Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello everyone.This is Sarah from Pakistan,25 years old female.I had been reading a lot in this forum and I can't thank enough to people who made this forum.However,I wish and pray that every asps patient gets cured.
Starting with my diagnosis.I use to have abdominal pain since 2 years,got ultrasounds done but nothing showed up.Finally in april 2017,I got diagnosed with asps.I had around 6cm mass and mets in both lungs.I consulted few oncologist and most of them told that its incurable and gave me palliative treatment approaches.I had been told to get chemo done first.Only one oncologist whom Im following primarily said to have resection of primary and lung mets both.
In may 2017,My primary mass from abdominal was removed with clear margins.Now problem is lung mets.I have around 13 mets.My oncologist wants me to get it resected.Whereas,two oncologist oppose this idea and said to try for chemo first.As the mets can regrow soon after surgery too.So its better to have chemo first to stop them from increasing.Also they're not sure if the chemo would work as asps is chemo and radiation resistant.I know that there's no definitive answer to treatment.But Im very confused regarding which treatment to opt for lung mets.Here in my country(Pakistan)we don't have any sarcoma specialist nor much researches or tarils are done.Im scared of the lung surgery as the damage is too much.I can walk,breathe fine now but after lung surgery my breathing would get effected.Im unsure wether to have thoracotomy first or to try chemo or medicine like sutent,watch their response and then go ahead for surgery.Plus I had been trying to approach Dr drewes(Germany)but haven't got any reply yet.
I would be very thankful if I can be benefited from anyone's treatment experience or help.My wishes and prayers for all fighting this battle

Hello Sara
Welcome to the forum

The section on metastatic disease , which is what you are up against unfortunately , is very typical with ASPS.

http://www.cureasps.org/forum/viewforum.php?f=36

With the removal of your primary comes the arousal of micomets that had separated from it long ago and settled to grow in one of the many possible places that ASPS can appear. Yours is the lungs.
Our son Josh had over 140 tumors removed from his right and left lungs in 2013. 8 months apart in Germany , with lung sparing laser surgery ,with Dr Drew's mentor, Dr Rolle.
I believe the in USA radiologist saw approximately 40?

This is a topic within the metastatic : Lung , section.

http://www.cureasps.org/forum/viewtopic.php?f=7&t=256
I am pointing out this topic as it discusses the micromet topic.
hhttps://www.ncbi.nlm.nih.gov/m/pubmed/2189944/

Consensus has somewhat dismayed folks from chemo as the cost seems not to bring on a benefit , to rid lung tumors in most ASPS patients.Any type of immunosuppressive Med seems to actuate the growth of ASPS.

The usage of TKIs has been discussed and used by many patients . But the important thing to discuss with your doctor is to whether they have some supporting papers to show how it will work for ASPS.

Our sarcoma is a systemic disease with a high resistance to chemo and radiation , as you've pointed out ,and needs to be respected for its persistent but indolent nature , in general .

I believe you mentioned previously of the surgeon operating on both lungs at once ? Is that still you're understanding ? I don't believe that to be possible .
I surmise that you asked them about lung functions returning as from your prior response ?

With the laser surgery in Germany Josh has full oxygen functions and also can go back if needed for laser
surgery . Will your doctor allow that ? Sectioning causes more scar tissue as I understand it so your lung becomes less elastic and consequently can't get the same oxygen volumes?

I would also PM Daniel and ask how he feels after a surgical thoracotomy

http://www.cureasps.org/forum/viewtopic ... =45#p10405

And discuss or PM Nhi about sutent

http://www.cureasps.org/forum/viewtopic.php?f=52&t=1293

Hi Sarah,
welcome here. We have different experiences here. Some people had laser assisted surgery in Germany (like my son Ivan), some rare ones had the long term success with the systemic treatment (Brittany with cediranib, others with the classic chemos ) and only had the cryoablation for the bigger lung mets or incomplete resection of the bigger mets. We even have a member who had only resection of her bigger mets - they have a statement from the surgeon that there were smaller mets noted during the surgery that he did not remove since it was not the laser assisted surgery that would make it possible but the reg. one that would require to cut to much lung tissue, and she is staying stable with no growth in the lungs for years now. There are different experiences but the more common one is that systemic treatment is usually only effective temporarily (all types as of now - incl. TKI like sunitinib (sutent) or immunotherapy) but later the lung mets grow even faster due to increased vascularization in the incompletely killed mets. But most of these treatment plans were performed before the new immonotherapy drugs came to market. We have a topic called ICI in clinical trials. The experience with the ICI is also mixed, with most people developing resistance to it at some point but the experience is very limited and we do not know what would be the typical response. There are also combinations tested to improve efficacy. Even if there are no clinical trials, sometimes these drugs are avail. on off label basic in the country, ask re. Opdivo or Keytruda. There are even the cases when the systemic traditional chemotherapy was done before of the lung surgery, then the mets resected and the patient is surviving without any recurrence for the years after that. Again, it is not typical.

Re. lung surgery. It is rarely curative for the people with the multiple lung mets as there are small that are usually missed (the ones that are smaller than 1 mm during the surgery). But it buys you a time to wait for the more effective systemic treatment to come to market.
I realize that going out of country for the laser assisted surgery might be not possible due to money/visas issues. Laser assisted surgery saves lung tissue. The regular surgery done in a different way cutting lung pieces with the mets in them so more lung tissue is lost and there might be pulmonary reserves deficit. It depends on the location. Find out if the cryoablation is avail. in your country as some centrally located mets could be ablated in place and other located on the periphery could be resected during the surgery?
Re.sarcoma oncologist consultation. There is really nothing new they would tell you as there is no definite answer and your plan mostly depends on the drugs availability in your country and the money for the surgeries available and on the patient's general health condition/decision.

I personally wouldnt start tyrosine kinase inhibitor like sutent, because sutent usually doesnt work forever and after you stop using it tumors can grow quicker than before. Immunotherapy is still not well understood in asps. There is little evidence that immunotherapy works better when disease has higher mutational load and disease burden is higher. This is complete opposite to other systemic treatments as I know. I personally would only use immunotherapy after surgery or ablation can no longer get rid of all your mets. If your disease is very aggressive then treating it with surgery and ablations can be very difficult even impossible. So far your asps doesnt sound very aggressive to me making surgeries and ablations best treatment option available at the moment.

Sara
How big are the lung tumors that are seen ?
You've had a CT scan of your lungs?
Just being at the beginning of your diagnosis of ASPS , I know how scared you must feel and I wanted to say that we all have been where you are at currently .

The articles are sometimes made to make us feel hopeless but please be assured that there is a lot of knowledge in this forum ,created by Olga , Yossi and Bonnie and others, that are here to give us better understanding and consequently , Hope .

Here's some success stories

http://www.cureasps.org/forum/viewforum.php?f=28
Love
Debbie

Thanks everyone for sharing this all.I had been constantly trying to get in contact with Dr drewes but neither Im getting any reply for the mail nor the secretary is available since 15 days.I would probably be postponing my lung surgery as Im not mentally prepared yet I guess.My biggest met is of 1.4 cm.My surgeon is operating both lungs together and he would be doing median sternotomy. Basically Im scared that this surgery is going to be so major and as there isn't any surety that lung mets won't reoccur.So I want to go for less painful method.I don't know how long I can wait as Im not having any systematic treatment nor Im getting any reply from Germany.Its hardest phase to be in.Im trying to get access to sarcoma centres in US too.I had been wondering if Dr Drewes don't take me for laser so I can go to US for lung surgery or not :/

Hello Sarah
I'm glad you are not going forward with the surgery .
Our surgery was performed as an axillary thoracotomy, and one lung at a time .
Have you sent your scans to Dr Drew's office ?

I understand your apprehension with having tumors and feeling like you aren't doing anything to take care of
eliminating them . It's a helpless feeling and we have all felt it , some of us more than others.
The US has great sarcoma oncologist , however no one to perform the laser surgery for multiple mets/ tumors.
Dr Auon can plan for multiple ablations of tumors as your 14mm tumor is a candidate for cyroablation .
He is in the state of Michigan .
http://www.cureasps.org/forum/viewtopic ... =401#p1292
His mentor , Dr Littrup, is in New York State

http://www.cureasps.org/forum/viewtopic ... rup#p10659

It is summer so it is completely possible that Drewes is on vacation. I actually had this problem with my mandibular met. Littrup was in holiday and I was in hurry so I choose surgery here in Finland. Surgeon literally started his holiday after my surgery. Surgery was very difficult so no other surgeon could have done it. I was very lucy. To me sounds like you have no hurry and you can safely wait few weeks or even moths. If biggest tumors aren't growing fast even longer. I had 5 tumors cryoablated by Littrup. Cryoablation for lung mets hurt only a little and I should know I specifically asked as little sedation as possible. You can most likely have general anesthesia for cryoablation if you like. Only downside is that cryo can only be used for max of 3tumors at once. But if your tumors dont multiply fast you can keep up with them with just cryoablations.

Sarah, Ivan has his contacts at the hospital in Coswig, Germany - he will ask if Dr.Drewes received your question. Did you send an e-mail or a post mail?

Dr Drewes just replied.He has asked for my ct and then he'd decide if he can take me for surgery or not.I'll be sending him today.
Basically the cost of this surgery is quite much(15000euros) single lung surgery.Its such a tough decision to make.What I want to know is for how long did people who had laser remained met free? And how many surgries a person can have in a lifetime?
I know that there isn't any surety but Im worried that if I spend most of my money on this surgery,how am I going to seek further treatment in future like cyroablation or even any new drug.Is it possible to fight this disease only surgically? A/c to my analysis which might be wrong,almost everyone surviving 10+yrs has to come on conventional treatment ultimately.I can either opt for Germany or I can try for US for some clinical trial,thoracotomy or any possible treatment there.Because here in Pakistan,there isn't any sarcoma specialist plus the thoracotomy Im offered here is median sternotomy in which both my lungs will be operated together.I want to know if conventional lung surgery is done same way in US too like operating together.Sorry for so many queries.

Thankyou so much Olga.So nice of you for the offer.Its feels so good that people here help each other with best of their knowledge.I had been so disheartened from whatever I've read and dr's told me.Im the only child of my parents.My parents are really old and this news has just shattered them.They are ready to have any treatment they can till the last penny.I so pray for everyone including me to get cured of this disease.Lots of love and prayers

Sarah - Ivan already asked his contact in hospital yesterday re. why there is no answer to you so I suspect this is not a coincidence that the answer from Dr.Drewes came:)
To answer your questions:

1. re. "how long did people who had laser remained met free? And how many surgries a person can have in a lifetime?" - metastatic patients are rarely met free - it usually means the mets are to small to be seen on any scans. Ivan needed 4 to bring his lung mets number to a level manageable by the cryoablations now. But he had hundreds mets.

2. re. "almost everyone surviving 10+yrs has to come on conventional treatment ultimately" - to survive 10+ years our people usually had to have lots of surgeries. No conventional treatment was able to hold them for that long without the good quality surgery. People often had the conventional treatment as a last resort and very often if had a minimal benefit if any.

3. Re. Type of surgery - how is the thoracotomy is done in US? Is depends on the locations/size o the lung mets. Clamshell incision (under ribs both lungs at once) or lateral thoracotomy (between the ribs on one side) are both used by the surgeon decision. The concern is not the approach but the tool used as the laser tool allows to burn small mets on spot without cutting the pie shaped pieces of lungs with the mets in them.

But it was before of the TKI or immonotherapy. May be there is a clinical trial or a compassionate drug program in Pakistan for any of these drugs: Keytruda, Opdivo, atezolizumab? With the limited resources it is very important to carefully spend the money on the more effective way, but really at this stage it is hard to say what way is more effective and provide more life time gained for the patient. may be it makes sense to cryoablate the bigger mets and to try to get into some clinical trial in US (which is pretty expensive too considering the living expenses, flights, and some hospitals will charge the consultation/scanning fees - it needs to be reviewed on case by case what the expenses are). I would not loose a single day though, contact all the interesting clinical trials coordinators and ask re. expenses associated with the trial, also contact the offices of the pharma companies in your country re. compassionate use of the drugs noted and discuss with your thoracic surgeon what amount of the lung tissue loss he expects and explain the alternative laser assisted surgery option to him.
Re. money management in ASPS treatment. As of now, we spend about 25-30 thousands dollars a year, sometimes it is more than 40, we live in Canada and in many cases we go out of the country for the most optimal treatment avail. at the moment. I am really sorry for the people that have no access to the treatments in their country and no money to get it elsewhere and yes, they have to plan carefully as how to spend their limited resources to buy more life time. For Ivan surgeries were pretty good option in buying time but it is not the same for everyone, usually people have some combination and the situation really changes lately with lots of promising new drugs coming to a market, the question is to be able to wait long enough.

Thanks a lot Olga for such a deatiled informative response.Im so sorry that I got late in replying.Had been going thru a lot here.So basically my lung surgery is postponed here.My surgeon also thinks that I should give a try going to US and getting help from there.I have applied for it and I pray that I get it.I'd try going to sarcoma center and would want to know if there's any promising treatment there.In Pakistan there aren't much really available.
Regarding Germany-I have sent my ct scan report but some how Dr Drewes didn't get it.I will try sending its pics to him.Whereas,my surgeon has totally opposed the idea of laser.He says that its almost the same and they are just using laser for burning the met,rest the stitches,cuts everything is same and they are charging way too much.So now I've to probably drop that idea :/
I know that some decision has to be taken quickly and Im unable to take one due to short of options here in my country.Im just waiting for the response from US and if I don't get it then I've to get my surgery done here

Sorry to say but your surgeon´s opinion on laser assisted thoracotomy is not correct.

Sarah
I agree with Mikko
He's in Finland , myself in the United States.
Our hospitals and medical system would be probably
30-40% higher than the Germany laser treatment .

Here's a beginning number if there are no complications
with the surgery , in the USA

This was a little over 3 years ago . 2014
https://www.ncbi.nlm.nih.gov/pmc/articl ... n_sectitle

You will find probably a cost variance of plus or minus 40% as more experienced doctors can charge more for their services .
Also our costs generally start at dollar amount and if there were to be complications -- excess bleeding , collapsed lung , more tumors than appear to the eye? , then more speciality doctors are involved which can equal More money for the surgery .
These are called incidental costs .

That is one of the reasons we went to Germany for our sons surgery . Also laser does not damage the lungs as severely .

Also the Germany surgery has little "hidden" , incidental costs .

Debbie