Saskia from Germany - Dx 2019 at 23 years old_RIP Aug 2020

[D.ap]

Thanks for the update. Add Q - skin mets biopsy?
About their arguments re. small number of the ppl on the study - that was my argument with our drs here in Vancouver, when they offered Sutent and would not cover Keytruda initially, I presented all the cases I found and told them that if they consider it reasonable to offer Sutent based on small sample statistic data, they can not say that Keytruda data is not statistically significant - they both relatively equally statistically insignificant.

Hello Tanja,
I certainly under stand your frustration with the limited study being ok on one hand but not on the other .
Is there anyone you could talk to that would be able to explain why the doctors are pushing for the trabectedin?
They would have a paper and or studies that would’ve why they are suggesting to do so.

Another question I have is which tumor was the molecular analyzation performed ?
Saskia had said there were 2 biopsies performaned?

[Saskia]

Hello Olga and Debbie

They suggested trabectedin or Doxo+Ifo because all others failed(tki and ici). This is the reason mentioned. I will ask for details on Monday at saskia's oncologist and on 30th Dec when we will be in Essen to have a second opinion.

Both biopsy results came up done on her leg, primary tumor.
She also had a VATS in May but without feedback as they had not enough tumor material.

Tanja
(pls have a wonderful merry xmas time)

[D.ap]

Thank you for the info Tanja
Was there a mitotic count on the pathology report?

I also want to point out that the reason Olga is interested to know if the skin lesions are indeed ASPS, is that if so , they could be used as an area to more safely radiate along with the correlating of an infusion , for an Abscopal affect .
Certainly a Merry Christmas to you as well.😊💕

[D.ap]

Ps
Has Saskia had lower pelvic scans?
How are liver values .

[Saskia]

Hello

Okay understood now re. Skin Mets to maybe radiate here for abscopal effect. Will ask tomorrow in hospital to do so before surgery on leg. Might help her lungs in advance then.
Right?!
Or also in combination with K.

Mitotic counts are not mentioned in any pathology reports :(

Complete pelvic MRI was in August. Clear. With MRI leg they went up a little and it was clear as well.
She had an ultrasound last week of tummy area to see if organs are good size. All fine.

GOT was 38 on 4th dec

Tanja

[Olga]

I am not sure re. skin mets being a good target for the possible abscopal effect creation. My question would be to confirm that these are the true mets and not the autoimmune adverse side effects of keytruda, there are multiple kinds are known that might be caused - versus the real mets.
As the target for the radiation it might be not that good as if it is in the skin or immediately under the skin (subcutaneous) the radiation damage to the skin will be extensive and I would avoid that as it suppresses the immune system. Healthy skin is an important component of the good functioning immune system. It might be a pretty convenient and easily accessible target for the intratumoral injection for the dr in Spain if needed at some point.

[Saskia]

Hello all and hope you had a wonderful Xmas,

Re. Mitotic counts, we found from VATS in may: 1 mitose on 10 HPF, but that's when they told us it's not enough tumor cells which they were able to take.
1st Biopsy from the leg in April we had 10% profileration mentioned.

2nd biopsy from leg in august: we have 3 pages of genetic analysis which I don't know what is important. Unfortunately I cannot upload it here because it always tells me the document being too large.
Olga, can I send it by email to your hands?
It is molecular diagnostic, oncomine comprehensive assay with different sequences done and fusions, biologic rating, etc.
E. G. Allelfrequency of 46% for fgfr4 mutation?!?... And more details if interesting.

Re. Skin: oncologist told us not to do a biopsy now but then remove the skin irritations during surgery of leg mid of January for pathology.

On Monday we will be in Essen for an appointment with Dr. Bauer, oncologist, for a second opinion. Surgeon in Frankfurt is awaiting his opinion for treatment.

Thx for all your help
Saskia and Tanja and family

[D.ap]

Saskia and Tanja,

Thank you for the update . The mitotic levels look typical of an ASPS patient .

They both indicate a 10% replication of dividing cells , I believe . 1/10 and of course 10 proliferation.
Our sons was deemed to of been 6/10. 60%

I’m not sure if you’ve had a chance to read thru the pathology section of the icureasps topic , so I am linking you 😊

viewtopic.php?f=61&t=1052#p8012

Stay strong and ask lots of questions !
Will be thinking of you both , all as it certainly affects the family .
Love ,

[Saskia]

Dear Debbie
Thx for the input especially re pathology reports.
I'll try to get through and compare but indeed saskias values seem to be usual asps conform. We struggled a bit because of fgfr4 outcome but had feedbacks that it is normal for 55% of all humans and to be somatic.

Feedback from Essen sarcoma center yesterday. We are happy the doc supports the surgery and resection of primary. He positions the surgery as only palliative regards Saskia's mobility and QOL but anyway....
He doesn't see a benefit of reducing tumor burden, nor better efficiency for treatment.
He's also not convinced of any abscopal effects.
He's of the opinion that radiation can decrease asps.

I tried to discuss those points and prove by all provided documents but couldn't convince.

He's very surprised that none of already started treatments had any impact so far and wondered why we started in May with Cabozantinib.
Therfor he suggests now, as he doesn't see Keytruda working as well right now, to start Sutent after surgery.

We phoned Saskia's oncologist afterwards to let her know.
At least we are happy to not have an outcome of the day with a no to surgery and a suggestion for chemo at the moment.

So mid of January will be the resection and maybe we will have 2 more rounds of Keytruda, then a CT lung to see what happened and only then would switch to Sutent.

What is your opinion about this ongoing plan?

Dr Bauer from Essen knows Dr. Wilky and will meet her in a month or so in Essen directly for a conference.
We for sure hope he could discuss Asps aspects then as well.

Have a wonderful last day of the year 2019 and best wishes for the upcoming 20!
Tanja Saskia and family

[D.ap]

Saskia
Found an ASPS patient who may of had a anti FGFR response to what were clinical trials phase 1, of durvalumab.
She had been on an anti FGFR Med and went into durvalumab with great success .

There is a doctor Albiruni R. Abdul Razakn in Olga’s neck of the woods ( Canada) who has several ASPS patients .
Maybe contact him and hire him for a consult ?

viewtopic.php?f=88&t=1399#p14421
The clinical trial link of 2017

https://www.ncbi.nlm.nih.gov/m/pubmed/30018044/
Full text

https://cancerimmunolres.aacrjournals.o ... /1001.long

[Olga]

From the article, it seems that they consider her responding to durvalumab (anti-PD-L1)? and not responding to other drugs before of that.

[D.ap]

From the article, it seems that they consider her responding to durvalumab (anti-PD-L1)? and not responding to other drugs before of that.

Moving to discussion in “about the trial”

viewtopic.php?f=88&t=1399#p14421

[Saskia]

Hello
Just to inform you. We're preparing for resection of primary on Tuesday. Saskia will be in hospital tomorrow already. She suffered a lot during this week of headache and her right arm can hardly be moved since a nerv we guess was damaged by last time contract medication for CT lungs mid of Dec.
So during her stay in hospital we will also try to see a neurologist for the arm and try to have an MRI head.
Thumbs up for the resection for now.

I will ask pathologist for the following :
- how much of the whole primary is asps?
-is there something necrotic or inflammation? How much of it?
-mitotic counts?
-profileration
-do a KI67 test for aggressivity of tumor/cancer

Skin irritation:
-is it metastatic?

Did I miss something important?

Is it important to keep samples in parrafin AND nitrogen in case we need something later?
E. G. For doc in Spain or any other studies?
I'm not sure what's better.

Hugs
Tanja

[Saskia]

P. S.
We will also discuss and ask for immunotherapy durvulumab+tremelimumab instead of sutent.
I understood you would suggest this way.

[D.ap]

Hi,

Thx for the link for comparison to Asps. This supports and stresses the point to have resection as well I guess to strengthen the outcome and effect of ICI or other treatment on Mets.

1st MRI of leg/primary: April 19
2nd MRI in August, no growth, stable
3rd MRI 4th Dec, growth 2cm since 1st Aug.

CT lung: may19 (over 100 small mets) and 1st Aug 19
They only told us there were more mets, not stable.

Treatment was:
May - aug: cabozantinib
2 weeks pazopanib
22nd Aug - 04th Dec. Keytruda + axitinib inlyta

Tanja and Saskia ,

I’m sure sorry to hear of the contrast complications .
Ie headache and nerve issues . Will Saskia have head to toe , scans soon ?

Our love and prayers are with you all as the surgery day approaches.

I would hope that the doctors will give Saskia some time to recover before embarking on another Med .
The combination duravalamab and Trem, is to my understanding a big step up from the Keytruda axitinib .
Also after the tumor is assessed of how it reacted to prior treatments( baseline of biopsy compared to current path report) your team may determine Saskia go to a different ICI?

With the evaluation of the removed leg tumor the pathologist will be able to see what affect the Caboz,the votrient and the Keytruda /axitinib have had on Saskia tumor ,my opinion .

The skin lesion is being removed as well?

Love and prayers ,