Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi Everyone,

My name is Ethan and I am a 42 year old married, father of four, diagnosed with ASPS in Oct 2020.

My story is strange...at least to me. I was deployed in Ashgabat, Turkmenistan with the U.S. State Department at the American Embassy when I started having pain in my leg (Aug 2020). The doctor who worked with me arranged for me to have an Ultrasound and then an MRI at a local hospital and we discovered a mass. When the doctor performing the ultrasound told me about the mass, I saw my life flash before me. Hopes, regrets, all of it. While I'm normally a very calm person that has lived through terrorist attacks and a near plane crash, I almost panicked with the feeling of helplessness. I had no idea how cancer (I didn't yet know what type) could happen to me.

Shortly thereafter I returned to the US and started the process of finding first a primary care physician and then a surgeon... during a pandemic... no easy task when you have just returned from traveling abroad. No one wanted to see me for several weeks, but I finally found a Primary care physician, who referred me to an orthopedic surgeon. After two biopsies, we found that the mass in my left leg was ASPS. At that time it measured 12cm x 6cm x 4 cm. When my surgeon confirm it was a malignant tumor (I didn't know what ASPS was yet) I again saw my life flash before me.

I was then referred to an oncologist who immediately ordered a PET CT. This unfortunately revealed about 40 spots in my lungs (both sides), several nearly 2cm in size. I guess I was supposed to have seen the written report from the PET CT earlier, but I hadn't...so when my oncologist mentioned that the cancer has spread to my lungs as a side note, I almost didn't catch it. I had to ask him to clarify what he had said...and then it sounded like a death sentence. My only knowledge of people with lung cancer was dated...and I didn't realize that lung cancer was different than ASPS that had spread to my lungs. Either way, right then I felt my world collapse before me. This was supposed to be a "regular" oncologist visit...not one where you find out that your life has forever changed.

I remember immediately calling my wife with the news, and being afraid to tell her. How do you say that over the phone? I did, and it sucked, but she got me through it.

It took again two biopsies to confirm that the spots in my lungs were also ASPS (Dec 2020)... despite hoping in vain that it was something else.

Just a couple of weeks earlier, near the first of December, my oncologist started me on Pembrolizumab (Keytruda).

I did approximately 6 cycles of 3 weeks before re-imaging my lungs and my leg. Neither had shown signs of growth...nor had they shrunk. Either way, my oncologist suggested we should try to remove as much of the primary tumor in my leg as possible...so I went back to my orthopedic surgeon from before. He refered me to a vascular surgeon, who referred me to a doctor with interventional radiology. Long story short, I had to have two surgeries done, on two consecutive days. The first surgery was to block two of the three major veins and two of the three major arteries that passed through the tumor and feed my foot. The next surgery was to remove as much of the tumor as possible without risking the limb. These happened in late May 2021.

Both surgeries went ok, but the orthopedic surgeon said he was only able to get about 40% of the primary tumor due to the location of the remaining vein and artery. This was disappointing. We had originally hoped for more like 80-90% removal.

After a month of healing, my oncologist refered me to a doctor that specializes in radiation treatment. This doctor immediately ordered a new CT of my chest and a MRI on my leg. The chest CT showed that the cancer in my lungs still seems to be stable (not growing or shrinking). The MRI unfortunately showed that way less than 40% of the tumor was removed...more like 10%. That was a lot of pain to go through for 10%. Nearly two months has passed since the surgery and I still can barely walk. I sit in the parking lot while my family goes shopping, because I can't be on my feet for very long. I drive my family to the beach, but I can't join because I can't walk from the car to the shore. Too much lost for only 10%. I wish I had not done the surgery. What is done is done.

Anyway, back to the story. My radiation doctor was happy with the results and jointly, we decide to pursue an aggressive treatment plan to attack the primary tumor in my leg. I will have 35 sessions, totaling 70Gy of radiation. He said that this should be enough radiation to stop the primary tumor from ever growing or spreading again. There are risks of significant side effects, but the benefits outweighed the risks. I will start radiation treatment next week using an MRI guided radiation system called Viewray. Fingers crossed.

So far I have not yet started Axitinib. My oncologist has asked me to hold off until after radiation. Then I will take it in conjunction with Pembrolizumab. I am about to have my 12th round of Pembrolizumab.

I have only in the last couple of months started to research what ASPS is and what it isn't. I have reached out to the National Institutes of Health and provided them with my medical records as part of an informal study on rare tumors that they are working on. I have asked they keep me informed of any experimental treatments.

From what I have read, this is something you live with and there are no cures. I am not willing to accept that. So now I spend my nights scouring the internet for information on new treatments and research on ASPS.

Sorry for the long post. It has taken me a while to compile my thoughts.

Take care everyone,

Ethan

Hi Ethan ,

Thank you for your service !

I’m sure sorry to hear of your diagnosis , being dx’d , of ASPS. It really was a hard pill for our family to swallow back almost 9 years when our son Josh was dx’d .😕

So my understanding is you’ve had about 2cms of primary removed and have been on Keytruda since December of 2020 ? How much muscle mass was removed over and beyond the primary ?
How many scans of your lungs have been performed while on Keytruda ?

How’s your surgery healing?
In being 8 weeks out of surgery with limited mobility ,I suspect there was extensive work around of muscles etc?
Either way be easy on yourself and certainly hook up with maybe a physical therapist ?

Ie The radiation treatment :
What are the cons to the procedure ?

Using radiation with close to a Keytruda infusion ,can possible create synergistic antigen treatment from that primary and or other tumors , for your existing tumors to be recognized by your immune system , however in my opinion the 70grays is going to be quite hard on your body anatomical and physically . It could be that treating your lung tumors could be as advantageous and have the same affect without the toxicity that the 70 grays might created ?
Asps is highly radio resistant and requires more than your average non radio resistant sarcomas so the injury could be significant to the area which might be rather large as it needs to be with margins of the existing tumor , I think .

When we started our journey in 2012, our son Josh was 32.
He will turn 41 this year.😊

The treatment options have changed dramatically and exponentially since we joined this forum .From chemos to immune check point inhibitors and ablations, there is truly lots to be utilized to defeat these metastatic tumors! Scans are our most sacred tools to plan and eliminate them at their smallest size if and when other targeted meds are not working .

Keep the faith !

Sincerely ,

Hi Ethan
welcome to our forum.
It is a hard blow to get when Dx with ASPS for sure. My son Ivan was Dx when he was 17 years old, he is now 35 yo, we live in Canada. His primary was in his arm and he had two consecutive surgeries to remove the primary tumor. Multiple lung metastases during first 5 years, followed by the brain, heart, adrenal glands and pancreas. Multiple surgeries (4 of them in Germany) and cryoablations (most of them in US), and when there were no options left when the heart mets appeared, Keytruda came to market and it was a great success story for my son. Now he is two years off the treatment with no progression and most metastases greatly regressed, but with the significant side effect - his adrenals totally gone perhaps from the autoimmune attack.
During all the treatments, pain of surgeries and stress of receiving news about new mets pop up or the old ones growing, he was able to live a very good life. Lots of sport, university, work, nature. He plays competitive volleyball, but it took very conscious effort to gain the full functionality after the loss of 2 muscles in his arm and to fully recover the lung function after the 5 lung surgeries. Even our travel for treatments and surgeries were fun and sightseeing in some sense.
We learned to be very proactive and careful with the treatments and doctors selection. There are significant limitations in Canada as for what doctors or treatments you can choose, but it did not stop us from getting second, third, forth or even fifth opinions here and abroad in cases when the problem was complicated. Ivan lung surgeon from Germany Dr.Rolle told to us in the very beginning that cancer treatment is a chess game, plan your next move very careful looking well ahead otherwise it will outplay you.
Now about your case. You have probably had it for a long, long time based on the size of the primary. The primary location is challenging, and it is not rare when the full removal without the loss of functionality is not possible. We had the member here who said that eventual amputation of the leg with the prosthetic replacement was the best outcome after few years of the unsuccessful surgeries. On the other hand, you still have the chance your immune system recognizes the tumor. I doubt that the surgeon had actually removed only 10% hoping to remove 40%, the MRI done on an inflamed wound is not that reliable. That primary surgery should be done by the sarcoma surgeon not by the general orthopedic surgeon, there would have been better pre-estimate done by the very experienced sarcoma surgeon re. possible surgical outcome.
About the planned radiation treatment. I am not a doctor and none of what we say here should replace the medical advice, but as it is planned now, it completely misses the point of the immunotherapy treatment ongoing, addition of Axitinib planned and lung mets being the main danger for your life, not the primary.
The proposed radiation treatment will of course kill the primary, reliably. It will also create the large rotten mass and severely damage your immune system for a long time, and your chances to get the benefit from the immune checkpoint inhibitor treatment. What it does in general, it releases the breaks that sarcoma applies to your immune system by false signaling. To get a benefit you need to have the well functioning immune system, after the radiation treatment like this it is going to be very shocked and down, with lots of damage in your leg.
When Ivan started immunotherapy, we were extremely careful not to damage his immune system, but to support it instead. He could have an incisional biopsy for a molecular study in the beginning of the treatment and he wanted it so badly, to gain more information about the make up of the tumor. But after very careful consideration we decided against it. Radiation treatments can be successfully combined with the ICI treatments but they are done differently, with the pinpoint targeted SBRT treatment of one small metastasis, hoping to improve the immune system recognition attracting its attention to the tumors as it dies.
Your case is definitely complicated after the incomplete surgery. Sometimes the tumor can be cryoablated so it gets smaller before the surgery.
What is the exact location of the tumor?

D.ap wrote: ↑Thu Jul 15, 2021 8:20 am Hi Ethan ,

Thank you for your service !

I’m sure sorry to hear of your diagnosis , being dx’d , of ASPS. It really was a hard pill for our family to swallow back almost 9 years when our son Josh was dx’d .😕

So my understanding is you’ve had about 2cms of primary removed and have been on Keytruda since December of 2020 ? How much muscle mass was removed over and beyond the primary ?
How many scans of your lungs have been performed while on Keytruda ?

How’s your surgery healing?
In being 8 weeks out of surgery with limited mobility ,I suspect there was extensive work around of muscles etc?
Either way be easy on yourself and certainly hook up with maybe a physical therapist ?

Ie The radiation treatment :
What are the cons to the procedure ?

Using radiation with close to a Keytruda infusion ,can possible create synergistic antigen treatment from that primary and or other tumors , for your existing tumors to be recognized by your immune system , however in my opinion the 70grays is going to be quite hard on your body anatomical and physically . It could be that treating your lung tumors could be as advantageous and have the same affect without the toxicity that the 70 grays might created ?
Asps is highly radio resistant and requires more than your average non radio resistant sarcomas so the injury could be significant to the area which might be rather large as it needs to be with margins of the existing tumor , I think .

When we started our journey in 2012, our son Josh was 32.
He will turn 41 this year.😊

The treatment options have changed dramatically and exponentially since we joined this forum .From chemos to immune check point inhibitors and ablations, there is truly lots to be utilized to defeat these metastatic tumors! Scans are our most sacred tools to plan and eliminate them at their smallest size if and when other targeted meds are not working .

Keep the faith !

Sincerely ,

Hi,

From what I can tell, I have had about 1 to 2 cm of the tumor removed, at most. That still leaves a sizable mass. In the end, the surgery was more of a de-bulking exercise tan anything else...one I wish I had not done. Because of this, the margin remov d outside of the tumor area don't really apply...the tumor is basically still there, largely intact. :(

Yes I have been taking Keytruda every three weeks since early December. So far I had a scan of my lungs in early March and another earlier this month. Both generally showed that the mets were stable ( no real growth, but no sizable shrinkage). You are right about the extent of muscle damage from the surgery, but most of my issues now revolve around nerve damage and my inability to bend my foot (I can, but not enough to walk without a serious hobble).

So, regarding cons of the radiation. The doctor that specializes in radiation (he seemed very competent and knew a good bit about sarcomas...not sure how much he knew about ASPS), stated that the con with an aggressive approach is that long-term, I may have limited flexibility nearly 2 years after the procedure. The other option was a conservative approach. With this one he said that it was just a matter of time before the tumor regained it's ability to grow/spread. He said that this could be between 6mo to 2 years, but it would happen. With those options, I selected the aggressive treatment.

I appreciate what you and Olga mentioned on the other downsides to radiation and how it may be detrimental to treating the mets in my lungs because of the radiation affecting my immune system. I have written to my oncologist and asked this very question.

One thing that I need to mention is that I am in Orlando. The hospital here is ok, but they do not specialize in sarcomas and I am only aware of one other ASPS patient. I was told that my orthopedic surgeon does specialize in tumors and he is supposed to be one of the best in Florida. After talking with him on various appointments, I have no doubt that he took out as much as could be removed while saving the limb. I just have this nagging feeling that in the end, I would have been better off sacrificing the limb to get rid of the primary tumor. I'm just not yet mentally prepared to do that.

Maybe the reaction to Keytruda is normal, but I had hoped for a more substantial change than what the scans demonstrated. I've just had my 12th round, and no growth (or at least no measurable growth) is all I have from it. Has it been this slow with everyone else? Should I be doing anything else to increase my odds of a positive reaction to Keytruda?

Take care,

Ethan

Olga wrote: ↑Thu Jul 15, 2021 11:02 am Hi Ethan
welcome to our forum.
It is a hard blow to get when Dx with ASPS for sure. My son Ivan was Dx when he was 17 years old, he is now 35 yo, we live in Canada. His primary was in his arm and he had two consecutive surgeries to remove the primary tumor. Multiple lung metastases during first 5 years, followed by the brain, heart, adrenal glands and pancreas. Multiple surgeries (4 of them in Germany) and cryoablations (most of them in US), and when there were no options left when the heart mets appeared, Keytruda came to market and it was a great success story for my son. Now he is two years off the treatment with no progression and most metastases greatly regressed, but with the significant side effect - his adrenals totally gone perhaps from the autoimmune attack.
During all the treatments, pain of surgeries and stress of receiving news about new mets pop up or the old ones growing, he was able to live a very good life. Lots of sport, university, work, nature. He plays competitive volleyball, but it took very conscious effort to gain the full functionality after the loss of 2 muscles in his arm and to fully recover the lung function after the 5 lung surgeries. Even our travel for treatments and surgeries were fun and sightseeing in some sense.
We learned to be very proactive and careful with the treatments and doctors selection. There are significant limitations in Canada as for what doctors or treatments you can choose, but it did not stop us from getting second, third, forth or even fifth opinions here and abroad in cases when the problem was complicated. Ivan lung surgeon from Germany Dr.Rolle told to us in the very beginning that cancer treatment is a chess game, plan your next move very careful looking well ahead otherwise it will outplay you.
Now about your case. You have probably had it for a long, long time based on the size of the primary. The primary location is challenging, and it is not rare when the full removal without the loss of functionality is not possible. We had the member here who said that eventual amputation of the leg with the prosthetic replacement was the best outcome after few years of the unsuccessful surgeries. On the other hand, you still have the chance your immune system recognizes the tumor. I doubt that the surgeon had actually removed only 10% hoping to remove 40%, the MRI done on an inflamed wound is not that reliable. That primary surgery should be done by the sarcoma surgeon not by the general orthopedic surgeon, there would have been better pre-estimate done by the very experienced sarcoma surgeon re. possible surgical outcome.
About the planned radiation treatment. I am not a doctor and none of what we say here should replace the medical advice, but as it is planned now, it completely misses the point of the immunotherapy treatment ongoing, addition of Axitinib planned and lung mets being the main danger for your life, not the primary.
The proposed radiation treatment will of course kill the primary, reliably. It will also create the large rotten mass and severely damage your immune system for a long time, and your chances to get the benefit from the immune checkpoint inhibitor treatment. What it does in general, it releases the breaks that sarcoma applies to your immune system by false signaling. To get a benefit you need to have the well functioning immune system, after the radiation treatment like this it is going to be very shocked and down, with lots of damage in your leg.
When Ivan started immunotherapy, we were extremely careful not to damage his immune system, but to support it instead. He could have an incisional biopsy for a molecular study in the beginning of the treatment and he wanted it so badly, to gain more information about the make up of the tumor. But after very careful consideration we decided against it. Radiation treatments can be successfully combined with the ICI treatments but they are done differently, with the pinpoint targeted SBRT treatment of one small metastasis, hoping to improve the immune system recognition attracting its attention to the tumors as it dies.
Your case is definitely complicated after the incomplete surgery. Sometimes the tumor can be cryoablated so it gets smaller before the surgery.
What is the exact location of the tumor?

Hi Olga,

Thank you very much for the caution on radiation treatment. I have reached out to my oncologist and asked him this exact question. I hope to hear back from him soon. I'll let everyone know what he says.

As I mentioned to Debbie, I'm I'm Orlando and the hospital here doesn't specialize in Sarcomas, much less ASPS. That being said, my oncologist has mostly followed treatment that matches what I have read here...with the exception of the radiation treatment. I have not gotten a second opinion on anything. Partially because I feel like I can trust my medical team here to do the best they can, and partially because I am not sure what I would get a second opinion on. My oncologist mentioned that I could go to either Tampa or Jacksonville FL where they have better cancer programs, but I don't see that being a real option for regular treatment...as it is, I'm probably seeing one doctor or another, getting one scan or anything or receiving treatment at least twice a week. Doing that elsewhere would be a logistical nightmare.

My family and I have considered selling our house and moving up to Washington DC...and we probably will in the next year or so...I suspect that DC has a greater capabilities for treating cancer, but that's just a guess.

As I mentioned to Debbie, I'm a little disappointed that Keytruda has not had a more substantial effect on the mets in my lungs or the primary tumor in my left calf. Do you know if there is anything else I can do to maximize my chances of Keytruda working? Dietary changes, etc? I always thought of myself as relatively healthy. While I was not the "going to the gym" type, I liked to hike, do house projects, and play with the kids. I stayed pretty active.

Any suggestions would be much appreciated. Also, thanks to you and Debbie for going easy on me with the lingo. I am extremely new to all of this and often don't understand much of what is posted in various blog posts because I don't know the lingo yet. I will learn...I just today learned what "mets" meant.... before it was just a baseball team.

Take care,

Ethan

Ethan,
ASPS disseminate early in course if its disease, and the primary has already disseminated micromets via bloodstream (floaters) that first trapped in the lungs and grow in earlier there. It is probably not an ugrent task to remove the primary right away or irradiate it as soon as possible, although I usually advocate the primary to be resected as early as possible but in your case more prep work needed to be done. De-bulking is never a good plan with sarcomas and can be only attempted if this is the only way possible to survive. The radiation oncologist is most probably trying to reduce the damage done by the locally disseminated tumor by the incomplete surgery. Get a second opinion on the redo surgery at the more advanced place from the sarcoma surgeon. You can keep the overall treatment incl chemotherapy and scans at your local hospital and get the specific problems solving outsourced to their specialized counterparts within the network, it is often done that way. There are advanced ways to deal with the difficult to resect primaries and may be someone can find a way to resect it with no irradiation.
Can you consult in Moffitt Cancer Center re. possible isolated limb perfusion to try to reduce the size of the primary to enable its resection? there are e-mails to contact
https://pubmed.ncbi.nlm.nih.gov/32162353/
https://pubmed.ncbi.nlm.nih.gov/29019175/
https://pubmed.ncbi.nlm.nih.gov/28214556/
also Department of Sarcoma, Moffitt Cancer Center, Tampa is another place where you could get the evaluation re. possible redo resection with the limb salvage.
The problem with suboptimal primary surgery is very common in sarcoma patients, my son had the initial surgery incorrectly performed by the local surgeon without the proper margins, followed by the redo surgery that was excessively radical - they removed 2 large muscles from his arm. As it was said later by the researchers, with ASPS the minimal anatomically defined margins are usually enough. But the incorrect initial surgery made it impossible to see where the initial tumor margins were...

Hi Ethan,
First of all, I wanted to say I'm sorry about your diagnosis. I feel for your post very much, probably because I see a lot of parallels between your case and my partner's. He was diagnosed with ASPS in June 2020, primary mass in his leg, and a few suspected mets in his lungs. The mets are too small to biopsy though, so it's speculation whether they're related or not. It has been a rollercoaster of emotions and information, especially during a time where the world has very few distractions to offer.
I am glad to hear you are on Keytruda. While the mets have not changed in size, that stability can sometimes be considered a response within itself. My partner recently went through surgery to remove his primary. Prior to the procedure, he was on Keytruda for approx 7 cycles and then 5 weeks of radiaiton to the tumor site. Luckily, surgery was a success and they managed to get the entire tumor, negative margins. I mention this because the tumor never changed size during my partner's treatment. However, when post surgery analysis was done, the tumor was revealed to be 80% dead. We believe this is because of the Keytruda, radiation, or perhaps a mix of both. It's hard to know. I recall my partner getting a scan during his treatment (I think it was a PET or CT?) and one of the radiologists remarked that the tumor seemed less vascular/active than past scans. Perhaps you could ask your doctors about this of your primary?
Anyway, I found that very interesting, and I thought you might too. Perhaps it will even bring you some comfort. While we always assumed the size of the tumor is what mattered, it was really the activity going on inside it, that had more value.

Hi Ethan,

I got a few questions.

1) Are you able to get exercise despite your leg? Something like cycling on a stationary bike to start. You need to actively work on rehabbing your leg, not just wait for it to heal. Progressive overload.

2) What is your diet like? You need to eat lots of veggies (and fruits, especially berries) as it changes your microbiome and affects PD-1 effectiveness many-fold.

Ivan

Hello Ethan, first I encourage you to answer Ivan's questions regarding diet and mobility. However, I wanted to welcome you since my son has been battling ASPS since 2007 in florida( Sanford). His first tumor was removed by orthopedic oncologist (Parker Gibbs at UF Shands). We have had 2 brain, 1 lung, and 1 oral surgery at Advent Health in Orlando. We have been traveling for systemic treatment to Miami since 2009. Have you had a brain mri too?
This site has helped us play this "chess game" or "wack a mole game" year after year. Let me know if we can be of any help. Best Wishes