Prairie from Nebraska - Dx 2015

[Vega343]

Hi there -
I think her biggest lung let is >4cm.
Her breathing seems uneffected, not sure how this is possible, maybe she doesn't exert herself.

Brain mets still there, unchanged.

I did see the Atezolizumab trial is open, we will see.

Any new amazing meds people have used lately that are effective? Seems like there are plenty off label that might be a good fit.

Thanks - MJ

[Olga]

MJ - we have few cases of the amazing responses to PD-1/PD-L1 blocking drugs - which atezolizumab is one of. Most responding people were/are on the Keytruda (another drugs from the same group) plus axitinib clinical trial in Miami. Most trials do no accept people with the brain mets. How many of the brain mets does she have, are they potentially resectable?

[arojussi]

Atezolisumab trial sounds great. Indeed Pd1-tarceting drugs work well in asps. Nobody knows why. Usually these drugs work well with cancers with high mutational burden, but for some reason they work well in kidney cancer and asps. Most studies I seen about pd1-inhibitors in sarcoma mention asps, because asps patients are most succefull ones. Currently there is no evidence that one pd1-inhibitor would be any better than other.

Most trials usually want brain mets to be stable. So if brain mets are small destroying them with gamma knife could be neccessery. If mets are big then surgery might be neccessery. In general side-effects from immunotherapy are easier than sideeffects from chemo or tki, but dangerous immnological sideeffects are possible.

[D.ap]

Morning MJ ,

Josh had an over 6cm tumor in his left lung , when he began Opdivo in 2016 and a 9mm skull tumor .
(He was 36 of course , currently 37 1/2. 32 at dx’d )
The 9mm has resolved ( believe after 6-9 months on Opdivo) and the 6cm has reduced to under 3cm.

Linking you : )

http://www.cureasps.org/forum/viewtopic ... =15#p10261

He was experiencing an airway blockage( ie coughing) prior to starting Opdivo , but no longer has that
tendency .

I hope this helps .
Keep the faith .

Love
Debbie

[Vega343]

Hi All -
Visit to NIH late June shown no growth of the lung tumors, or the pelvic tumor, and the brain lesions are very small (almost undetectable). So we remain on Cediranib study for at least 3 more months - next visit mid September. Biggest issue is her keeping her weight, managing some pain, and low energy levels.

Olga and Jussi - not sure if they would treat the brain met's with gamma knife or not.

Still looking at options for when her lung tumor growth surpasses the 20%,
might do the Atezolizumab trial at NIH (which I hear is going well, and they opened it to more people)
watch and wait and keep watching (docs say some kids tumors don't grow for years after they get off Cedirinib)
Optivo sounds like a hit for Josh!!! Hooray!! This is outstanding to read!

Take care all -
MJ

[D.ap]

Hello MJ

Truly good to hear from u!: )
Has Prairie gotten bone scans ?
We are very happy with Joshua’s results.

[D.ap]

MJ
Wrote a larger response above to post and I timed out.: /
Short and long Josh was down to mid 90 lbs
We incorporated butter, oils and protein powders,boost, avocados , boiled eggs , nuts( not peanuts ) and nut butters.
After the reduction of his 6cm lung met the weight started to come back on. Prior 6cm size caused profound cough / gagging . That began to fade! And is gone today .
: ) His current weight is 139, too.

Where is Prairie’s over 4cm lung met?
Would it benefit her to have that addressed after she ends with the trial ?
Also there is an excellent article Olga posted on lowering the thyroid thru diet, as lowering the levels of the thyriod itself, brings higher thyroid stimulating hormones(TSH) being produced . That production of higher TSH has been clinically proven to show better results with TKI , results.

http://www.cureasps.org/forum/viewtopic ... 497#p11592

[Vega343]

Hi old friends. Debbie, appreciate your success w weight gain!
We had scans and NIH visit this week. Showed slight progression, but long story short, we are now over the 20% progression threshold for the cedirinib study, and need to be done with that treatment. (Removed from study after 33 months).
We are planning a month off treatment, and then starting Atezolizumab study with NIH. Sounds like they have some very good results with adults over the last 2 years, including at least one case of 100% reggesion, so ready to begin. Any feedback on this drug is welcome.
The logistics will be tough, back in DC from Nebraska every 3 weeks, but we do what we need to do!
I am looking forward to P feeling better as the cedirinib moves out of her system, and hopeful for next treatment.
Comments /questions welcome.

[Olga]

Sorry to hear re. 20% growth. The feedback re. Atezolizumab is good - it is one of PD1/PD-L1 inhibitors (also called immune checkpoint inhibitors or ICI) that are fairly active in ASPS and we have our patients responding very well across 3 most known ones - Keytruda, Opdiovo and Tecentriq (atezolizumab).Tom is on it, look for his updates, he is having a very good response so far, 18 months into the clinical trial.
They generally remove the breaks from the immune system or body have (and cancers use to escape the immune surveillance). The response depends on the immune system so do not rush in, let her recover from the cediranib side effects. Try not to use antibiotics before/during the Atezolizumab treatment. Make sure to study and understand drug specific toxicities as locally there are going to be very limited understanding of what the symptoms mean. Let us know when she is started already and we will look for the updated side effects info, they are very unusual.

[arojussi]

Check point inhibitors indeed work well in asps and responses are often durable. Responses can be mixed and it can be really difficult to say if immunotherapy is working based on scans alone. Pseudoprogression is relatively common in asps. There are few ways to increase response rate like pan selective beta-blocker like propranolol and radiation or ablation to one tumor, but having these in clinical trial can be difficult.

[D.ap]

Morning Mary Jo

I’m sorry for the progression report . It sure makes a family anxious to say the least...

I agree with Olga that your first order of business is to allow Prairie to get back to normal after being on cediranib for 33 months.

How is she doing ? Is she on thyroid meds at this time ?
How’s her weight?

Atezolizumab has been an awesome Med for multiple ASPS patients but the patient needs to be healthy, as I know you feel. It can have side affects as is true with all the immune check point inhibitors .

With each Med your team is able to make better preparations for Prairies future needs .

Love

[Vega343]

we have been amazed at tumor reduction on Atezolizumab trial from NIH in the US. 40% tumor reduction in 5 months! Her largest lung tumor was over 4cm. She has had little to no side effects of this treatment!

Link to the trial:
https://clinicaltrials.gov/ct2/show/NCT ... SPS&rank=1


This is a 12 yo, who previously took cedirinib for 3 years. Cedirinib had tolerable, yet hard side effects of diarrhea and low appetite (weight loss). The cedirinib did stunt tumor growth significantly over time.
We are on clinical trial, so I don’t know how effective it’s been across the board,but the docs have said it seems promising for both kids and adults.

Lung scans

609CEF92-68FF-4C0F-AA9A-5511CC94B96C.jpeg (214.93 KiB) Viewed 7923 times

[Olga]

This is a very good news! We already have few people on atezolizumab trail and few people on the other immune checkpoint inhibitors - PD-1 and PD-L1 with many good results stories. Prairie is so young, her immune system should be able to develop the strong tumor specific memory and beat this.