Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi fellow ASPS fighters! My son sammy was diagnosed last year at age 12 with ASPS.
He had no systoms. We just happened to find the lump on his left thigh. After 3 mos.
ct scans there now seems to be a change in lung mets, so our oncologists are discussing
medicines and or future surgeries. \

I just wanted to personally thank all those who posted past and present treatments.
They will help us in our future decision making!
Blessings,
cindy stokes

I can relate to your story...I was diagnosed at 13 with a lump on my left thigh. I am currently 23, married, and have 2 wonderful boys. There is hope! My first recommendation would be surgery. Remove all you can before it has a chance to grow or spread. I have a few other posts under Sherry's story (I think) and under the herbal treatments. I'm currently trying the Frequensea again and I will let everyone know how my scans go in a couple more months...we hope for more shrinkage. Feel free to e-mail me too.
Sherry

Olga, 'F', and Bonnie,
Thanks for sharing your knowledge with me about ASPS and Treaments.
As for our latest update, The ped oncologist and the orthopaedic
oncolosgist showed our scans to their thoracic surgeons and both agreed
to wait on surgery. However, we were not at those meetings. I do know
sam has multiple nod in both lungs. I do not know the exact number or
how large. They did mention they did not want to risk losing excessive
lung tissue. However, it seems by waiting we will risk the nod getting
bigger so having to remove more tissue?
Before starting the Gleevec, The ped oncol. assured me it would not
keep him from doing a trial or other drug later. Also, wanting info
on Gleevec if it only helps with CMet vs. PDGF and VEGF.
Thankfully sam is still able to play basketball and flagfootball.
Blessings to All,
Cindy

Hi again Cindy,

It sounds as if it is a similar situation we found ourselves in. My husband and I are both doctors so we could read the chest CT scans ourselves and discuss them wih the radiologists and surgeons.

When it looked as if we only had 5 or so, thoracotomy was planned for both sides. The idea was that if all could be resected, some patients can have complete remission. But when the number went up after one side was done, and more lung segments / lobes were involved (17 visible by fine cut CT), suddenly the surgeons said don't operate, but wait. The idea was no longer potentially curative, but palliative. They would only go in and operate if a large tumor were found to be threatening in a dangerous area. All of the nodules on CT were still quite small (most a few mm, all smaller than 1 cm).

The idea of waiting surprised us - because depending on how you think new tumors arise, leaving the tumors in place will only require more healthy lung to be removed in the future, potentially higher total tumor burden (making it harder to respond to medicines), and potentially greater sources for new metastases.

We had a very honest surgeon at Seattle Childrens tell us why she was no longer an operate right away candidate. When we told him about laser resection, he recommended we pursue it - as this approach could allow more lung to be spared and central tumor metastases to be removed. That was how we ended up at Dr Rolle in Germany.

Feel free to email me off list if you have any questions. 'K' also feels well - asymptomatic. We have her run a mile a day on the treadmill and she has no restrictions in PE. Usually lung metasases are asymptomatic until they are dangerous (high tumor burden, eroding into a bronchi etc). You cannot rely on symptoms to tell you how serious the disease is. If there is a chance for complete remission / cure, you don't want to miss it.

BTW, early on in our investigations I collected case histories from the alveolarspsarcoma.net site and copied them into a word document. I just looked at it for case histories mentioning Gleevec. There were 4 patients in the list I made who were tried on Gleevec. One person reported lung met stability for 2 years (sometimes ASPS can do this in the absence of drug), then progression- but the other 3 reported a complete absence of response (new mets and met growth).

Blessings back to you!

'F'

Hi, 'F',
Our cases do sound very similiar with regards to when to do surgery.
Sam's CT scans had been stable for a year. I hope we have not missed
our opportunity for surgery. Both dr.'s want to try systemic treatment.
We have an appt. with the ortho. oncologist first of Dec. so I want
to revisited the idea of surgery. From what I have read, Dr. Rolle is
the only one to do the Laser resection??
Thanks again for your response and hope 'K' is doing well!
Peace,
Cindy

He certainly is the one who has the most experience. Apparently there is a new laser in the final stages of approval by the FDA. But of course then you have to have hospitals buy the laser and learn how to use it.

Perhaps at your next visit you could find out how many tumors there are, and whether they involve all the lobes of lung. You might even ask if the doctor can show you the scans. We are allowed to have copies of the scans made on CD - we just need to wait 5-10 min after the scan and they give it to us. At UCLA getting a copy of the scans cost us $5. If you consider a second surgical opinion, you will need to have a CD of the scans that you can mail.

The other thing to be aware of is that "stable" in clinical trial lannguage can mean 20% growth.

'F'

Just a quick update to say we did start sam on Gleevec.
So far no real side effects. He will have a CT scan
in January to evaluate his status. We will also meet with
his surgeon too. My prayers are with each and everyone
of you this Holiday Season!
Hopeful,
Cindy

http://cureasps.org/forum/viewtopic.php ... =539#p3051

Sun Feb 06, 2011 7:26 am wrote:Just a quick update that my son is still taking ArQ daily for 23months now
and last scan showed stability. For now we will stay on this
path since he is doing so good (high school, basketball, etc.)
Best wishes to everyone in 2011,
Cindy

Re: Website History and Purpose - Jim Hess

Postby jcs2007 » Sat Feb 22, 2014 12:35 pm

I just wanted to take this time to thank all you who have contributed to this website which was so invaluable to me when my son was first diagnosed in 2007. I was so lost and this website gave me invaluable information and most importantly support and hope. My son is still on ARQ trial and a recent radiation treatment to one lesion in the lung which we are waiting on the next ct to see how this met responded. Again, I agree that we are all on the same mission to cure our loved ones.

Hi Cindy

Hope Sam is able to get good results from his lung procedure.
Thoughts and prayers are with you and your family

Love
Debbie and family

Cindy,
hi, could you update your son's story? Is he off ARQ trial now?

Dear Cindy,
I was so sorry and saddened to read in your thoughtful and supportive November 3rd post to Jussi that your dear son Sam apparently developed a met in his cerebellum which was recently removed. I am very grateful that the met was able to be successfully resected and that Sam's balance is improving. My very best wishes are with him for a continued successful recovery and dissipation and complete resolution of the debilitating effects of the cerebellum met and any post op problems. As you may or may not know/remember, Brittany also had a met removed from her cerebellum in 2005, and she very thankfully fully recovered from having the met and undergoing the resection to remove it. I had been so encouraged by Sam's seemingly long term sustained successful response to his ARQ-197 treatment. I am heartbroken and concerned about the development of his cerebellum met and the possible implications that he may have now developed a resistance to the ARQ-197 treatment. Will he be allowed to continue with his ARQ-197 treatment despite the development of a new met, or has he been removed from the Trial? If his ARQ-197 treatment has been discontinued, will he now begin another type of systemic treatment to try to prevent any further disease progression? You are always so kind, caring, and supportive of everyone on this Forum, and I Hope that you know that we are all here for you also to provide strengthening support and encouragement on this difficult journey which we all share. Take care dear Cindy and keep in touch with the Board as you are able.
Reaching out to embrace you with special hugs, deepest caring, healing wishes for dear Sam, love, and continued Hope,
Bonni

Thanks for your kind response. We will discuss the next step for Sam regarding staying on ARQ or trying something new since he had new brain and pancreas met. I had remembered reading about others like brittani and Ivan who had similar metastasis which reminds ASPS patients to be diligent with scans. Also, it was recommended he have gamma knife radiation to the cerebellum as a preventive move so if you have any opinion please advise. Another possible medicine that was mentioned was Pembro so we will know more next week. However, Sam has recovered remarkably after brain surgery without physical therapy so far. Best wishes to all on this board and I will let you know what our next step will be.

Cindy - once again, can you please update us on what the situation was/is with the new mets. What is with the panc met. Was there only one brain met. What was the size.
Ivan had both resected without any radiation. His brain surgeon - the head of the brain surgery here in Vancouver and an excellent dr, said the surgery was clean resection with the negative margins with the mets taken out encapsulated so there is no need for the radiation. Ivan also recovered from the brain surgery very well and fast, may be easier than from the other surgeries he had - although any brain surgery is VERY dangerous.
But the main problem here is that ASPS patients with the brain mets almost never have them single. It means that there are might be some dormant ones that will come later. But they are not the local recurrences on a spot of the previous resection. They are the new brain mets. From now on he needs to be very closely monitored for the brain mets - 3-4 months interval MRI for 2 years and may be 6 months later. Just make sure it is MRI not CT. Also the same has to be done for the panc area - adrenal/pancreas/kidney area MRI needs to be done - if he already had it treated. Had he?

Dear Cindy,
Thank you for the updated information about dear Sam's current situation. I am so deeply sorry that he has now had disease progression to his brain and pancreas, which I know from Brittany's experience is unfortunately not uncommon with this insidious metastatic cancer. I was SO Hopeful that Sam's ARQ-197 treatment would prevent metastasis since he had such a successful seven year response to it, but as we all know FAR too well, ASPS patients can develop resistance to their previously successful systemic treatments at any time. You mentioned that you will be discussing treatment plans for Sam in regard to his continuing with his ARQ-197 treatment, but it is my understanding that if there is more than 20% disease progression by RECIST measurement criteria, that the patient cannot continue with the ARQ-197 Trial, as was the case with Brittany when within two months of beginning the ARQ-197, she heartbreakingly developed more than 20% growth of her lung mets. Regarding the recommendation for "gamma knife radiation to the cerebellum as a preventive move", I strongly agree with Olga's wise input. If Sam's cerebellum met was completely resected, it was probably encapsulated, and there would be no reason for "preventative" gamma knife radiation unless there are other mets in the cerebellum, since gamma knife is used to target individual tumors. As Olga said, based on the nature of this disease, there are unfortunately probably other dormant brain mets which will eventually develop, but not at the site of the resected cerebellum met. Regular and vigilant brain MRI's every three months are essential to monitor the development of any new brain mets so that they can be found and treated at the smallest possible size. Additionally, between scans, it is critically important to be aware of any new symptoms of headache pain, dizziness, nausea, muscle weakness, loss of balance, vision problems, etc. which may be indicative of a brain met.
Pembro which is also called Keytruda may be an excellent systemic treatment option for Sam as it seems to have shown some successful results for some ASPS patients on this Board who are currently being treated with it. It is my understanding that a patient needs to have their tumor tissue tested prior to beginning Keytruda to determine if their tumor contains PDL because patients whose tumors test positive for PDL are apparently better candidates for having a successful response to this promising new Immunotherapy drug. In the meantime, are there any plans to try to resect/ablate/or treat with radiosurgery Sam's met in the tail of his pancreas? If the pancreatic met is resectable or treatable with ablation or radiosurgery such as Cyberknife, it seems to me that would be the highest priority to help reduce Sam's tumor burden and better enable a systemic treatment to be more effective. My heart breaks for all that dear Sam, you, and your family are going through with these new difficult challenges and treatment decisions, but please know that you are not alone and that I and others on this Board are here to provide shared information, input, and strengthening support and encouragement. Take care dear Cindy and keep in touch as your time and the situation allow.
Reaching out to embrace you and Sam with special hugs, caring thoughts, healing wishes, much love, and continued Hope,
Bonni

Hello again dear Cindy, In reviewing your posts on Sam's Personal Updates thread, I was unable to determine if he ever had his primary tumor in his left thigh resected. When your time allows, could you please clarify this question for me? Thank you. With more special caring thoughts, healing wishes, love, and continued Hope, Bonni