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

[Saskia]

Yes I saw the MRI and CT after surgery of head. Removed areas looked clean.
I'm most scared of the mentioned multiple remaining Mets and hope they can be treated by gamma knife. How many could be done in one session? Is this depending on the locations?
Most scaring is also that we have to fight for everything very hard. I also have the feeling they're giving up but Saskia is so tough and so strong. I so want something to be working. We've been told that no one wants to do any surgery on her anymore and talking too often about palliative care.
We still want to try the gamma and immunotherapy.
Re. Leg: only bone met is planned to be radiated. OK?
Tanja
Re. Opdivo: you think is better than Atezolizumab?
We found one studyII for Atezolizumab, do you have other reports or public journal for prove?

[D.ap]

Tanja
Hello . I personally feel that you need to begin the interview with the radiologist and get their feed back on what they know about gamma knife .
That would be the best use of the time while Saskia gets stronger .
Mention circadian rhythms and scheduling in the morning the radiation treatment, when brain activity is low and the radiation is known to be more affective while discussing radiation treatments.

viewtopic.php?f=8&t=557#p2570

Will they be checking on the pathology of the brain tumors soon? My limited understanding is that yes they of course were tumors but were they creating the exponential growth rate ( progression )or was the ICI inflammation(pseudo progression ) the majority of the large mass being seen on the MRI?
There have been cases of inflammation not tumor progression being surgically discovered with brain mets, while on ICIs.
Pseudo progression determination would be a case to continue with Keytruda at the right time .


Olga posted an excellent article in 2019 on the pseudo progression , while patients had brain tumors removed to of been thought to not of been responding to ICIs.

viewtopic.php?f=92&t=1771&p=14008&hilit=Pseudo#p14008
The progression during Saskia increase , certainly needed to of been treated . With the surgery having been performed , is the most known reliable method to stop metastatic Asps to date .

The 1.4cm definitely needs ablation sooner than later however as Olga pointed out the staples and recovery (control of the swelling ) need to be addressed first.
There is an ASPS patient in Canada who was on atezolizumab with great success .

viewtopic.php?f=83&t=1295#p9909

Our son Josh also had great success with Opdivo .

viewtopic.php?f=82&t=1297
He and another young man had great results with much of their disseminated diseased areas .
Thinking of you all ,

[Bonni Hess]

Dear Tanja, Thank you for your thoughtful detailed updates in the midst of what I know must be your complete emotional and physical exhaustion. Olga and Debbie have provided you with some very good information and input which I agree with. It will be important to discuss with the neurologist and radiation oncologist the specific location and number of dear Saskia's multiple smallest brain mets to try to determine if they can all be treated with radiosurgery such as Gamna Knife. Based on our personal experience with Brittany's brain mets, Gamma Knife is unfortunately not successful for larger brain mets, so it is critically important that Sansia receive radiosurgery treatment for her brain mets as soon as her brain resection inscision has healed well enough to have the surgical staples safely removed. I echo Debbie's concern and questions about what may have caused Sansia's uncharacterisic-for-ASPS rapid disease progression and explosive spread of her mets. However, after 18+ years of hard earned experience with this insidious and very challenging disease, I know that it can be very unpredictable, and every patient is unique. I also know that there is always reason for Hope and to continue pro-actively fighting, no matter how overwhelming the situation may be, nor how negative or pessimistic the doctors are. Prior to Brittany beginning her Cediranib Clinical Trial ten and a half years ago, her disease had become very aggressive with rapid metastatic spread to her brain, pancreas, spleen, duodenum, small bowel intussusceptuon, and a subcutaneous met on her abdomen. We were told that her Life threatening pancreatic met was unresectable and untreatable, but we refused to accept the doctors' dismal prognosis, relentlessly researched and searched, and found one spot left in a Clinical Trial in Edmonton, Alberta testing the promising new Cediranib drug which was able to cross the blood brain barrier and had provided initial treatment success for an ASPS patient in England. Brittany's doctors in Seattle, Washington and most other doctors had never even heard of Cediranib and were completely unfamiliar with it. My message to you dear Tanja, is that doctors don't always know everything, especially about this extremely rare and poorly understood disease, nor do they always have the most accurate or up-to-date ASPS treatment information, so please stay strong, informed, and pro-active as you continue to fight to save your courageous daughter"s precious young Life. With deepest caring, healing wishes, warm friendship, and continued Hope, Bonni

[Saskia]

You're the only ones giving hope :(
Thanks so much :)
Saskia is supposed to come home on 5th and I need to order some things before like special bed, toilette and wheelchair. She s having physio and massages right now and is in good care at palliative department at the moment.

Decision of Tumorboard today:
On 10th appointment with Uniklinik Frankfurt for information about WBR!!!
I insisted to contact pls Saphir Radiology to have their opinion on gamma knife pls. Our oncologist will do her best. If Saphir would agree it might happen that we need to pay for it ourselves in case we have no proof that this is the right treatment for asps brain Mets.
Decision for WBR was because of multiple Mets.
I still don't know how many. Maybe there are 20. I have no idea.
As far as I understood you said that ALL small and visible Mets can be radiated, right? With gamma knife? Or which one?

I understood that the usual WBR does not work on Asps brain Mets in general.
I think it's because of the sort of radiation.

Do you have public reports, journals, studies or anything medical proving this?

I have the feeling that this is not known to the doctors and they only have the knowledge that it works on different brain Mets from other sarcomas or cancer.

I'm completely devastated at the moment and the only thing holding me up is you and the fact to hVe an overnight sleep next night at Saskia s room :) and that she's coming home next week.

She's still so tough and is exercising to move her arm and leg again.

Tanja

[Bonni Hess]

Hello again dear Tanja, I am so grateful that dear Saskia is receiving good care and that she is recovering well enough to come home in a week :) There is truly no place like Home for healing ;) I am sorry about the obstacles that you are encountering in your efforts to obtain the best brain met treatment for Saskia. Olga or Debbie may have specific links to documented data regarding the success of Gamna Knife for small ASPS mets. The only thing that I can offer is our personal experience with Brittany's successful Gamma Knife treatment for several of her small brain mets. It is my understanding that several brain mets can be treated with Gamna Knife at the same time, but I assume that several different Gamma Knife treatments would be necessary to treat a large number of brain mets such as 20 like Saskia has heartbreakingly been diagnosed with. These are questions which you need to address with an experienced interventional radiation oncologist. Additionally, regarding the doctors' strong recommendation for WBR, you need to demand that they provide you with documented data regarding successful WBR treatment for ASPS mets. This is definitely a treatment decision which should NOT be made without adequate research and documented data regarding WBR treatment success with other ASPS patients. Too many doctors naiievely recommend treatments for ASPS patients that may be successful for patients with other types of cancer, without knowledge of, or experience with, notoriously radiation resistanr ASPS. Continue to be strong, pro-active, and to let Hope lead you through each day, and to know that you aren't alone on this difficult journey dear Tanja as those of us on this Forum are here to help you with shared information and strengthening encouragement and support. Holding you and dear Saskia very close in my heart and reaching out to embrace both of you with gentle hugs, special caring thoughts, healing wishes, warm friendship, and continued Hope, Bonni

[D.ap]

Hello Tanja
I’m incredibly happy to hear you are able to stay with Saskia . It’s a moms wish and so so very important .💕

I truly agree with Bonni in that your docs need to present a case for whole brain treatment .
Asps is a VERY rare sarcoma and it’s not easily attacked with low wide spread radiation .
Is there data available that lets them to believe that Saskia has had a good response to the Keytruda and the whole brain treatment will be successful in her regiment and the now time table of treatments ?
I’m far from a doctor but as a mom I’d like to know of how this plays into my child’s present as well as their future well being.
Found a 2019 write up on whole brain treatment and ICI

From Whole-Brain Radiotherapy to Immunotherapy: A Multidisciplinary Approach for Patients with Brain Metastases from NSCLC

https://www.ncbi.nlm.nih.gov/pmc/articl ... po=44.4030
Obviously you’ve all had ici then are being offered WBR
The brain mets preliminary were not factored into preemptive treatment ? Not seen so ICI may have actively activated remote mets?
Please realize that palliative care Isn’t the end of life given.
It certainly can be a service to aid a family to be able to regroup . For the patient to get stronger and let the family be the family . Not the doctor .❤️
Love to you all ,

[Saskia]

Hello you lovely people,
Just wanted to let you know we are happily home from hospital since wednesday. Everything works fine. Doing physio to get more and more rid of paralysis of saskias right side and are relaxing and sleeping to be prepared for Monday to discuss radiations. Keeping thumbs up to get the correct treatment. I know all those appointments will again get exhausting but needs to be done. I hope brain Mets did not grow and abscopal effect did its job as well already. Pray for it.
Ivan's and Brittany's journey with asps gives us hope always.
Thanks for your support.
Tanja and Saskia, bro and dad

[D.ap]

Hello Tanja,

I’m so incredibly happy to hear of Saskia coming home and her improvements. Thank you for including us in this news.

Was a pathology report written on the brain tumor yet?

Sending hugs and prayers today and always .
Love,

[Saskia]

Hello
We had an appointment on monday at UCT Frankfurt and Saphir Radiology. UCT recommended WBR plus boosts on visible Mets but with no further reports, studies etc for success on asps brain Mets. Decision from their side to stop growth, sometimes reduce. Nobody was aware of radiationresistable asps let's say in general. I asked if they had any success on asps patients already. Nope.
At Saphir we had a good consultation to compare WBR incl boosts with cyber knife on the other hand and finally decided (for sure) for cyber knife.
Gamma knife is not applicable anymore.
As we don't have an MRI post surgery details will be discussed next week. On Saturday we'll have the MRI.
21st will be planning CT and MRI at Saphir and the following week radiation. Maybe 1-3 fractions depending on how many Mets and their locations.
We are relieved so far.

Re. Pathology report of brain mets: it only confirmed asps Mets.
I'm sorry but I didn't understand what you meant by "remote Mets maybe caused by ICI".
Could you pls explain this and what we need to know exactly or what it would mean or effect on any outcome or treatment?
We tried to figure but don't get it unfortunately.

Additionally we are sooo happy to have approval for 6 more rounds of Atezolizumab.

Love
Saskia and Tanja

[Olga]

Super happy to hear that the consultation with Saphir went well and Atezolizumab approved for 6 months more. Now it is VERY important to schedule the atezolizumab doze and the SBRT treatments at the same time, starting +/- 1 day. Is Saskia still on steroids?

[Saskia]

Thx Olga for the reminder. We'll keep this in mind and will hopefully work out.
We'll have bone met radiation sometime later anyway which is also a second chance for combination.

Tanja

[Bonni Hess]

Dear Tanja, Thank you for your thoughtful update. I am grateful that things are moving forward in a positive direction to treat dear Saskia's multiple brain mets without the concerning use of unproven WBR. It is very frustrating, although given the rarity of ASPS, not surprising that the doctors were not aware of the well documented radiation resistant nature of ASPS. It is always very concerning and upsetting to me that too many doctors continue to recommend and advocate standard treatments for ASPS which have no documented success for treating this extremely rare disease. This is why it is critically important and essential for the patient/patient's family to be as pro-active, as knowledgeable, and as well researched as possible, as you have thankfully been. Please give dear Saskia and yourself gentle hugs from me, stay strong and pro-active, and keep in touch with this Board as you are able. With special caring, positive thoughts, healing wishes, and continued Hope, Bonni

[D.ap]

Hi Tanja and Saskia,
I echo Olga’s and Bonnis happiness to moving forward with treatments that have been successful with ASPS. As well as I’m hoping Saskia is feeling better with each days progress .😊

I wanted to reiterate from my above post to mention the statistical success of circadian rhythms and scheduling in the morning the radiation treatment, when brain activity is low and the radiation is known to be more affective while discussing radiation treatment.

viewtopic.php?f=8&t=557#p2570

Also in reference to path report, there should of been a degree of in depth reporting on proteins other than positive for TFE3..
Biomarkers of immune cells maybe ?
I feel that report will tell how well the Keytruda ( ici) was affecting Saskias brain tumor and consequently maybe how it was acting systemically , with her disseminated ASPS.
I’m not a doctor however there are studies out there that show those markers of ici succession success and or flow charts , and let those reports aid your team in helping you with Saskias care . Possibly as Olga has stated in making sure Atezolizumab Infusion and radiation sched being of top priority ?

Is Saskia still on steroids ?

[Saskia]

Hello lovely people
I'm always so pleased to hear or read how long you guys stick to good treatment with success and love your lives and enjoy. We know how hard this is.
On Friday we had all preparations for cyber knife this upcoming week for saskias remaining brain Mets. Latest update we had were 6 which had been growing indeed from 21st Jan to 15th Feb. E. G. from 14mm to 22mm or 7mm to 11. So we need to speed. She had MRI and ctand the mask prepared for radiation. We had to confirm not to radiate the tumor bed. Which we decided because no tumor cells are showing there at the moment and to reduce the fact to suppress immunosystem. Hope this decision was correct. We can do cyber knife anytime when anything is showing again we decided.
I also asked to do it in the morning. Let's see if it works for saphir Radiology.
Unfortunately we cannot combine it with approved immunotherapy Atezolizumab at the moment because Saskia is still on cortisone and it will take 3 to 4 weeks I guess to reduce this :(
We will start Sutent, maybe in combination of D L methadone. Have a CT lung in the beginning and after 6 weeks to check if it works. Then will decide if to continue, if she's feeling fine with it or if to switch to Atezolizumab then.
The reason for this idea is that insurance company wants to see good results after 6 times immunotherapy and we don't want to waste this chance if the effect would be less in combination with cortisone (dexa). I read that the medial effect will maybe only be after 5 times up to 11 :(
We hold our thumbs up.

Option of Combination of radiation and immunotherapy we will grab at once in case bone met of her femur will be radiated once.

What do you suggest to do with her subcotan skin Mets at her neck which are really not nice to look atand we also already not on purpose scratched a little bit? There is a clump of ca. 3 and 1 big.
Somehow I feel that it would be really easy to remove them by local Anästhesie to just get rid of them and reduce tumorburden further on.
What do you think?remove the 3 and the one big?
Then there are still very very little ones on her back. Let's say 2. Which hopefully will react to treatment.

Have a wonderful sunday and cherish life. We are thankful

Tanja

[Saskia]

P. S. We still don't have a more detailed report about the brain Mets nor skin met removed except the fact it's asps. I also think this is ridiculous and not enough. I have the feeling that there's nothing known at pathology about testing any biomarkers of immunotherapy or anything you mentioned. Mmmmhh...