Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi Everyone. I’m Louise from the Philippines. 25/F

I was recently diagnosed with ASPS. I had pain in my right ulna (forearm bone) for about a year and was initially misdiagnosed as muscle strain. I didn’t have it checked for months. This year (Jan 2019) when I did an X-ray it showed a lesion in my ulna and did biopsy to confirm ASPS. Afterwards, I had a full-body PET CT scan and it showed multiple nodules in my lungs. My medical oncologist prescribed Pazopanib and I just started taking it today. If you have any tips and advice, it would be much appreciated. Thank you and bless you all!

Hello Louise

I’m truly sorry to hear of your diagnosis , however you found a group with lots of years of knowledge about alveolar soft part sarcoma (ASPS).
How big is the ulna tumor ?
What was the reason for not removing the tumor first , as opposed to leaving it in place ?
There is a wealth of medicines being found to not only stabilize but systemically kill the metastatic tumors that ASPS can produce.
Our family has seen lots and lots of progress( discoveries ) in our journey since August of 2012.
There is lots to be hopeful for .

Hello Debbie,

Hope you are doing well.

The ulna tumor is around 4cm in diameter.
They have not removed the tumor yet because they wanted to see if Pazopanib will have an effect to it.

I'll go back after a month of taking Pazopanib to check if there are any changes.

So far, side effects include fatigue, stomach pain and diarrhea.

Hoping for better days ahead.

Best regards,
Louise

Just an update on my case. After three months on pazopanib, my primary tumor size has gone down to 2cm2 from 5cm2. Based on what you all know/ experieced, is it better to remove the primary tumor or just wait until the Pazopanib stops working? Thanks

Hi Louise,
It is a very good regression so far. If it gets even smaller than that - lets say close to 15-15 mm or even 10-10, then it can be cryoablated instead of surgical resection. Also it is already borderline good size for the radiosurgery, and the bone might fill in by the new tissue replacing the necrotic tissue as the healing goes. You are on the pazopanib for 3 months now and it is reasonable to expect that you have few more moths till the resistance might possible develop, so you could safely start the local treatment planning while staying on the pazopanib for now.
I also wanted to note, that we did not have any cases of the primary ASPS in a bone although it is not rare that we see cases of the metastases to the bones, including arms. My guess is that your primary might be not found yet or it might even has regressed - there are the cases like that when the primary would spontaneously regress but the metastases would remain and grow.
You have not mentioned what influence the pazopanib had on the lung mets.

Louise
The news is wonderful !
I really like Olga’s suggestion as using cryoablation verses surgery . That would most certainly be a-lot less trauma and with a good interventional radiologist , just as effectively successful , in eradicating the tumor .

Have you done anything different besides pazopanib to get such a dramatic reduction ?

Hello everyone!

Just wanted to share the results of my latest scan. So one lung nodule increased in size but my onco said it is still stable based on the RECIST scale, all other mets are stable. Also I have one nodule in the liver that decreased in size based on the scan.

My onco advised to reduce my Pazopanib dosage to 400mg from 800mg because of my persistent diarrhea, appetite and weight loss, and ocassional stomach pain. I’ve been on 400mg for a week now and my arm tumor feels like it is swelling and there is a bit of edema going on my arm. I’ve checked this with my onco and he says I will have to go back to 800mg once my diarrhea is gone.

Still no surgery whatsoever after my biopsy.

Also found out while rummaging through my old documents my primary was in my left thigh which was removed in 2007. I was a child and didn’t know about this until now.

Any thoughts or advice?

Thank you.

Sincerely,
Louise

Hi Louise,
Thank you for the update. What is the size of the metastasis in your arm now (as I now understand your primary was removed in 2007 and the rest of the tumors are all metastatic)? You might want to ask for the radiosurgery if it is relatively small, or for the RFA if it is really small.
Also the lung met that is growing, it could be cryoablated, it is better to deal it when it is relatively small. Despite it is stable under RECIST, it is growing as for ASPS this is a definite growth.
Other than that, Keytruda would be the next systemic drug that you can go to but it might be hard to obtain trough the insurance to pay for it.

Dear Olga,

Thank you for replying. The bone met in my arm measures 25x23x62mm. Is that considered small for the radiosurgery? And what sort of radiosurgery should I bring up to my doctor?
I’m also thinking about cryoablation and will bring that up to my doctors too.
I will also check if Keytruda is available in my country.

I was asked for an X-ray of my arm to check the progress of the tumor and will update here once I get the result tomorrow.

Thank you and have a good day!!

Sincerely,
Louise

Hi Louise, the size does look a bit to large for the radiosurgery or cryo as the bone can break. The best scanning modality for the bone mets is the MRI with the contrast, and I assume they want to start from the X-Ray to outline the preliminary borders first. Let us know what the updates sizes are.

clvbenedicto wrote: ↑Fri Jul 05, 2019 3:59 am Just an update on my case. After three months on pazopanib, my primary tumor size has gone down to 2cm2 from 5cm2. Based on what you all know/ experieced, is it better to remove the primary tumor or just wait until the Pazopanib stops
working? Thanks

Hello Louise
So good to hear from you .

So has your metastatic arm finding stayed the same since 2019 less the length ?
How are the lung tumors being seen?

Truly glad that to hear of looking to treat your arm tumor .
The team will stop the pazopanib treatment prior ?
Love,

D.ap wrote: ↑Sat Jul 18, 2020 2:08 pm

clvbenedicto wrote: ↑Fri Jul 05, 2019 3:59 am Just an update on my case. After three months on pazopanib, my primary tumor size has gone down to 2cm2 from 5cm2. Based on what you all know/ experieced, is it better to remove the primary tumor or just wait until the Pazopanib stops
working? Thanks

Hello Louise
So good to hear from you .

So has your metastatic arm finding stayed the same since 2019 less the length ?
How are the lung tumors being seen?

Truly glad that to hear of looking to treat your arm tumor .
The team will stop the pazopanib treatment prior ?
Love,

I just had an X-ray done yesterday and my orthooncologist said he might also request an MRI as he is considering to remove it. They are just worried about the lung mets as I’d need to stop Pazopanib for around 6 weeks so he will discuss with my oncologist. I will update you once I get results.

I will also suggest cryoablation to the tumors in the lungs as advised in this thread by Olga.

Thank you for responding.

Hi All,

Good day!

Sharing with you the response of my oncologist when I suggested cryoablation and Keytruda:

“Cryoablation may be, an option but I have no experience with it and don't know if it is, available. If you would like to try Keytruda your biggest obstacle is cost around 200T per treatment and usually 18 treatments may be needed. But it will not cure a metastatic lesion and no guarantee it works in ASPS because sarcomas tend to respond poorly to immunotherapy.”

He also advised that I should continue Pazopanib for now until October when I will have my next Chest CT scan for the lung nodules. My ortho said he will check on radiation treatment for my bone tumor while on Pazopanib.

Any thoughts?

Thank you!

Hi Louise, I have a question. Is your pazopanib treatment covered by the insurance or you have to pay for it? I have no idea how the system works were you are treated at. In my son's case they would cover Sunitinib but not Keytruda and we had to flight for it to be covered. Some other ppl managed to get it from the company producer in their countries - you can look for the info https://www.keycare.ph/home.
We have a list of the published articles with the info on its efficacy in ASPS specifically. Your oncologist is only partially correct - it is not very effective in many sarcomas, but thankfully for ASPS patients, it is very active in ASPS. Along with other ICI drugs - the same type of drugs produced by other companies.
see http://www.cureasps.org/forum/viewtopic.php?f=76&t=1482

Olga wrote: ↑Mon Jul 20, 2020 10:55 pm Hi Louise, I have a question. Is your pazopanib treatment covered by the insurance or you have to pay for it? I have no idea how the system works were you are treated at. In my son's case they would cover Sunitinib but not Keytruda and we had to flight for it to be covered. Some other ppl managed to get it from the company producer in their countries - you can look for the info https://www.keycare.ph/home.
We have a list of the published articles with the info on its efficacy in ASPS specifically. Your oncologist is only partially correct - it is not very effective in many sarcomas, but thankfully for ASPS patients, it is very active in ASPS. Along with other ICI drugs - the same type of drugs produced by other companies.
see http://www.cureasps.org/forum/viewtopic.php?f=76&t=1482

Dear Olga,

I currently work for an organisation under the UN and my treatment is fully covered by their medical insurance plan of the organization. So thankfully, they are paying for the Pazopanib. I will have a look on the link you sent me.

Just to let you know the medical advances here in the Philippines is quite slow or late so I’m not expecting the treatments here to be as advanced as other western countries. Sadly.

Thank you!