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

[D.ap]

Tanja
Are you all comparing to a prior MRI of the brain ?
Where are the satellite images ? relative to the 2 frontal clustered 6cm images?

Update - 6pm USA time .

[Olga]

Surgery is a good plan, it is good that they can do it really soon. The smaller mets can be treated with the radiosurgery (GammaKnife is better), in ASPS the small brain mets are totally killed by the radiosurgery, tell that to the oncologist - we have numerous cases ppl live years after the brain mets discovery - bigger mets need to be resected and the smaller ones planned for the radiosurgery in conjunction with the immunotherapy - there are multiple articles that in melanoma metastases to the brain combining the radiosurgery to the immunotherapy greatly improves the results.

[Bonni Hess]

Dear Tanja, I am so sorry for having not been able to respond to your posts the past several months, but have been immersed in personal family health and other issues, and am very grateful for the very good advice that Olga and Debbie have been providing for you. I am deeply sorry for all of the increased challenges that dear Saskia has been facing in her courageous ASPS battle, and for her new diagnosis of multiple brain mets. I completely agree with Olga's input in her last response. I am very grateful that surgery has been promptly scheduled to resect Saskia's largest brain mets, and I agree with Olga that the smaller brain mets should be treated with radiosurgery such as Gamma Knife. In past years, our daughter Bruttany has successfully undergone resection of 3 large brain mets and Gamna Knife treatment of a couple of small brain mets. Since beginning her thankfully very successful Cediranib Clinical Trial treatment ten and a half years ago, Brittany has very thankfully not had any new brain mets or other mets. Based on extensive research and observations of other ASPS patients, I would personally warn you against allowing the doctors to treat Saskia with Whole Brain Radiation (WBR) which in my experience is NOT successful in treating/preventing development of notoriously radiation resistant ASPS brain mets. One patient who we personally knew and had met, underwent WBR which devastatingly caused severe short term memory loss, rapid brain met development and growth, and the tragic loss of his Life within a couple of months following his WBR treatment. Unfortunately, too many doctors naiively recommend WBR for ASPS patients. Unlike intensive targeted radiosurgery like Gamma Knife, Cyberknife, or SBRT treatmenr, WBR cannot safely administer enough radiation to fragile brain tissue to destroy ASPS brain mets. My very best wishes, most positive thoughts, and greatest Hope will be with you and dear Saskia tomorrow for a very successful outcome to her brain met surgery. I know far too well what a very frightening and difficult time this is for both of you, but try to stay strong, let Hope lead you through each day, and know that you are not alone as those of us on this Forum are here to provide information and strengthening support for you. Take care of yourself dear Tanja and please keep us updated as you are able. Reaching out to embrace you with gentle hugs, special caring thoughts, healing wishes, warm friendship, and continued Hope, Bonni Hess, mother of now 37 year old Brittany diagnosed 18 and a half years ago in July 2001 at age 19

[D.ap]

Saskia ,Tanja and family ,
Prayers coming your way today and always !💕

Love ,

[Saskia]

Dear lovely supporters
Hell of days behind us.... On Tuesday and Thursday Saskia was near death. Surgery was planned for this Friday morning but we had a call in the night to come in because Saskia went to emergency surgery straight away. We didn't manage to see her in time, two minutes too late, but she wouldn't have recognized us anyway.
The neurosurgeon was called in for her and in the morning we finally saw her again. Everybody was surprised she was breathing fine and extubation was no problem. Both big Mets are removed successfully. She is on intensive care and this afternoon was happy, laughing and talking to us.... Okay... Slow motion with all those painkillers...
We now pray and hope for recovery of her paralysis she developed more and more on her right arm and partially leg (where also the primary was removed). The head pressure was too high during the last days.
We re so happy and thankful for this outcome and hope she will stabilize further on.

Thanks for all the sightful information for upcoming radiation topic. I have no idea when all this will start.
Radiation for bone met in her leg. And radiation for the head.
As far as I got it the docs want the areas of removed head Mets be radiated and one more met which is on her right front overlap with a measurement of 13x12mm. I think there is an additional tiny met on the top of her head.
When do you think all this will be considered?
And is gamma or cyber knife better for the 12x13mm met?
And for the removed met areas sbrt or what is it called?

Thanks Olga and Debbie for covering up and answering so many questions I had. And hello Bonnie again. Hope your family issues are all lovely and peacefully resolved and health is back to the loved ones. I again started crying when I read about the successful journey Brittany had with asps and I thank God for the stabile outcome and the life she can live. I bet you still have nightmares about it.
At the moment I cannot imagine to cut pictures of the last days out of my head. Saskia lying there, not reacting anymore and so pale. Thank God she had so much strength to stand this 2nd surgery in such a short time. Her head looks terrible with all those cramps in it. This is scaring me.

Good night and bless you for all the knowledge and your emotional support
Tanja

I always read your answers to Saskia to keep her positive
Good night... I definetly need some sleep

[D.ap]

Tanja and family ,

Wow ! I’m so so sorry to hear of the happenings prior to Saskia’s surgery but am incredibly glad she was at the hospital to have the surgery .
Our love and prayers to Saskia and family and caregivers, today and always .
Love ,

[Olga]

Tanja - GammaKnife is the best modality for the brain mets but it should be done pretty soon as the size is getting to big for the successful treatment by it. Ask the brain surgeon when. Right now the main thing is to recover some health to be able to stand all the blows your girl has to endure

[Saskia]

Thank you all,
Can the gamma knife be done while still those iron cramps/staples are in her head? Or do we have to wait until they're removed?
Tanja

[Saskia]

And what about the necessity of radiation at the areas where big Mets have been resected? Is this area too big to be radiated or is it maybe small now, did decrease by removing? (maybe stupid question, but it is somehow out of my league of imaginable pictures).
Sorry. What would we do without your big knowledge of ASPS?!
I want to understand as much as I can because it is so hard to discuss with doctors smiling at us, but we want to do the right thing

[D.ap]

Tanja - GammaKnife is the best modality for the brain mets but it should be done pretty soon as the size is getting to big for the successful treatment by it. Ask the brain surgeon when. Right now the main thing is to recover some health to be able to stand all the blows your girl has to endure

Tanja
Olga has a very good group of suggestions , for Saskia to get stronger , and to consult the brain surgeon to time the gamma knife procedure .
The remaining met is 1.4cm and located where?
When we obtained negative margins with Joshua’s primary of his calf, we consulted a radiologist who told us there was a 15-25% chance of the sarcoma to reoccur.
So we did not have it treated and it’s not active to this day 8 years later .
I believe the resected area would require margins to be treated , but I am not a doctor .
Get that gamma knife consultation set up so when Saskia is strong enough , it will be available to be used when the docs feel she is strong enough .

One thing I do know is alveolar is best fought when a patients body is at its most healthiest and they’re most rested. It sounds logical but it is very very important in combating this typically slow growing sarcoma from changing to a more aggressive state .

Hope today finds you and Saskia better rested .
Much love ,

[Olga]

About the resected brain met bed. The big difference between sarcoma metastases and primary cancers in the brain is how they grow. Cancers infiltrate the tissue and form some kind of snowflake so if you resect them, you have to resect lots of healthy tissue in between the branches. ASPS metastases are like cherries, when they grow they push the surrounding tissue out to make the space for themselves. So basically when the brain mets are removed, very little brain tissue is lost around it (the margins) and ideally the brain tissue would spread back if there is no brain met anymore. Ivan had the brain met removed and was out of hospital in 3 days, but it was smaller and removed with the negative margins (a rim of healthy tissue around it was taken). He did not have a radiation to the tumor bed and no recurrence. You have to discuss the surgery details with the surgeon - if this cluster was removed with the margins or not.
A radiosurgery increases the intracranial pressure a lot due to a swelling around the met so that probably going to be a limiting factor in timing, and also if they need to perform an MRI to map the location - can not do it with the metal stuff.
Saskia is on steroids now anyways, may be they can do it now, I do not know. You have to be ready to mood changes as the time on steroids goes, the high dose makes ppl very irritated, bossy and demanding as they overdose eventually. Just make sure you understand there is nothing personal but it is drugs in the system.

[Bonni Hess]

Dear Tanja, Thank you for your faithful updates in the midst of all that you and dear Saskia have been through. I know FAR too well how extremely physically and emotionally exhausted you must be. I am deeply grateful that you are so pro-active and knowledgeable, and that you are providing so much strengthening love and support for your precious young daughter. I am SO VERY grateful too that dear Saskia's largest brain mets were able to be successfully resected and she seems to be recovering well from the surgery. I strongly agree with all of the information that Olga shared with you in the previous post.. Based on our personal experience with our dear Brittany's brain mets resection, as well as my extensive observations of other ASPS patients' brain met resections, ASPS brain mets are typically encapsulated, and according to Brittany's excellent neurological surgeon, "pop out like marbles". Because they are typically encapsulated, they thankfully don't have fingers that extend into adjacent brain tissue like most malignant brain tumors. Therefore, no post-op brain radiation is usually required to the resected tumor bed.
However,, I am personally unfamiliar with a case of ASPS brain mets being in a "cluster", as you described Saskia's, so this situation may be the reason that Saskia's doctors are advocating post-op brain radiation to the resection site, and needs to be discussed with the neurosurgeon. I, like Olga, urge that Gamna Knife be done for Saskia's smaller brain mets as soon as possible since ASPS brain mets grow much faster than ASPS mets in other parts of the body, and Gamna Knife is unfortunately not successful for larger ASPS brain mets, as we devastatingly learned following Brittany's failed Gamma Knife procedure 14+ years ago for her parietal and cerebellum lobe brain mets which subsequently had to be surgically removed heartbreakingky resulting in the loss of her peripheral vision. I understand that Gamma Knife cannot be done until Saskia's metal surgical clips are removed, but Hopefully she will heal quickly so that the clips can be removed soon, and a Gamma Knife or other radiosurgery procedure done. In the meantime, you both need rest as you recover from all of the physical and emotional trauma that you have been through. Take care dear Tanja, know that I and the others on this Forum are here for you and Saskia, and give her and yourself gentle hugs from me. Reaching out mother to mother and heart to heart to embrace you with my deepest caring, healing wishes, and continued Hope, Bonni

[Saskia]

Hello, need to be short as for really less sleep, but I'm thankful for your warm and helpful words from the bottom of your hearts. This so helps to calm down a bit in all this struggle.

Surgery report is not yet written but we've been told all was successful. So I guess removed with good surroundings. All doctors are pretty satisfied with Saskia's condition now. Not bossy anymore but laughing and happy and strong and thankful. She's incredible..... And hungry at the moment like she hadn't been before since weeks.

OK, my points before I'll fall asleep:

Radiation:
Talked to oncologist that we consider and would wish gamma knife and not WBR. She will discuss this with the Tumorboard. Cooperation partner hospital according radiation does not have gamma knife. So Saskia would need to be transferred to UCT Frankfurt which practices with Saphir Radiology. Yeah! I mentioned Dr. Black as you suggested before already but unfortunately he is on vacation until March!

Do you have medical publications or reports about gamma knife being best for asps skull Mets?

On latest MRI report there are 4 more Mets mentioned.
High frontolateral 4,8mm
Right frontolateral 4,8mm
Right frontolateral 1,4cm (I think this is the one I mentioned 12x13m already)
Left parietal parasagittal 6,4mm

So the report says under evaluation: multiple supratentorial Mets.

Oncologist considered there might be like 10, 15 or 20.
I'm confused but she will recheck with a radiologist to have a real diligent look at it. So I asked for exact sizes, locations and numbers.

If there are several small ones, but definetly under 1cm plus the bigger one (gamma knife for it) what shall we do with the smaller ones?
Also gamma knife or wait if abscopal effect comes or immunotherapy will force them to decrease?
Is there a minimum size for gamma knife?
Maximum is 30mm?

Therapy:
Oncologist will request Atezolizumab (PDL1) at insurance now because another ICI PD1 might not be approved by insurance anymore. Not sure

Pathology report primary:
90%vital and active, 10% regressive, necrotic
Stadium: ypT4, ypM1, L0, V1, R0 (at resektat)
Regressiongrading ref. to Osteosarkom Salzer-Kuntschik is 5.
Highest mitotic counts is 12 pro 10 HPF
MIB-1/Ki67 proliferation rate is 15-20%, in hot spots up to 35%

Unfortunately the removed skin lesion is confirmed to be asps.
(Saskia has other skin Mets at her neck. Shall we let them be resected or is it a good thing to watch them maybe decrease by immunotherapy etc.?

This is all too much for me right now. I only function like a robot. It's crazy.
Sometimes planning pLliative teams for our home, sometimes fighting everything and also trying to be happy for my little peanut Saskia.

Her paralysis on her right side with arm and leg is not so much better unfortunately. I'll give it a time and do massages daily.

Good night
Tanja

[D.ap]

Tanja ,
The paralysis could be from swelling ? Time is the healer .

Let’s regroup and we will try and find papers to give your team , as I personally feel they are on top of what’s best for Saskia .👍🏼
Multiple images 10-20 could be something other than ASPS?
Have you view the MRI of what was pathologically deemed ASPS( surgically removed )in the brain ?

Let us know .. certainly get some much needed rest and give Saskia a hug from all of us.

Our families love to you .
💕

[Olga]

Tanja - good resection for primary, R0. There is probably no point to have the leg irradiated.
Brain metastases that are visible can all be treated but if the ICI is planned (atezolizumab is also good we have few good stories with it) you should try to get the radiosurgery sooner and the ICI later for the immune system to recover if the steroids are needed and at the same time if the steroids are not planned. They can also apply for Opdivo off label?