Willem from the Netherlands - DX 2013

[Olga]

Hi Willem,
ask the surgeon re. isolated limb perfusion - done before of the surgery in attempt to reduce the size of the tumor and to make it to move away from the nerves/veins/bone - if it shrinks a bit. It is done in Europe widely for sarcomas now, we do not have any experience with it in our community. ASPS is a chemoresistant disease but who knows, may be ERASMUS MC in Rotterdam where lots of work was done had ASPS patient before? Investigate it. Also consult re. cryoablation of the tumor to shrink it and neutron radiation therapy. All attempts have to be done to preserve the walking ability.

[WilL]

Unfortenately I got bad news from the surgeon, they think it is to risky to resect:( Espescially because of the nerve and the bone. And they think surgery without resect everything is not a good option because of possible faster growth after the surgery and worser healing of the wound. Cryo-ablation was in order to the surgeon also not a good option, espescially for the nerve. And they don't see the advantage of resecting the primary for the lungs, because they think that surgery of the lungs is not an option. He also knew about isolated limb perfusion but didn't have too much expectations from it.

I think the best option to do now is to ask for a second opinion. Any suggestions for a specialized hospital?

[Olga]

Hi Willem,
do not be discouraged with the bad news - we deal with it almost every time we get a problem with Ivan's ASPS - it is always all the bad news from the local drs and they do not have a solution as how to fix it - but there are other wonderful drs in the world that could have different methods and can help you.
I have two questions for you:
- Have you consulted Dr.Rolle re. lungs? He is retiring very soon, few months may be. The bigger lung mets need to be treated ASAP anyways - resected or cryoablated, they are getting really big and it is not in your interests to let them grow further.
- What is your current treating hospital?
There is pretty active sarcoma unit at the ERASMUS MC in Rotterdam. You can also consult Dr.Issels in Germany he is specializing in unresectable soft tissue sarcomas - to make them resectable, I would ask them if they had any ASPS sarcomas and what was the response with their approach - they use a regional hyperthermia in addition to regional chemotherapy:
Klinikum der Universität München-Campus Grosshadern, München, Germany.
Rolf.Issels@med.uni-muenchen.de
We have the members who lost their limbs due to locally advanced tumor, but they still get to live - I know someone with ASPS who lives after his leg was amputated till the knee and he had 2 lung surgeries, and is doing very well. I realize it sounds pretty horrible - the prospect of loosing the leg, but you should consider alternatives and face the reality. Besides there are more ideas now as how to make the unresectable tumors - resectable, but you have to work on it - neutron radiation therapy, cryoablation, hyperthermia, regional chemotherapy - they expert in this areas have to be consulted personally, only they can say if it could work for you - to save the limb but still to resect the tumor, otherwise it is going to destroy the bone.

[MartinBube]

Hi Willem,

I would just want to 100% support what Olga wrote. Local doctors that had 1 case of ASPS in their entire life or not even seen a case can be very discouraging with their advises. it is a rare disease and they do not always know much. However I often struggle to understand why they do not use their connections and knowledge to find better treatment plan than suggesting always what is their own opinion

Please take a look at all the options that Olga suggested to you and keep your spirit up because that is the best remedy for fighting this damn disease every day.

[MartinW]

Hi Willem, i tried to PM you. I'm located in the Netherlands. You could think about a second opinion from dr. van Coevorden. He is a surgeon and deals with the very few ASPS patients in the Netherlands. He is open to comments from this forum and knows that this board has very deep knowledge about ASPS.
http://www.avl.nl/topmenu/afdelingen/chirurgie/

[WilL]

@Olga, I just send my latest CT-scan to Dr Rolle, he was willing to look at it, so I'm curious about his opinion!
My current treating hospital is LUMC in Leiden. I will check the possibilities in Erasmus MC. Furthermore I think I'm going to ask for a second opinion in AVL Amsterdam, recommended by MartinW.
Thanks for all the advises and support!

[D.ap]

Willem

Dr Rolle is truly a good soul
Please let us know when u get the word from Professor Rolle
Love and prayers tonight and always
Debbie

[WilL]

Hereby an update from the investigations done last weeks. Unfortunately Dr Rolle thinks it makes no sense to resect the lung metastases. Also if the primary would have been removed he thinks there are too many spots in the lungs. The two biggest sport in both lungs should have been removed by lobectomy, and after that there are still a high number of spots left.. And that was a big disappointment. On the other side: I got the same message 2,5 years ago. But we got hope by all the good stories of Dr Rolle on this forum and the somehow positive message from the surgeon about removing the primary the first time I had an appointment.

Luckily we got an appointment at the Erasmus MC for Isolated Limb Perfusion for my primary, recommended by my surgeon and Olga. The idea of the ILP is that they are going to connect a heart-lung machine to my leg, by connecting it to my artery in my upper leg and a vein in my lower leg. Then they are going to supply a standard chemotherapy together with TNF and hyperthermia. They are closing of my leg to the rest of my body and supply it for 2 hours. They say most of the people (80%) showed a response to the treatment. The treatment is especially meant to control the size. The best result is that the tumor would become resectable. The treatment will be done at the beginning of January. Hoping and praying that the treatment will be effective!

[Olga]

Hi Wil, I have a question re. these two biggest sport in both lungs that Dr.Rolle says should have been removed by lobectomy. Can you consult with some advanced cryoablation dr if they can be ablated? It looks like they have to be addressed rather soon as they are going to became a problem. The best Dr. to ask is Dr.Littrup as he is the top cryoablation doctor who might be able to ablate in the locations no one else would - he can give you his opinion long distance without the need to travel, you will need to send him a copy of the CD with the latest CT scan. His info is here:
http://www.cureasps.org/forum/viewtopic.php?f=55&t=1003
the problem is that one ablation is now close to $20,000 and you need 2, but it potentially could save you 2 lung lobes. How big are these two centrally located lung mets? I would send him a scan, and he might be able to recommend someone skilled in Europe to perform that (although I do not expect that anyone would be even close to him as his specific experience goes back by 10 years).
On the other hand we have a member here - Amanda M from Canada - she had one lobe removed and some chemotherapy treatment for the rest of the lung mets and is surviving many years after:
http://www.cureasps.org/forum/viewtopic.php?f=4&t=249
I am glad to hear re. ILP and hoping for the primary eventual removal or control. There are few very interesting drugs in development now but it seems that their efficacy is higher with the lower tumor load in the body.

[D.ap]

Hello Willem

Thank you for keeping us upto date on your treatment .
Thoughts and prayers coming your way in January .

Love
Debbie and family

[WilL]

It's a long time since my last post so I thought it is time for an update:)

I did the ILP to the primary tumor in the leg in January 2016. I got an MRI for the results of the ILP a couple of months later. The MRI scan showed that the tumor activity was very low. I also could feel that very well, the tumor felt weak and mobility increased. That summer I went for mountainbiking in the Alps for one week, so my condition was very good. However I could feel that the tumor activity increased after about half a year after the ILP because of the increased pain.

Together with my doctor we decided to attend the Cediranib trial in London. At that time (June 2016) there were only 2 places left, so I was a bit lucky! Unfortunately I got the placebo in the first instance, so the first 4 months were just for nothing (in my opinion:)) From the end of October I received the real medication. In the first instance the pain in the leg increased. However, the first scan after 2 months of medication showed 20% decrease in the lung mets! From January 2017 the pain in the leg decreased. My last scan at the end of December showed a decrease of 30% of the lung mets, so it continued to decrease! I also could feel that in the cough which disappeared. And the size of the tumor in the leg is like the size more than 5 years ago. It seems to be decreased with even more than 30%, which I hope to discover by an MRI scan.

Then the side effects of the Cediranib: my biggest problem is diarrhea and lose of weight. Because of that the dose was lowered to 2/3 in September 2017. Now the side effects are manageable and my weight increased with 10 kg.

So I'm very grateful and happy at this time! I hope this will encourage others!

[Bonni Hess]

Dear Willem, It was SO VERY good to hear from you and to hear that you are having a successful response to your Cediranib treatment. So often people who have posted and participated on this Forum lose contact which is always very concerning given the unpredictable and sometimes terminal nature of this insidious disease. I am sorry that your Isolated Limb Perfusuion ( ILP) procedure was ultimately unsuccessful in providing sustained shrinkage of your primary thigh tumor, but am very grateful that the Cediranib now seems to have shrunk it 30%!! Now that the primary tumor is smaller, and therefore I assume more resectable, are the doctors giving any consideration to trying to surgically remove it? I know that surgery is not possible while you are taking the Cediranib due to the dangerous increased risk of hemorrhage caused by the Cediranib, but in Brittany's case she was allowed to temporarily discontinue her Cediranib for about 2 weeks in order to have a small non responding abdominal met resected, and then she resumed taking the Cediranib. Since you haven't updated the Forum for the past two years, I don't know if you continue to follow it without posting, but VERY thankfully Brittany has now had 8 and a half years of continued sustained disease stability and no new tumors since beginning her Cediranib Trial in April 2009. Additionally, several of our ASPS Community patients are having very encouraging successful responses to the very promising new PD-L and PD-L1 Immune Checkpoint Inhibitor (ICI) drugs including Pembrolizumab (Keytruda), Nivolumab (Opdivo), and Ateziluzimab (Tecentriq), so there is much reason for healing Hope. My very best wishes are with you for continued Cediranib treatment success with stabilization of your disease and shrinkage/disappearance of your primary tumor and lung mets. In addition to your regular Clinical Trial chest CT and thigh MRI monitoring scans, I Hope that you are receiving at least every 3-6 month abdominal/pelvic scans and an annual brain MRI and full body bone scan to adequately monitor your disease. Take care dear Willem, try to keep in touch and the Forum updated more often if possible, and know how very happy and grateful I am for your Cediranib treatment success thus far. With special hugs, caring thoughts, healing wishes, and continued Hope, Bonni

[D.ap]

Willem

Wonderful news of shrinkage .
Really good to hear from you .
How has your primary changed since 2013?
It was around 10cm near your knee ?

Debbie

[WilL]

Thanks for the replies, I will try to give the updates more frequently! However life is keeping me busy and it's also nice to have a sort of vacation from being busy with the disease..;) I visited the forum a couple of times last two years, so I was a bit aware of the good results of the PD-L and PD-L1 Immune Checkpoint Inhibitor. It gives hope that there are a couple of backup options!

My primary was about 10 cm in 2013, indeed near the knee. However it was hard to give it a size because it was not a nice circle. It grew with about 2 cm at October 2015. From then on I don't know the exact measurements, but it grew more till I started with Cediranib. Like I said, right now it looks like the size it was in 2013 or even smaller. We didn't spoke of recection of the primary, in my opinion it is better to go on with the trial right now. Probably if the tumor starts growing again (which I do not hope for sure!) it is a good time to evaluate that. In 2013 they decided not to resect it due to the risk of complications and the uncertainty in the benefits of resecting.

Willem

[D.ap]

Willem
When we tried a TKI, we had the opposite effect ..progressive disease .
Joshua also was down to 98lbs.. mid 2016. 6 months after and discontinuing sutent.

That is 40lbs less .than his today’s weight, ( after 31 treatments of Opdivo. 18 months?)

Your lung tumors are still over 1cm?

Opdivio that Josh is on , has very little side effects and has reduced his tumors by 70%!

http://www.cureasps.org/forum/viewtopic ... 1297#p9919

Also with your primary in place , I suspect your weight will return very slowly .

Are you at your optimum weight ?