Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Dear everyone,
I am a mother of 13 years old son “P” from Thailand who was dx ASPS on March 2016 at his back of right knee at 4cm x 3cm x 3 cm. The tumor was resected with clear margin 1mm due to limitation of surgery ten follow by RD.
In March 2016 => CT chest-scan = No lug met, PET = No met in other parts.

But....
In Oct 2016 => CT-scan found about 10 mets size 2mm -4mm in his lungs.
I have sent PM to Olga for supporting information and suggestions that I got very well quick feedback from her and very informative.

This case in Thailand is extremely rare and difficult to manage. I would like to have information, suggestion from everyone for this battle. I am in a dark world now..

Yesterday, we met Onc doctor that his plans as below.
Surveillance
1. CT-Chest in Dec
2. MRI- Brain in Dec
3. Bone scan in Dec.
Biopsy and surgery
1. He suggested to Biopsy from biggest 4mm in Lung to make sure this is ASPS or not. The technic will be VAT . Shall we have this Biopsy?
2. Or I shall waiting for next 2 month if met grow? Do we need Lug Biopsy?

If confirm ASPS in lung
1. He suggests for Chemo or drug (TKI) as systematic treatment.
2. Seeking surgery for lung met in Thailand or Other countries.

Dear all, if you have any recommendation or any input please kindly feedback to me.
I am very appreciating Olga for her always supporting.

Thank you everyone…
Phura.

Hello Phura,

I'm very sorry for your sons diagnosis,however it is good that you have found this forum. I'm ASPS patient myself diagnosed almost 2 years ago.

According what I have read till now I would not recoment doing a biopsy as that is the way rhat usually ASPS developes.

Chemo has shown that is not effective with ASPS.

There is a hospital in Germany that few people from this forum have used with a method that operates the lungs with minimal destruction of good tissue but if this is available in your part of the world would be much better.

I'm sure that very shortly you will get more info from the people on this forum

Phura, hi.
1. Re. biopsy. I do not see any reason to do it as it will not change the treatment plan and will complicate the surgery by scarring. ASPS almost always metastasizes to the lungs first, so just wait for the next scan and see - if they are growing and there are more became visible from the micromets state, they are ASPS. Even if they are not ASPS they need to be treated. If in doubt, request re-review of the initial primary pathology results.
2. Re. Chemotherapy and other treatments versus surgery.
ASPS is a very rare disease so when the studies are done, the treatments are very non-uniform - people get different chemotherapy regimens in a different circumstances, and most often they get the chemotherapy treatments for the unresectable metastatic disease. In our understanding, ASPS is not able to kill bigger ASPS mets so even if there is some response, they will come back eventually. But there are ASPS cases when the bigger mets were resected by the surgery and the micro mets were killed by the chemotherapy - we have few people here on the board who had different types of the chemotherapy and the surgery to remove their bigger lung mets and they are living the normal life 10 years after the treatment. They had some single cases of the mets removed paler in their life but in general in appears that their micromets were mostly killed by the chemotherapy. So I disagree with the blank statement that chemotherapy is not effective in ASPS, it is just used in the situations when it is not effective.
I already sent you this link in a private message:
https://www.ncbi.nlm.nih.gov/pubmed/?term=15087950
Alveolar soft-part sarcoma responsive to intensive chemotherapy.
you will see the abstract of an article there:
but if you can not open it, go to the Pubmed.gov and make a search by PMID: 15087950
I contacted Dr.Nickerson and he said that the girl is till doing just fine.
Discuss this article with the oncologist.
What is the difference between the conventional chemotherapy and TKI. They are different types of drugs. You can read on the internet about it.

My son had 4 laser assisted lung surgeries in Germany, 1 brain surgery in Vancouver, 1 pancreatic surgery in Germany and close to 20 lung cryoablations mostly in US for newly appearing lung and other mets during 13 years with ASPS and he still have numerous micro mets. 2-3 treatments per 1 year.

The surgeon can only resect what he can feel - the mets that are bigger than 0.5 mm. The smaller ones are still there and will grow as the times goes. The hope of the chemotherapy is to eradicate the micro mets. We had few cases when it was successful, when the chemotherapy was dome together with the surgery to remove the bigger mets and people living now normal lives 10 years after the surgeries. Bigger ASPS mets can not be eradicated by the chemotherapy, they have to be resected.
But we also had cases when the chemotherapy was not effective.
With my son - I would do it differently now and try to eradicate the micro mets by the chemotherapy first, have 2 courses only and then go for the surgery so they can analyse the smaller mets when resected, if they got killed by the chemotherapy. Then we would decide if to do another 4 courses or not depending on the result of the analysis.
But the chemotherapy is damaging and hard and often does not help.
Alternatively you can start with the surgery as we did and just wait for the cure to be found as we do, but the surgeries are very damaging for the organs as well, scarring affects their functions.
TKI are usually only effective temporarily when they work at all, then usually resistance develops. Our member from China told me that they have 2 cases of the complete response to TKI called sunitinib and another one called pazopanib but the mets came back 2 years later.
Discuss with the oncologist but eventually you will have to decide yourself. I would wait for the next CT scan in any case to see the dynamics.

Hi Olga,

I have asked Onco via email that he said the procedure will be Video Assisted Thoracic Surgery (VATS) as surgery will not do as Biopsy (I am not sure if I missed understand), but capsulate all possible nodules. Then send it to lab phathology. =>
1. Is this step need to be done or not? Or
2. just wait for next CT?
3. Or shall be done after CT-next 2 months.

Ref. Onco said that he has to make sure that is ASPS before proceed Chemo or TKI and he said that we will also so wait for next CT too.
Then Chomo/TKI might be started in Dec or Jan.

After review all information in articles and in Web-board that I am considering if we shall start with Intensive Chomo instead of TKI . I am not sure that Onco informed about TKI (Sunitinib 78,79 Cat 2B) which is available in Thailand.

Pease kindly let me know between Intensive Chemo or Sunitinib?

and Please recommend about this plan?

Again thank you very much to Olga as always quick supporting for my discussion with all doctors and very useful information.

And thank you in advance for all recommendations...
Phura.

Can you please clarify this:
is the oncologist proposing to use VATS to remove all the visible lung mets? Does he want to wait with it till the next CT scan and do a VATS surgery after the next scan or right away? Has the thoracic surgeon already reviewed the scan and thinks that all the mets can be removed by VATS?

Hi Olga,


Answer:

Q: is the oncologist proposing to use VATS to remove all the visible lung mets?
Ans: As per CT scan , Onco said that only 1 met (4mm) that near lung surface which is can be removed by VATS , then sent to Lab. for check if ASPS. Tomorrow, we will meet Surgion who will do VATS for confirmation about this procedure because Onco is not expert as surgion ten he cannot answer about technic. However, he said that VATS cannot clear all lung met by this technig as he understands. I informed him about LAPM in German that he also willing to contact the doctor in Cowig.

Q: Does he want to wait with it till the next CT scan and do a VATS surgery after the next scan or right away?
Ans: He said that depanded on our choices, we have time to decision and for VATS is also waiting for evaluation too.

Q: Has the thoracic surgeon already reviewed the scan and thinks that all the mets can be removed by VATS?
Ans: Tomorrow, we will meet him in the first time due to the Onco just refered my son case to him and then we will consult him about this. But for VATS is more lost of Lung tissues? We also will consult the surgen about LAPM in German too.

Pls let me know , you recommendations.

Always thanks...

If they are only talking about taking 1 nodule for the test to verify if - it is ASPS, then I would not do it. Just wait for the next scan and if there is a growth in sizes and numbers then it is ASPS. ASPS is almost always metastasizes to the lungs first. No need to inflict the lung scarring just to prove it, the clinical studies are already done and it is a known fact about ASPS. If VATS is done, there is going to be a scarring and a bleeding on the surface of the lung and the best chance for the laser assisted surgery is lost. And yes, there is more lung tissue lost using VATS as they can only make it staple assisted resection - cut the piece pie shaped, roll the edge and staple it. Not that significant when the only one nodule is resected located on the surface though, but just does not make a sense. If you go for the surgery in Coswig, they will test the resected nodules and you get the report to bring it home.
Meet with the surgeon anyways. It is necessary to have a good supportive team locally. They are needed after you get back from the surgery, to perform and read scans and to treat the possible complications.

Dear Olga..
This is always valuatble information!!
We will keep you update for tommow result.
Many thanks....

Hi Olga,

For your updating.
I just got CDs from 2 hospitals for 1st CT-lung scan in March, 2nd Scan in Oct. I am preaparing to send to Coswig hospital and confirming address with Dr.Drewes's secreatry.

Thankss..

Hi Olga,

Today we have met Surgeon doctor that he said VATs and open surgery are not recommended due to serveral mets, it will effect to lugs tissues.

Seem.. hopeless..

We are not sure if we shall start with intensive Chemo ? We dont know type of Chemo that shall be. If Sutent is not work.

I have sent articles and link as you provided to him alreday then will meet him for treatment procedures.

To go to German for Thai people, might more complicate than US/EU.. difficult VISA for surery specialy .. anyone have this information?

Pls let me know your recommendations.

It is pretty normal to get this answer from the local thoracic surgeon - all of us who went to Germany for the laser assisted surgery got the same local response. This is why we had to fly to Germany as this type of surgery is rarely available, especially on am expert level. Without this laser tool the surgeon is not able to resect all mets without taking out to much of the lung tissue. We live in Vancouver, Canada - it is a big town with a very good thoracic surgery department but we got the same answer. In some cases you just need this special surgeon that you are ready to fly anywhere.
There is no rush for the surgery, if Dr.Drewes accepts your son, he will give you a letter for the visitor visa. Medical tourism is a very frequent occasion in Germany, not unusual at all.
We had to fight with the local doctors initially too but now they are pretty respectful of our opinion here as they know that we are very carefully reviewing all the options that are there and always look for the evidence based treatment decisions.
This is what you need to learn from the start - any steps that you take in a treatment have to be based on the documented success. If the oncologist is advising to start the TKI (Sutent as I understand), you should respectfully as him to provide you an article or the result of the study supporting his advise. I based our decision to go to Germany on the publications of the articles about the laser assisted pulmonary metastasectomy on the Pubmed. Always use the articles from the Pubmed.gov as they are pier reviewed and not all the articles get published there, unlike an internet in general with all the self proclaimed healers and miracle makers of all sorts.
Also wait for Dr.Drewes to answer. It is not always they are able to do the laser assisted surgery too.

Hi Olga,

I am sending the disc of CT-Chest to Coswig..

May I have your recommedations for below plans.

Plan A:
1. Shall we go for LAPM first after confrim by Dr.
2. Then CT-Chest, MRI Brain, Bone scan in Dec
3. See the result.
4. Future treatment.

Plan B:
1. Waiting for next CT-Chest, MRI Brain, Bone scan in Dec 2016.
2. LAPM at Coswig.
3. Follow up CT-Chest, MRI Brain, Bone scan
3. See the result.
4. Future treatment.

Plan C:
1. Waiting for next CT-Chest, MRI Brain, Bone scan in Dec 2016.
2. See result, if not OK => Chemo or TKI (Sutent), 2 courses.
3. LAPM at Coswig.
4. Follow up CT-Chest, MRI Brain, Bone scan
5. See the result.
6. Future treatment.

In the mean time, do you have suggestion for nutrition? shall go for alternative medical?

Again many thanks...

I am sorry, but I can not give you a clear recommendation. I expect that Dr.Drewes is going to wait till the next CT scan as well to see the speed of growth and if there are more mets appear.
The chemotherapy question is very difficult one and there is no known answer. If it works and kills the micromets, then the problems with it are justified. But if it does not work, there are very grim consequences - the immune system is affected and the vascularity is increased which may lead to increased speed of growth in the smaller mets that are going to be missed during the surgery. If going with the chemotherapy I would have 2 courses before of the surgery to be able test the smallest resected mets and see if they are dead or at least partially necrotic. Then it makes a sense to go with another 4 courses. Have you discussed an article of Dr.Nickerson with your oncologist?

Hi Olga,

1. The CT-Chest scan will be Coswig by This Friday Nov 4,2016. The doctor said that he will review as soon as possible and return answer to us.

2. An article of Dr.Nickerson was send to our Onco and we will meet him on next weekend. He is very active for this case, learning and kindness also willing to contact Dr.Drewes too.

3. As we have checked in personal story , a kid also response for "interferon alpha-2b"
http://www.cureasps.org/forum/viewtopic ... 7&start=15, we also will discuss with him for this, also TOK case.

4. As your recommendations to review Sutent/Sunitinib that seems did not success in any case. I dont know if any success on this TKI?

Serveral benifit information from this forum!! I hope all parents, and kids success quaility of life.

Sorry for always asking..Many thanks for your time.

I would like to have also sugestion for what kind of food, nutrition for kids to prevent any new met..hope.. if anyone have please kindly send me a link or suggestions.

Thank you very much.

Dear Phura,
I am so very sorry for your young son's ASPS diagnosis, but am grateful that you found your way to this Forum and have reached out for information, input, advice, and support from our ASPS Community. Olga who is extremely knowledgeable about and experienced with ASPS, has provided you with some excellent information and advice. I completely agree with what Olga has said based on my 15+ years of ASPS experience and my extensive research, observations, and networking with other ASPS patients and families during our daughter Brittany's difficult 15+ year battle with this extremely rare and very challenging disease. I strongly agree with both Olga and Martin that a VATS surgery to remove just one lung met to definitively determine if your son's lung mets are actually ASPS is an unnecessary procedure since ASPS almost always metastasizes to the lungs, and the procedure would only create scar tissue, possibly cause a spread of the ASPS tumor cells into the blood stream, and subject your dear son to unnecessary pain and suffering. As Olga has said, following the lungs and other parts of the body with appropriate regular scans is the best way to monitor this disease. I am grateful that a brain MRI and bone scan have been scheduled for December in addition to a follow-up chest CT, but I strongly encourage you to also request a pelvic and abdominal scan
(preferably an MRI to prevent radiation to the pelvic abdominal organs, but a CT with contrast if an MRI won't be provided due to the higher expense of an MRI). It is essential to have regular complete scans to adequately and successfully monitor this disease since even if there is no increased growth or disease progression in one area of the body, it may occur in another area as we discovered many years ago when Brittany's regular scheduled chest CT inadvertently showed a met on her liver which otherwise would probably not have been found until it became symptomatic when it would then have then been too big and too late to successfully treat. Being very pro-active, as knowledgeable as possible, and vigilant with regular and appropriate scans is extremely important in fighting this insidious disease. Regarding your questions about chemotherapy or other systemic treatments, I personally feel, and I think that Olga agrees, that systemic treatment should be reserved for when there are no other treatment options available to remove or destroy mets such as resection, ablation, or radiosurgery because if a chemo or other systemic treatment fails, then you may have no further or very limited other treatment options. This was the case when after eight years of fighting this disease with 23 resections/ablations/radiosurgeries Brittany developed an unresectable/untreatable/Life threatening met in the head of her pancreas so we were forced to seek a systemic treatment to try to destroy the met and stabilize progression of her disease. Through invaluable information graciously shared on this Forum by an ASPS patient from England, and with extensive research we found a Cediranib Clinical Trial in Edmonton, Alberta, and VERY thankfully the Cediranib has now provided Brittany with seven and a half years of disease stability thus far, and it appears to have destroyed all of her innumerous and widely disseminated scan visible mets (you can read about Brittany's Cediranib experience in the Cediranib topic on the Discussion Board). As with everything with this very rare and poorly understood disease, there don't seem to be any definitive answers so these critically important treatment decisions need to be made on the basis of being as knowledgeable and well informed as possible. Regarding your question about nutritional recommendations and advice, in my personal experience and extensive research and observations, diet and nutrition unfortunately do not positively affect nor prevent progression of the disease and development of new mets, and there is some data which indicates that nutritional supplements can actually strengthen the cancer cells as well as the normal cells, and can therefore cause disease progression!! It is important that you discuss any nutritional supplements with your son's oncologist, especially if your son is on a systemic treatment for which there may be adverse consequences to eating some specific foods such as grapefruit. I know that you must be overwhelmed with all of this information and everything that you and your dear son are dealing with right now dear Phura, but stay strong, stay pro-active, know that you are not alone on this difficult journey, let Hope lead you through each day, and keep in touch with this Board as you are able.
With special hugs, caring thoughts, healing wishes for your dear son, and continued Hope,
Bonni Hess, mother of now 34 year old Brittany diagnosed 15+ years ago at age 19 in July 2001