Cure Alveolar Soft Part Sarcoma International (iCureASPS)

I am new to this forum and I must say that its so therapeutic to just have an outlet to share ideas and experiences. I am not new to ASPS as I was first diagnosed in 1988 with a spinal tumour that turned out to be asps. I was 18 at the time, getting ready for fresh week and leaving home for a great experience at university. Sadly my life changed forever as many of you are aware of the shock of having such a rare disease. Somehow they managed to remove the tumour followed by months of radiation. I was young and fearless but still scared beyond belief. I made it through with support from family. 26 years later having let go of the fear of moving forward and having a family I was so unpleasantly surprised to find another tumour, this time in my abdomen. I had put this behind me forever I thought, got married and had two wonderful kids. This can't be happening, but it is.

We were able to remove the primary mass in Oct 2014 with a full resection, the post op did show malignancy in 1 localized lymph node. On Dec 1st I had a follow up CT that showed metastases in 4 nodes of approx 1 cm. The folks at the cancer centre basically brought me in and told me that there is nothing that can be done . No Chemo or radiation. They did offer me a clinical trial using Sinutabin and another drug. I am still confused as to why my options are so limited based on their recommendation. I am not accepting the news as I feel that there must be something that can be done. Of course I hope that the mets in my lungs stop growing or remain very slow.

Does anyone know if its possible to make a diagnosis of the mets being untreatable based on a CT. Wouldn't you need to do a biopsy.

I don't want to give up as I am otherwise healthy and in good spirits.

Hi Mark,
welcome to our forum.
Your story is very interesting as it is very rare we meet ASPS patients with such a long time between the first being Dx, then the recurrence so long after that. Although I have seen the cases like that described on the Pubmed.
Where are you getting treated? The position of your drs is somehow surprising to me since they are aware of that your ASPS mets are slow growing and very limited in number - it opens so many treatment possibilities now. The diagnosis of ASPS mets could be done based on the CT scans alone and we actually advocate not to have the lung mets biopsied - the lung mets are very frequent in ASPS and you should just have another consecutive scan to see if the suspicious nodules have grown during the time interval in between the scans - make it like 3-4 months at least as otherwise it will be hard to say. In the meantime just live your normal life.
Another scans that need to be done are brain MRI and bone scan to check if there is anything else.
If these nodules are mets, and there are only few of them, you can eliminate them with the cryoablation (freeze out), radiosurgery (burn out), RFA (also burn out) or to have a surgery by Dr.Rolle in Germany where you will have to pay your own money but it gives you an option to have this amazing dr to palpate all your lungs to find any mets that could be missed by the scan. We've been trough all this treatments and have first hand experience with them so you will be able to use our collective experience if needed. We - I mean all of us here - share what we do and with what result - for our own benefit so we are not left without options when there are many available. Systemic treatment options should be reserved for the cases when there are no local treatment options left as the drugs usually only work temporarily until the resistance develops.
Say hello to your family from our!
I am Ivan's mom, read his story in the personal updates.

Hi Mark

I am sorry you had to revisit your alveolar soft part sarcoma with another episode of surgeries
Are you here in the states?
As Olga has said your doctors should not be so quick to throw their arms up. That is totally unacceptable in my book.
However I am really glad you were able to remove the sarcoma with good margins . Good for you and the doctors involved. Hope you are feeling better with each day
Josh is our son. Primary dx in 2012 with lung mets and 140 out of 5 plus still remaining

All my love
Debbie

Josh
http://www.cureasps.org/forum/viewtopic.php?f=4&t=750

Ivan, Olga's sons link

http://www.cureasps.org/forum/viewtopic.php?f=4&t=66

Hi Olga and Debbie

Thank you so much for your support, it has been much harder this time around, when I had my first ASPS finding I was only 18 so a lot more fearless. I am very interested in Dr Rolle to see if he can help me, I live in Toronto Canada. Everyone keeps telling me to go to the States but from what I am reading ASPS has had good results with Dr Rolle.

Is there any information about the costs and overall experience.? My Oncologist in Toronto is running the clinical trial that is using Sunitinib and Cediranib but I don't know if going on the trial will exclude me from pursuing an option like Dr Rolle.

Again, I just wanted to say that this website is so helpful, thank you so much.

AM

Mark, you just forgot what you felt the first time around:)
We live in Vancouver, BC. Ivan was first Dx with lung mets in 2004 and they were very multiple. We choose not to go with the systemic treatment as they (usually) only work temporarily - both Sutent and Cediranib and when/if the resistance develops then you find yourself with the mets that grow faster than before.
Ivan has been trough the surgery with Dr.Rolle 4 times, as his multiple lung mets neded 2 rounds of the clean up. We had no other treatment options. Now their number is reduced to a single digits that we manage with the cryoablation - and yes, we go to US - Detroit - as the best cryoablation dr works there. He has developed this technology a long time ago and although the cryoablation is avail elsewhere now - we even have it locally in Vancouver - Dr.Littrup in Detroit is head and shoulder above of everyone else as he does this procedure for about 10 years.
In your case you have a choice between Dr.Rolle and Dr.Littrup before you need to start thinking about any drugs. Laser assisted surgery is better done on a clean non damaged lung and Dr.Rolle usually finds a lot of mets that are not seen on a CT scan, but on the other hand cryo ablation is a less painful procedure avail. in 1 hour flight from you...about $12,000 Euro per a lung is a cost of surgery in Germany (at least it was before) and about $10,000 USD is a cost of one cryosession in Detroit (sometimes they can do 2-3 mets in one lung in one session). With the surgery you go to Germany for about 3 weeks, each lung is done separately so if the mets are located in 2 lungs you will need to go twice with an interval of few months in between. Dr.Rolle is a leading thoracic surgeon in Saxony province and a very specialized surgeon in a laser assisted field. I am not at all surprised that this surgery is not avail. in Canada or US as it is a very different type of surgery when the already trained surgeon would need to stop operating and go to Dr.Rolle to learn this new skills. Unfortunately he is going to retire soon, may be next year.
reading
Laser assisted surgery:
http://www.cureasps.org/forum/viewforum.php?f=51
Cryo:
http://www.cureasps.org/forum/viewforum.php?f=55

You have to get some understanding what a clinical trial is. When the trial is conducted it means they do not know yet if the treatment able to extend the patient's life (OS - overall survival). Besides they now have a new approved goal they can test - not the life extension but PFS - progression free survival, when the overall survival is not tested but for how long the disease growth can be stopped. I have seen studies results when the PFS was increased but the OS was decreased when the disease grew faster after the resistance developed. In some cases the better RFS leads to better OS but not always. For us it means that a clinical trial should be looked at only if there are no other options left. With ASPS the life expectancy is long, so we have to play wisely.
I think that RFA - another type of ablation - is avail. locally in Toronto and may be even cryo, I am wondering why it was not offered to you?

Hi Mark

"We were able to remove the primary mass in Oct 2014 with a full resection, the post op did show malignancy in 1 localized lymph node"

How was this determined?

A biopsy or scan?


The primary was where and how big?

Dr Rolle will need to know these incidentals as well I believe


Debbie

Hi.

Yes, it was a full resection of the primary which was approx 10 X 8cm located just under my rib cage on my left side. Post op they told me that out of 7 lymph nodes 1 local showed malignancy.

From what Oncology has told me there seems to be 3 mets approx 1 cm and 1 slightly more then 1 cm. I am trying to gather all of the info required for Dr Rolle. I hope that the size of the mets don't exclude me from consideration for the laser. I feel like I am running out of options and time. At the same time I am trying to manage the heightened anxiety that this is causing, fun times for sure. !!!!

Thanks
Am

Mark

We all know that anxiety believe you, me!
Have you had a Lung scan performed yet since your surgery ?
If you have get a copy or have your doctor forward a copy to Dr.Littrup
Call first and talk to I think Barb and see what else she might need
It doesn't hurt to ask

As Olga so graciously offered, ASPS has a history of slow growing but taking care of it surgically should really be considered first and foremost.

You are doing a great job
I hope after a good nights sleep things , life feels a little better.

Dr Littrup info
http://www.cureasps.org/forum/viewtopic.php?f=55&t=835
Love
Debbie

Thanks so much Debbie, I am learning so much through this journey. I am in the process of getting all of the info for Dr L in Detroit as well as Dr Rolle.
I am hopeful for a miracle or even a few more years with my wife and kids. I am so thank full for everyone on this forum and I pray for your families.
Am

Mark,

As we are appreciative of you !

Set your sites to grand kids however cherish everyday as I feel you already are seeing

You are part of our community As Olga has said "not a club you want to be a part of "
In other words not your choice but let me assure you we welcome you with open arms
Please feel free to PM any of us anytime
When time OR energy allows, we will respond .

Our love
Debbie

Ps Prayers are welcomed in my world always In all shapes and most forms : )

mark2014 wrote:Thanks so much Debbie, I am learning so much through this journey. I am in the process of getting all of the info for Dr L in Detroit as well as Dr Rolle.
I am hopeful for a miracle or even a few more years with my wife and kids. I am so thank full for everyone on this forum and I pray for your families.
Am

Hey Mark
How you feeling and doing?

We will be possibly pursuing the Cediranib/Sutent trial here in the states.

How about you?

Where you at in your care?

The Josh P family

Dear Mark, My name is Bonni Hess, mother of now 32 year old Brittany who was diagnosed with ASPS 13 and a half years sgo at age 19 and continues to courageously fight this insidious disease now in a Cediranib Clinical Trial which has very thankfully provided her with sustained disease stability and dramatic shrinkage/disappearance of her innumerous and widely disseminated mets for the past almost six years which is proof that there IS Hope and there ARE some promising treatment options available unlike what your doctors inexplicably/inexcusably told you. I am so very sorry for the resurgence of your disease after an incredible 26 years of disease stability and apparent NED, and I am sorry to be so slow in responding to your initial post which I somehow missed during the busyness of the Holiday season. I Hope that by now you have been able to pursue and obtain treatment for your lung mets, and that you are doing and feeling well. I Hope too that you will post an update soon about your treatment situation and that you will be an active participant on this Discussion Board which is an invaluable source of shared researched and anecdotal treatment information as well as strengthening support and encouragement from those of us in the ASPS Community who share this difficult battle and journey with this challenging and, as your case demonstrates, very unpredictable disease. Take care Mark, be very pro-active and as well informed as possible, stay strong, let Hope guide you through each day, know that you aren't not alone in your renewed battle with this extremely rare disease, and keep in touch as you are able. With special caring thoughts, healing wishes, and continued Hope, Bonni Hess

Hi Mark

I share in Bonni's words and hope that everything is going well in your life and you are finding answers.
Write when you are able.

Love
Debbie

Hi Mark,

I'm Martin and I'm the last that joined the forum. However you did had battled this disease long time before I even knew it existed.

I'm very sorry for your diagnosis but I'm sure that you will find access to a lot of doctors that actually know what they are doing and hopefully you'll beat it once again.

Basically I was lost almost 2 months ago when i got the diagnosis but a lot of people here and their experience helped me in getting the right things and decisions done. We are all here to support each other because the battle is ours. I know you will find a way through.

best wishes I will pray for all of us every day.

Hi Mark and all,

This is Anita from India . My son was diagnosed with ASPS with his abdominal muscle in 2011. He was all of 16 then.

He has multiple subcentimetric nodules in both his lungfields not sure to be mets . They have reduced in size and number in last two scans and is veing evaluated on yearly basis ( from CT to X Ray now ).

How are you doing now. Can you update us on your treatment and progress please .

Wish you and everyone the best .
Regards
Anita