see it as a lot of suffering for little return.
Brad and I will press hard at the appointment today. I have no idea the type of experience this medical oncologist has, which is also concerning. The orthopedic oncologist surgeon we met with yesterday has a good deal of experience with sarcomas, but I'm not sure about ASPS specifically. It was a little jarring to hear him say they were going to hold off on surgery for now. If the tumor remains, isn't it basically guaranteed that it will continue to spark the spread of the disease to other places? I see you're based in Vancouver, so are we actually. Do you have any local doctor recommendations?
Thanks,
Naynay

Are you in Vancouver, BC? Have you already had a sarcoma oncologist assigned at the BCCA?

Hi Olga,
I thought I would provide an update since a lot of the initial information I posted is now misleading. (I almost wonder if I should remove the initial info altogether.)
Yes, in Vancouver, BC. We actually met with the medical oncologist at BCCA yesterday - Dr.Simmons. Brad has a really good feeling about her.
It turns out the orthopedic oncologist surgeon misspoke while delivering the news to us on Monday. Once we met with Dr. Simmons, she informed us Brad would not be undergoing chemotherapy, it will be immunotherapy (Keytuda/pembrolizumab). Which makes a ton more sense and was a big relief to us! We also found out that the lung mets are not ALL 1cm in size. Only the biggest one is that size, the other 4 are quite a bit smaller. This would have been nice to know from the get go, but I appreciate Dr. Simmons clarifying with us.
In the next couple weeks, Brad will undergo a PET scan, as well as a brain MRI, prior to beginning treatment. He will also meet with a radiologist re: the primary tumor in his leg. They will discuss the feasibility and side effects of radiation in combination with immunotherapy and Brad is going to broach the subject of removal again. It sounds like that option is not off the table, we just need to speak to the radiologist since they probably won't remove it without shrinking it first. I think they also want to possibly hold off on removal until a few cycles of the therapy are done since it will be a good gauge of whether he's having a response.
I know Ivan was on Keytruda, although he is no longer on it. How long was he on it total? How did he feel during his cycles? Brad has been told to take 3-6 months off work at the start to see how his body adjusts to the drug. We were wondering how likely it is that he will return to work after that - he likes to be busy!
Thanks.

Hi again
It is a good news that he was referred to Dr.Simmons - she is Ivan's current oncologist as well and overall we are satisfied with her. The question of whether to remove the primary or try to see if there is shrinkage is a complicated one. If the surgery to remove it right away seems to be potentially damaging to his leg function, the attempt to use a radiation and K to shrink it justified, as it is very important to try to save the full function.
It is very satisfactory for me and Ivan to hear that ASPS patients are now offered Keytruda. Ivan as denied 2.5 years ago as it was (and is) not approved for ASPS, it was very stressful and we had to fight for it, writing an appeal and a whole article to prove it is a reasonable approach in ASPS. They were impressed and finally approved it for Ivan - at the cost of 12K per a patient per a month I agree they have to look hard into approving such an expensive drug. We even started at our own cost, paid for two infusions and then they approved it, and now we paved the road for you guys. Very satisfactory indeed. Ivan was on it for 2 years.

Hi Olga,
Thank you so much for the work you and Ivan did to get Keytruda here. Obviously we wouldn't have the glimmer of hope we do, without it. I saw pub med is publishing a new study on Friday re: metastatic ASPS and dual immunotherapy. I'll post it to the forum once its available - hopefully it will be full of positive and promising news. In the appointment, Dr. Simmons mentioned being an advocate and it's amazing to hear when its effective.
You'll notice I've changed the the post subject to 'Brad', as well as any mentions in my past posts. When I initially posted, I didn't know how Brad would feel, privacy wise, about putting his journey online. So I used his middle name - Ronald. He's since looked at this amazing community and actually plans to join under his own name so I thought I would update accordingly.
Look forward to connecting further and providing Brad's updates here. Thanks for having a space like this. Since ASPS is so rare, it makes both of us feel like we're not so alone in this journey.

Hi all,
A few updates on Brad's progress. It's been a busy couple weeks.
Brad had his first immunotherapy treatment (Keytruda) at the end of June. It went well. He had no immediate side effects or reactions. A week and a half later, he continues to feel good. After a long wait, his new mountain bike finally arrived and he's been hitting the north shore mountains a lot. His next treatment will come in a couple weeks. We anxiously await to see how next round goes.
A day before his treatment started, he had an appointment with his oncologist to review his PET scan. To preface, we already knew about Brad's primary tumor in his calf, but also 5 lung nodules they suspect are metastasis.The biggest nodule is 1cm, the rest are significantly smaller. On the PET, they saw a high SUV in the primary tumor, which was no surprise. However, the nodules in the lungs had a significantly smaller SUV. The oncologist stated they expected to see similar SUV between the two areas since it's the same cancer. There's even a note in the report that states that some activity they saw in his right lung lobe is more likely to be inflammatory. While we can't know for sure at this point, this could mean the nodules in Brad's lungs might not be metastasis at all. The rest of Brad's body also came back clear. No visible metastasis anywhere else. The brain still remains a question as we're waiting on MRI results to be sure.
The next steps will be to watch the lungs as he works through immunotherapy. If the primary tumor's SUV comes down, but the nodules stay the same, it would be a good indicator the two are unrelated. The oncologist said if that happens, it might be a good idea to re-open the 'surgery sooner than later' topic.
Brad has his first appointment with a radiologist at BCCA, later this week. They will be discussing the pro's/con's of immunotherapy + radiation. We worry about leaving this too long. If there is no metastasis in the lungs, shouldn't removal of the primary tumor be actioned ASAP before there's any chance for metastasis to occur? While we agree with keeping Brad on Keytruda for the time being, we don't want to lose any time if we have a window where the disease is still localized. Any thoughts on that?
Also, has anyone here had experience with immunotherapy + radiation? I know there have been some studies that showed good results, but I'm wondering if there are any real stories people could share here as well?
I'll update again once we receive the brain MRI results and have the initial consult with the radiologist. Until then, my fingers are in a perpetual state of crossed.
Shayna

Shayna, hi, thank you for the update. Unfortunately it is almost never holds true that ASPS is still localized even if found early on a smaller size, as it disseminates very early, and the metastases stay small or even dormant for a while, sometimes for decades (single cell attached somewhere or a clusters of few cells, not growing and therefore not detectable by the scans). Also it is a known fact that the smaller ASPS tumors have smaller SUV (sometimes are not even detected by the PET scan at all), it increases as the blood supply develops - bigger ASPS tumors have very developed vascular system to feed them. But I would support irradiating the primary tumor or resecting it if the oncologist proposes it as the primary ASPS tumors are very immune suppressive and can make the immunotherapy less effective.
Re. possible irradiation - you would need to find out exactly what is the goal and what type of irradiation is proposed. Is the tumor is resectable without any additional intervention? Is the irradiation planned to be used to improve the immune system recognition of the tumor? we used that strategy for Ivan when his soft tissue deeply located metastasis was irradiated using SBRT (high doses/few treatments every other day) starting on the same date as his second K dose was. Making sure the skin is not damaged (affects the immune system performance). What is the size of the primary? not all the technologies are suited for all sizes. Ivan's soft tissue met was about 20 mm (I am not sure but about that)

Hi Olga,
Thanks for weighing in. The oncologist did mention that the size of the smaller nodules could relate to the low SUV. However, she also remarked that she has seen nodules that size with a high SUV as well. It's very hard to know what's going on in the lungs right now since the nodules are all way too small to biopsy.
Reading your description of Ivan's SBRT treatments, it sounds very similar to what they had initially suggested for Brad, prior to switching to the immunotherapy route. I wouldn't be surprised if the irradiation strategy ends up being the same, but we'll have to see what the radiologist says in the upcoming appointment. Brad's primary tumor is approx 7cm x 4cm (length x width) - so substantially bigger than Ivan's. I'm not sure if that makes Brad more of a candidate, or less. Originally, prior to finding the lung nodules, the goal was to shrink the tumor to a point where surgery and recovery were easier. Although we never asked directly, we got the impression that surgery without shrinking the tumor's size wasn't really on the table. (I'll have to write that down and ask so we can be 100% sure though.) Since the resection will take a margin of healthy tissue with it, the overall feeling we got from the surgeon was 'the smaller, the better' when it came to the tumor size. There is a chance Brad could lose a very small amount of mobility in his foot after surgery, so perhaps he had that in mind when he made the recommendation.
Do you have any references for ASPS primary tumors being immune suppressive? Or is that something you encountered during your own journey with Ivan? I've never read/heard that (maybe it's just automatically implied with cancer itself?) and would like to educate myself if you have anything on hand. Hearing that only furthers the feeling that removing the primary should really be back on the table...
As always, thank you for your replies.
Shayna

Shayna, of course the oncologist has seen the smaller lung nodules with a high SUV - just not the ASPS. Basically as I understand, she is lying out the foundation to ask for the primary tumor resection. 7*4 is a pretty large mass.
re. primary tumors being immune suppressive (not ASPS specific, but already noted in ASPS)
http://www.cureasps.org/forum/viewtopic.php?f=22&t=1234
also some reading:
https://pubmed.ncbi.nlm.nih.gov/30439456/

That's our hope. I know Brad would feel much better knowing the primary is out. It will be interesting to see if the first rounds of immunotherapy have any effect on the size. It might take radiation out of the mix altogether. A lot of factors to consider and a lot of routes this could go.
Appreciate the links. Will take a look at them before Brad heads to his next set of appointments.
Shayna

Brad and Shayna,
Stay the course .😊
Phone was loosing power .
Brad should never be left in the lurch ,so to speak .
Asps is a so very very unknown , other than surgical removal is a given .😏
Love ,