Cure Alveolar Soft Part Sarcoma International (iCureASPS)

I was diagnosed with ASPS of my tongue in July of this year. I live in Pittsburgh and went to UPMC but also had a second option at Johns Hopkins. We decided to get surgery there with surgeons from the otolaryngology group and I had 80% of my tongue removed. Surgery went well although I have some difficulty speaking, eating and drinking now. I returned to Pittsburgh to receive radiation with Dr. Clump at Shadyside Hospital. I just finished 3 weeks ago and have been getting my energy back. My mouth sores are finally gone but I still have significant pain in my throat which keeps me from eating. I use my feeding tube for most of my nutrition. I felt a lump in my breast a few weeks ago and was sent for a diagnostic mammogram and ultrasound. They felt it looked suspicious so sent me for a biopsy. I just got news it was also ASPS. This lump did not show up in the PET scan I had in July which I am confused by. Does this cancer grow that fast? I am now ordered another PET scan once they can get my insurance company to approve it to determine if it has spread to other areas. I am praying to GOD that it has not. I am 43 and have an adorable 8 year old son who needs me as well as a fantastic, supportive husband. Why couldn’t I just get a regular cancer if I had to get it? WHy some rare sarcoma with a poor prognosis? I am so scared for my life.
I am considering whether I should go back to Hopkins and meet with their sarcoma team or try Sloan Kettering sarcoma team? I just want to live and know that this sarcoma will eventually take my life but I am hoping for many more years before that happens. If anyone has any suggestions I am open to hearing them. I have been reading some of your stories and they are hopeful but also fill me with a heavy heart because of the struggles and fear you all face with this disease.
Courtney

Hello Courtney
My name is Debbie and our son was dx’d( diagnosed ) in 2012 and at the time had a 4 year old daughter .
He’s now 7 years from being dx’d and his daughter is 11!

I can so relate to your anxious feeling of having been dx’d with ASPS but be assured ,awareness and much understanding of our rare sarcoma ,has grown exponentially since our journey began 7 years ago .

You are correct that our indolent , typically slow growing tumors don’t always “light “ up like faster growing dividing sarcomas. So yes they can be missed by a PET.
Ct scans AND MRIs are best used as well as bone scans .
X-rays as well, for some initial diagnosis. The useage of machines , technicians and with the use of the machine contrast , helps to “light up “ our indolent tumors to find them.

How large was your tongue tumor and how big is the chest tumor ?
Are you in contact with a sarcoma oncologist ?

The first days are sure tough on everyone after being dx’d but be assured , there is hope . Knowledge is the key as it is rare and most doctors have never seen a case of ASPS so learn all you can and find someone to help you as well. As you need to be able to take care of you.😊
Hope this helps.

Courtney, I just want to add to what Deb said - there are new treatments that greatly change the ASPS outlook. People with the very extensive metastatic ASPS are now surviving for much longer after treated with immunotherapy and additional treatment for some metastases that became resistant to it. All the studies on ASPS are done before of that and might be not accurate in the prognosis.
As per other common cancers being easier than ASPS, it is not true. When metastatic, they are rarely curable with the chemotherapy only providing few months or a year of delay. The advantage of ASPS being slow growing, gives us - first the hope to survive long enough to the cure to be found, second - carefully evaluate the options available now, without the rush, with the proper consultation, and often manage the single metastases on the case by case basis. And have the normal life between the metastatic events.

Olga and Debbie,
Thank you for responding to me. It made me feel better reading your messages. My tongue mass started out 2.8 x1.9 x2.6 and quickly grew to 4.0x 3.4 x 2.8 they claimed likely bc of the needle biopsy and surgical biopsy. My breast mass is 1.4 cm. I was ordered a pet scan but asked the radiology oncologist to consider brain mri and chest ct instead since it seems PET CT’s often miss things. I am waiting to hear back from him. I have an appointment with Dr. Burgess at UPMC on the 21st of Nov as she is a soft tissue sarcoma specialist and has done trials on ASPS.
I am also going to get additional opinions at Sloan Kettering and Johns Hopkins. I am getting most of my nutrition through my feeding tube right now as it hurts to swallow but trying some smoothies. I have been having difficulty gaining weight as I have lost 20 lbs since July.
Prayers and love to all of you.
Courtney

Hi Courtney,

Sometimes for the brain scan, it is better to do CT than PET since my oncologist told me that ASPS slow growth could be undetected only by PET.
Me too got ASPS while raising 1, 3, and 5 y/old kids, but on the bright side, it's better than another fast-spreading cancer right?

Best regards,
Daniel D.

Daniel, You’re right and I need to look on the bright side of things (like a slow moving cancer) instead of focusing on how this is going to kill me someday. How wonderful that you have 1, 3, and 5 year children but how hard it must be going through this while raising them. I do know that being diagnosed has already changed the way I look at life and I spend much more time playing games and making slime with my sons instead of just watching tv with him or letting him play video games.
They did change the scan to a CT and PET and ordered a brain MRI which hopefully my insurance company will agree to as they denied it 6 mos ago.
Thanks for reaching out.
Courtney

Just got results from brain MRI and pet/ct scan. Brain is clear but spot in bone of head. Also spots showing up in both legs, spine, pelvis, both humerus and clavical. I can’t believe it. I had scans in July and none of this showed. I am so scared. I have a meeting with a sarcoma specialist tomorrow at UPMC in Pittsburgh and other appts scheduled at Johns Hopkins and Sloan Kettering. I am in compete shock and disbelief. I don’t want to lose this battle with ASPS anytime soon.

Courtney,
It is a very hard news to get, hang in there.
Did you only have the PET scan in the summer - not the PET?
I would definitely verify the PET findings with the MRI in al least some locations. Most of these newly found mets are in the bone, correct? might be some rare bone condition. The other thing to consider is that the mets might have been there but were smaller/less active or even dormant and the massive surgery and radiation you had weakened your immune system and increased the progrowth factors during the healing process so they grew by few mm and became seen now.
In my opinion you need either immunotherapy off label treatment (Keytruda or Opdivo are often avail. off label if your oncologist makes the case for the producers/or the insurance refuses to cover) - see the write up on Keytruda here:
viewtopic.php?f=76&t=1482
or you need a clinical trial with the immunotherapy drug. They should be something avail. locally. There is probably nothing different that would be offered at the MSK I am afraid, but it is good to have the second opinion.
The advantage of having the treatment off label is that you can add the radiation to the single metastasis in order to try to potentiate the immune recognition, so called abscopal effect. The clinical trial might not allow that and it has to be done at the same time with the drug - the same day or 1-2 days before or after

I did have a PET/CT scan in the summer which did not show any of these Mets. It was done in July. I met with Dr. Burgess at UPMC in Pittsburgh and she feels I would be a good candidate for her clinical trial where Atezolizumab is administered. I am currently in Baltimore and have an appointment with a sarcoma specialist here tomorrow to get a second opinion. I am going to also alter my diet and eat more healthy. I don’t eat terribly now but have read a lot of articles about how cutting out refined sugars, dairy, carbs, ect. may help cancer patients. I am willing to try anything at this point to live as long as possible...

Ckwelk wrote: ↑Sun Nov 24, 2019 2:48 pm I did have a PET/CT scan in the summer which did not show any of these Mets. It was done in July. I met with Dr. Burgess at UPMC in Pittsburgh and she feels I would be a good candidate for her clinical trial where Atezolizumab is administered. I am currently in Baltimore and have an appointment with a sarcoma specialist here tomorrow to get a second opinion. I am going to also alter my diet and eat more healthy. I don’t eat terribly now but have read a lot of articles about how cutting out refined sugars, dairy, carbs, ect. may help cancer patients. I am willing to try anything at this point to live as long as possible...

Hi Courtney ,
A second option is never a bad idea , however I believe Dr. Burgess has seen Asps , has another patient ASPS patient in your area? Atezolizumab has shown great success with lots of ASPS folks .

On another note of sugar etc diet contributing to cancer. Be aware that ASPS has been shown to use lactate as a vehicle to thrive ,as this incredibly vascular tumored sarcoma lacks oxygen .

With that being said , it most time adjust to a low oxygen
hypoxic deregulated environment and goes from a glucose metabolized to a lactate metabolized transition. Your protein intake along with your carb decrease , if not dealt with a balanced diet could support tumor growth .
It can create lactate for ASPS to thrive .

Certainly talk to your doctors.😊

https://www.atkins.com/how-it-works/lib ... ise-in-men

Ps I like this utube presentation by Dr.Kevin Jones .
He and others of the Utah institute , help to discover the lactate transport that ASPS can utilize in its progression .


https://m.youtube.com/watch?v=fVKiQnOCsU0

D.ap wrote: ↑Sun Nov 24, 2019 5:43 pm Ps I like this utube presentation by Dr.Kevin Jones .
He and others of the Utah institute , help to discover the lactate transport that ASPS can utilize in its progression .


https://m.youtube.com/watch?v=fVKiQnOCsU0

This is kind of weird. ASPS primary may grow in high lactase areas. But primary isn't our problem, is it?

ASPS mets occur in brain, lungs, bones, abdomen. Are all these high lactase areas? Doubt it.

So does diet and what we put into our bodies really matter after already haven been diagnosed with metastatic ASPS?

It is the question that we do not know the answer to. But right now since you are considering to start the immune-checkpoint inhibitor trial, you should be focused on the diet and other factors that are found to improve your chances to respond to it, not on the general cancer-food relationships as you might be in a different unique situation not to follow the general advice.
ICI - immune-checkpoint inhibitors are the unique class of drugs, that are completely different from the traditional cytotoxic checmotherapy drugs that were used before or TKI drugs that are newer.
In general, all immune-checkpoint inhibitors are blocking some immune system factors that cancers use to evade the immune system surveillance and avoid being attacked and destroyed by the immune system hunters - i.e. the breaks are removed. You should understand that there are the immune cells that cooperates with the tumors to prevent the other cells to destroy the tumor cells. There are numerous immune checkpoint inhibitor drugs that are currently approved or in the clinical trials. We have cases when out patients respond well to most of them - we have the parts of the board dedicated to ICI drugs (keyrtuda, Opdivo, atezolizumab etc)
Few factors are found to improve the immune system attack on the cancers after the breaks were removed - a diet that improves the microbiome (symbiotic bacteria that lives in the lower part of the digestive system), avoiding antibiotics that damages the microbiome, avoiding the steroids that reduce the number of the lymphocytes, building the muscle mass (that you need the proteins for and also exercise), having a diet sufficient in Zn to increase the lymphocytes number, reducing the overall inflammation in your body by having better quality sleep - dark and quiet bedrooms are preferable for the melatonin production, more exercise, less stress, more positive emotions.