Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello I'm Mania from Poland. My sister is 23 years old, her name is Paula she was diagnosed with ASPS FEB2009. She has a very large tumor 12cm in the right iliac region. Doctors first plan was to remove tumor by surgery. I don't know why they had to cut Paula to say this: "Planned operation was abandoned due to necessity to increase the extent of the surgical procedure to hemipelvectomy and lack of patient’s consent to it. " this is what the hospital documents said. As you can guess hemipelvectomy is a hard choice especially when you don't really know other options and when there is no plan.
What we were told that there was no other option than surgery to treat this horrible cancer.
Polish doctors seem to have no plan of treatment. Are they are helpless? we keep asking.
They agreed to put Paula on SUTENT treatment now. After one month they want to make CT scans and see if it's working. It's considered as experiment since SUTENT is not an official medicine refunded by our government.
Options in Poland seem to be very little and chaotic, everything takes too long, doctors are too busy, or don't know what to say.

Since the diagnosis was very difficult and took ages here, we were put in contact with pathologists from Boston General Mass Hospital in the US. And once the diagnosis in Boston confirmed the one in Poland, we were told that the tumor should be excised with negative margins and removal of the tumor will be very challenging and should be done by highly experienced sarcoma surgeons.
and that dr. Francis J. Hornicek is an excellent surgeon in General Mass. If no one is brave enough to do the surgery he comes to action. I guess that will be our case. First the Tumor should be reduced in size.
I am more then curious how that can be done? Sinse ASPS is resistant to chemo and radiation?
In all that chaos we had another strong punch in the face - CT scans (done on the beggining of Sutent treatment) showed mets in lungs - 3 visible (not larger then 9mm).
Now a bit about Paulina. She is wise, beautyfull, happy girl, got engaged just before Dx. She is so strong and
believing she can fight and win. She is in good condition. Failed surgery made her worse , but she is
almost back in shape, only with pain, only with the concious there is this big JUNK inside her.

We are a bit lost as a family to deal with all this. I'm happy to find this forum
It's very encouraging to read about all of You being so brave and believing.
We are saying hello.
Mania+ Paula

Welcome to the forums Mania and Paula
I am very sorry Paula that you were diagnosed with ASPS *hug* I was also three months ago.
I read the post a few times and am trying to think of a way in any way i can help and i have a question...
Is the doctors you are seeing sarcoma specialists? This is very important! If they are not i will call my doctor on Monday and ask him for a referal in Poland and post what he says to you. In the USA we have airlines that help send families to doctors in other places around the world for free. Do you have this also in Poland?

I am very new to ASPS and seek a lot more information and care from the regular posters then i can even give back on this forum still.
But the others that made this forum are wonderful and know a LOT more them i do! I am sure soon you will be receving posts back from them

With hugs and hope...

Amanda R

Hello
Paula had the best polish medical crew taking care of her - sarcoma specialists in Marie Curie Oncology Center in Warsaw. The doctors are rally great. But
because of horrible unfixed healthservice system there is very little money going for cancer treatment from the country budget,(from the European Cuntries - Poland is at the end of the list , that's why there are very low survival rates concerning cancer patients). With our doctors - It's like with great sailor on
poor boat, his chances to fight an ocean are smaller than on a big strong ship, right?
ASPS is rare enough here, I guess doctors had only few cases such as Paula's, maybe none-wIth such big tumor. They are ambitious and it is hard for them to say - sorry we don't know what to do.

I wish he had airlines helping send families to doctors in other places around the world for free like You do. there is no such option here. Although our family started fundraising, and if there will be any hope anywhere in the world we'd fly there. For now - Boston Generall Mass gives us hope. We'll see if it's for real, not only our wishfull thinking.
I know I sound very bitter here, it's just I wish we were sure we had anyone who was a good guide. Now there are so many new things, and the clock is ticking.
BUt we are not loosing our faith in winning this difficult fight!
mania

Dear Mania,
I am so deeply sorry for your dear sister's ASPS diagnosis and for the difficulty and frustrations which you have had with the available medical care in Poland, but I am so grateful that you found your way to this Web site and that you have reached out to the ASPS Community for information, advice, and strengthening support. It does sound like the doctors are trying, but are handicapped by lack of medical funding, although heartbreakingly, there is currently no miracle cure for ASPS no matter where in the world a patient is receiving medical care. Because ASPS is such an extremely rare and poorly understood disease, it is vitally important to be very pro-active, be as well informed as possible through research and networking with other ASPS patients, make only informed treatment decisions after exploring all options and researching all of the available treatment data, and seek the most knowledgeable doctors with expertise in treating sarcoma, and preferably ASPS. It is also very important to not just accept one doctor's opinion or recommendation, as doctors can and do make mistakes. It sounds like you are trying to do all of these things in spite of the geographical and financial obstacles which you are dealing with. Hopefully the Sutent will be effective in shrinking the extremely large primary tumor, but in the meantime,the highest priority right now seems to be to find a surgeon who is willing/able to resect it as soon as possible. Either Dr. Francis J. Hornicek at Boston General Mass or 'F''s excellent recomendation of Dr. Frederick C. Eilber at UCLA sound like good resources, and should be consulted immediately. Paula is very fortunate to have such a devoted and caring sister to help her with this very challenging battle. My heart breaks for what I know that you, Paula, and all of your family are going through, but please know that you are not alone and that those of us on this Forum are here to try to help and support you on this difficult journey which we all share. Let Hope guide you through each day, and please keep us updated as you are able.
With special caring thoughts and continued Hope,
Bonni Hess, mother of 27 year old Brittany diagnosed in July 2001

Dear Mania,
I am in Germany right now with my son just had his 4th lung surgery done by Dr.Rolle in Dresden and I want to tell you not to assume that the best surgeons are located in US. They do have really good surgeons but other countries do to and I even think that there are great abdominal surgeons in Germany, great sarcoma oncologists and surgeons in Italy and may be in Poland itself and you should contact a few of them as well to see what their opinion is, they all are different. The surgery in US is a very expensive - it can cost 30-40,000 dollars or more with one day staying at the hospital cost close to 2,000 and in the intensive care unit up to 3-4,000 dollars.
On the subject of the surgery itself, and hemipelvectomy proposed, we have a few people here on the board with the similar location. None of them had a hemipelvectomy and most of them had it resected with the close margins or positive margins, some of them had an introoperative radiation. Actually when the lung metastases are found, this radical resection is rarely offered as there is a little hope that this radical mutulating resection will be able to render a patient cancer free as it has already disseminated. I have a link to an article somehwere and I will send it to you when I get home that sarcomas rareöy invade the close located organs or compartments.
The strategy to try to shrink the tumor is a good one, ASPS is found to be sensitive to one type of the radiation - neutron - there is a facility that does it in the US close to Boston (it was about 25,000 US when I aksed 4 years ago) and there is a facility of this type in Europe, I will find it later on this week when we get home and post here. The one in Europe accepts patients from all the Europe. Also the embolisation can be done,

Hey
Thank you all so much for the advises. I will surely do as you suggested.
Any extra option then we planned is like a new battery - gives more power and strength and faith. thank you for that.
We will be happy to receive any links or addresses or information regarding asps specialists.
'F' - You were very lucky to meet dr.Eilber and avoid this horrible surgery hemipelvectomy (it's so hard for me to even write this name).
I hope we get some luck as well. I will send the CDs with imaging to him for sure.
Olga, I will try to search and ask about neutron radiation in Europe, but if you come across any news about this facility I will be very gratefull to You.
You are very right great doctors are everywhere. We are just so new to this, we are clutching onto anything that gives us hope. Somehow it happend we
got connected to GeneralMass in Boston - and everything seems to match so well - we are hoping it's the best option. But all of You are so right, there has to be second opinion. I hope thanks to You we can get some new opitons of help for Paula.
I'm very gratefull for all Your posts.
We are sending lots of spring sunbeams straight to your great hearts.
Mania

Hello
We have a big question:
Here is shortly the situation
We need for our polish doctor resulst of VEGFR, PDGFR, C-kit, EGFR. Since all of the tissue from biopsy is in Mass General, we are asking for
Results of these receptors to be made at Mass.
It took some time to find out that:
GeneralMass does cKIT and EGFR - they do not do VEGF and PDGF.
They will be happy to send a parafine block to another institution if I can identify one to perform these latter 2 stains.
(?!?!)
To be honest, I don't know what should I tell them.
Maybe it's best to request it comes back to Poland?
Maybe someone has a suggestion. I'd appreaciate it.
thanx
m

I think that there might be not enough tissue from the biopsy to be able to get the reliable tumor profiling. You might be better doing that after the surgery on the fresh tumor - where ever it will be done.
I have a question about the primary tumor - is there a bone involvement on the MRI scan?
About neutron radiation therapy - there are some articles collected on the LMS web-site http://www.leiomyosarcoma.info/neutrons.htm and this is some link to that facility in Europe http://bnctweb.jrc.nl/page.php?11
I did not reviewed much this treatment thought.
Consult Pf.Vogl from Frankfurt/Main if the embolization of the primary tumor can be done:
http://radiologie-uni-frankfurt.de/cont ... x_eng.html and http://radiologie-uni-frankfurt.de/cont ... x_eng.html he will need to see a recent CT scan on CD that you can send to him after he agrees to review it. Tell him that the goal is to downsize the tumor to allow its resection.

I answered this off list. If Mass General has the block and they think it could be profiled, Clarient labs is a big reference lab in California - and they have testing for VEGF, PDGF etc. For US citizens, Clarient lab testing is usually covered by health insurance (it does routine labs too).

Here is their general page: http://www.clarientinc.com/

The form to send with tissue is here: http://www.clarientinc.com/portals/0/pd ... %20Req.pdf
(Immunohistochemistry)

Truth is - if it is ASPS and big, it probably has a high likelihood of expressing VEGF, I think (based on other profile results)

There is a new article FROM France re. technique to reduce pelvic tumors to allow their resection, you might want to consult them re. Paula case:

[Pelvic perfusion for localy advanced tumors]
http://www.ncbi.nlm.nih.gov/pubmed/19211365

I do know that Dr. Fritz Eilber (UCLA) is familiar with pelvic perfusion for advanced sarcomas - we didn't have to use it in 'K''s case.

Another question came up - about referrals outside the Kaiser system - and in case this helps someone else, it is possible for Kaiser physicians to request an out-of-network referral for rare conditions such as this rare cancer. That would mean a consultation would be covered on the Kaiser plan.

At least - that used to be the case...and when I was practicing at UCSF, Kaiser docs sought second opinions not infrequently...

HI THERE!
Olga, 'F', thank you for information - this is really amazing of You, you are being very helpfull in this hard process of gathering information
before Paula actually decides what treatment options to use. We will show it to any doctor we talk with.
I feel this site is such a big info place, with so many caring people.
'F', TC of Paula and letter with her history is already at Dr. Fritz Eilber (UCLA) office. I don't have any answer yet. But hopefully it happens soon.
I'm counting on his expirience in pelvic subject.
My expirience with clarient company is excellent, thank You for that 'F', Paula's specimen is already being tested in the lab. I can't believe it works so well, and people being so respectful and helpful.

'F',regarding your last post - I'm not sure I understand what Kaiser system is? what should I do check how it works?

Now a bit update about Paula:
She had TC scan today. After 1 month of taking sutent 3 mets in her lugns shrinked from 9mm to 4mm. Primary Tumor(12x9x6) is stable.
Meaning Sutent is working.
We are very happy to hear this.
We are waiting for PETscan results, coming in few days,and
getting ready to fly to GeneralMass in Boston in one week. Doctors at GM requested MRI of pelvic area to see and evaluate the bone marrow. We are hoping they can take away the tumor and put prosthesis for illiac bone, and that quality of Paula's life will be ok. We also wander what
they can do about mets in lungs. We are very interested to get to know Boston treatment plan.
I will be posting more soon.
Summer greetings to all of You
mania

Bravo Mania! Keeping you in prayers-

Sorry .. Never mind Kaiser. Another family contacted me offlist and I got confused. They were Kaiser plan people also getting a second opinion with Fritz.

Blessings

Hello!
We are in boston now, and it was so good to hear that doctors want to do the surgery.
just to remind polish surgeon attempted surgery, but failed saying tumor is unresectable.
i'm very impresed by how medical system works in hospital. we felt we were taken with respect
and great care.
The surgery will be in 2 weeks. it is very chalenging, also because of previous
surgery done in poland wich created a lot of scartissue. Dr Srpingfield from General Mass couldn't understand why polish surgeon failed with
surgery. after analysing ct scans, mri's, pet scan they couldn't see any reason the tumor couldn't be taken out.

Doctors from GM want Paula to stop taking sutent, and then go back on it after surgery. They say it's great it's working,
but it won't shrink the tumor greatly, only a little bit. So surgery has to be performed as soon as possible. Previous surgery and radiation created a lot of
scar tissue which is another important factor complicating surgery. During the surgery there will be intraoperative radiation to protect the ledge sheel. There also
will be postoperative treatment with protonbeams (10 days 20grade in 2 doses).
Surgery has a 50% risk of complication, and 5% risk of amputation of right leg.
Paula will limp while walking. If it happens they have to remove bigger part of iliac bone and her leg will be 2-3 cm shorter.

Mets in lungs showed no activity in PETscan. And doctors say it's crucial to remove primary, and then take care of lungs.
So, well I'm trusting we are in goods hands. Having signs confirming it all around us.
Now we are putting our energy together to raise founds and go and fight!
Best regards to all of You
mania

Hello Mania and Paula
YAY! I am so happy to hear you are in wonderful hands and that she will be having the tumor removed!
You both will be in are prayers and please post back and let us know how she is doing and how you are holding up thru all this.

Loving light to you both!

Amanda R