Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Dear Mary Jo,
Thank you for your very gracious words and thoughtful update in the midst of the increased challenges and all that you are going through with precious Prairie's ASPS battle. I am glad that you are finding the information and special caring and support that is shared on this Forum to be helpful. This CureASPS Discussion Board is an invaluable source of up to date researched and anecdotal treatment information and strengthening support and encouragement for patients and families throughout the World who are fighting this extremely rare disease, and I just wish that more ASPS Community members would actively participate and share because shared information is truly one of our most powerful weapons.
I am so grateful that dear Prairie is recovering well from her surgery and that she is now Home ( the best place to be!!) continuing her recovery. It sounds like you have been very active in exploring and discussing with the oncologist the best treatment plan to follow now that the large pelvic area primary has been successfully removed. I am grateful that you persevered in insisting on a brain MRI, but I am so deeply saddened and sorry that four brain mets were found and that the doctor feels that they are unresectable due to their small size. Although you said that the multiple small brain mets diagnosis is "probably not a game changer", I personally feel that it should change the priority of treatments, and Gamma Knife of the four mets should be explored and pursued as soon as possible, if the mets actually can't be resected because of the small size or dangerous location. I encourage you to seek a second opinion from an oncological neurosurgeon regarding the resectability of the mets. Based on my unfortunately extensive personal brain met experience with Brittany's previous multiple brain mets and observations of other ASPS patients, I strongly feel that you should not just monitor ASPS brain mets and wait for them to get larger!! They need to be immediately addressed and treated/resected for the best chance of a successful outcome. With Brittany's first three brain mets in her parietal lobe and cerebellum, we were told that they were unresectable because of the location which the initial surgeon said would almost certainly devastatingly result in Brittany completely losing her vision so we went forward with Gamma Knife. Unfortunately, by the time the brain mets were diagnosed they were already too large to successfully respond to Gamma Knife and after five months of steroid Hell and continued and increased brain swelling and growth of the mets, our only option was surgical removal. We researched and found an excellent oncological neurosurgeon who felt that he could, and agreed to, resect the mets. He was able to completely remove the mets and preserve Brittany's vision with the exception of her parietal vision which unfortunately could not be saved because of the tumor location, but Brittany compensates and copes very well with that vision loss by just turning her head to see things in the peripheral field of her vision. Also, in considering and exploring systemic treatment, now that dear Prairie has been diagnosed with brain mets, you will need to find a treatment that can cross the blood brain barrier to try to prevent the development of any new brain mets. My research has shown that the results of studies on Sunitinib being able to cross the blood brain barrier are very limited and somewhat inconclusive at this time because the presence of active brain mets precludes Sunitinib treatment as well as Cediranib. However, based on Brittany's thankfully successful and sustained 6 years and 5 months response to Cediranib during which time she has very gratefully not developed any new brain mets despite her previous history of multiple brain mets, we feel that Cediranib thankfully does appear to cross the blood brain barrier to prevent new brain met development. Regarding your question about the need for the oncologist to detail the number and size of Prairie's lung mets, I think that he definitely needs to detail the size and location of the mets in case there are any large or dangerously located ones that need to be immediately addressed and treated in terms of possible Cryoablation. Lung met laser resection with Dr. Rolle is definitely a possible treatment option based on the large number of Prairie's lung mets, and one which I encourage you to explore and pursue, but it is my understanding ( Olga can correct me if I am wrong) that Dr. Rolle will not accept patients for treatments if they have mets in other areas of the body which is another reason to treat Prairie's brain mets as soon as possible and prior to any lung met treatment. I know and understand that you must be very overwhelmed with everything that is going on, all of the information and opinions that you are being given, and the difficult treatment choices and decisions that you are facing right now dear Mary Jo, but you seem like a very strong young woman and you are to be commended for your very speedy proactive approach to obtaining as much information as possible and exploring all of the treatment options. Please continue to reach out to those of us on this Board for shared information and input based on our knowledge and experience, and know that we are all here to try to help in any way that we can. Please try to take care of yourself and let family and friends help with whatever you may need in the midst of all of the physical and emotional exhaustion which I know that you are experiencing.
I am personally not a Facebook member because I devote my computer time to ASPS research and networking, but my husband has a Facebook account so I will ask him to participate in your campaign to have Taylor Swift visit with dear Prairie at the upcoming concert which would be such a very positive and happy experience for your precious little girl who has courageously been through so much. I am a strong believer in the importance of a positive attitude fostered by positive and fun experiences helping to strengthen the immune system to better fight this insidious disease. I Hope that Taylor Swift will compassionately honor your request to give dear Prairie the gift of some well deserved happiness and smiles in the midst of all of her heartbreaking pain and interrupted childhood joy.
Reaching out to embrace you with deepest caring, gentle hugs for precious Prairie and you, healing wishes, love, and continued Hope,
Bonni

Mary Jo

Found my specs .

I sure am sorry to hear of the brain scan showing up some specs of some kind of deposit.

I would venture to say they are 3mm or so?

With our son Joshua's MRI in August scan to December scan , his tumors grew from hardly discernible to 1 cm .
So, if possible, I would perform an MRI 1 month from now rather than waiting 3 months just to get a feel as to what the lesions could be.

I hope the pathology gives you some better answers to help with your radiation decisions.
Considering the size of Prairies tumor and lung lesions and possible brain lesions Olga and Bonni will have a better idea of possible systemic treatments either before or after treatment of lung lesion's.


The CT scan showed the lung lesions to be how big?

Love
Debbie

Thanks to both. I am guessing largest lung mets to be 1 cm, maybe 10 of that size and others smaller. Who would interpret this info for me? What nureosurgeon would you suggest?

Hello again dear Mary Jo, I am personally unfamiliar with the names of pediatric oncological neurosurgeons and radiation oncologists in your area for you to contact to obtain a second opinion regarding resection or Gamma Knife of dear Prairie's brain mets, but your primary oncologist should be able and willing to provide you with some referrals. Brittany's excellent and very skilled neurosurgeon who successfully resected her "unresectable" parietal and cerebellum brain mets is Dr. Dan Silbergeld at the University of Washington Medical Center in Seattle and her excellent and very experienced radiation oncologist who has performed her three Gamma Knife procedures is Dr. Jason Rockhill also at the U of W Medical Center in Seattle. I don't know if they treat pediatric patients but you could contact their offices if you are interested in coming to Seattle. Based on my 14+ years of extensive ASPS experience, observations, and research, the size and number of Prairie's innumerous lung mets is unfortunately very typical of ASPS and is about the same as Brittany's lung met situation was when she was first diagnosed. Once Prairie's brain mets have been treated with Gamma Knife or resected, I strongly recommend Cryoablation of her largest most concerning lung mets by Dr. Peter Littrup who pioneered lung met Cryoablation, or laser resection by Dr. Rolle. There is some data that indicates that Cryoablation may contribute to a more successful systemic treatment response which is another reason to pursue Cryoablation of Prairie's largest lung mets prior to her beginning the Sunitinib or Cediranib treatment that you have been considering. My special thoughts and best and wishes are with Prairie and you for tomorrow's doctor appointment to remove Prairie's drainage tube and to receive the patholgy results from the resected primary tumor, and I will be anxiously awaiting your update regarding the next treatment plans when your time and the situation allow. Holding precious Prairie, you,, and your family very close in my heart and most caring thoughts and sending special hugs, healing wishes, love, and continued Hope, Bonni

Dear Mary Jo,

I just wanted to say hello and extend my support toward you, your daughter and your family. My daughter Annika was dx in 2013 when she was 7 years old (you can read her story in Personal Updates). She turned 10 this year and your story resonates very strongly with me. Her diagnosis came as a complete shock to my husband and I, but like you, we have spent a lot of time reading, researching and talking to others in similar situations to try and understand this disease and give Annika the best possible outcome.

We are now monitoring her ASPS through regular scans (although we still have to fight for some of these) and hoping that this is all we will have to do. If we can offer support in any way or even just be another family with a child of a similar age with ASPS to talk to please don't hesitate to contact me.

Kind regards,
Katrina

I am not sure why Deb. suggesting that we are not absolutely sure to the DX of Prairies cancer - after the primary resection there is now a very sufficient supply of the tumor tissue for the pathology to properly Dx. Is the report ready from the surgery? Although I understand what Deb means - I too have a feeling that it behaves more aggressively than a typical ASPS in a small child - with the primary/lung/brain mets being present right away and multiple, but there is a big inter-patient variability between the cases. A radiosurgery for the brain mets when they are still small is a good idea as they are very resistant to any systemic treatment. Usually pediatric hospitals cooperate trough the COG - Children’s Oncology Group? try to find the most experienced radiosurgeon to perform the procedure.

Dear Mary Jo, Regarding Debbie's comment, I did not know if/that there was ever any question about the accuracy of the ASPS diagnosis. Can you clarify for us if a conclusive ASPS diagnosis was already made, which I assume it has been. Although for some reason Debbie seems to have doubts about the diagnosis, and regardless of whatever the definitive diagnosis is determined to be according to the resected tumor pathology report, the facts remain that Prairie's scans heartbreakingly show innumerous lung mets and 4 suspected brain mets, all of which need to be addressed, priortized, and treated as soon as possible for the best outcome in trying to manage and control Prairie's cancer. In response to your question about who might be able to help you sort out the number and sizes of Prairie's innumerous lung mets, her primary oncologist should be willing/able to do this, but if not for whatever reason, I suggest that you request and schedule an appointment with a radiologist to personally look at, review, and discuss the chest CT with him/her. Also, if you have not already done so, always request a written copy of the radiologist report as well as CD copies of all of the scans for your own personal records and at Home review. Familiarize yourself with what you are looking at on the scans so that you can be as knowledgeable as possible when looking at and reviewing the scans with the doctors. With this disease, knowledge is empowering. Take care dear MJ, stay strong, and keep in touch as you are able. With deepest caring, healing wishes for precious Prairie, much love, and continued Hope, Bonni

Hello again. I do agree with Debbie and Olga that Prairie's disease heartbreakingly certainly seems more aggressive, rapidly progressing, and extensive than that of most of the pediatric ASPS patients who we are aware of with this typically more indolent cancer, but as Olga has said each patient is different. We all know that this extremely rare and poorly understood disease can be very unpredictable. What is not unpredictable is that mets need to be treated/resected at the smallest possible size and not just watched to see if they will grow, and that regular, adequate, and appropriate scans need to be done to ensure finding any new mets at the smallest most treatable size rather than waiting for them to become symptomatic as many doctors inexplicably/naiively/inexcusably too often recommend and do. With more special caring thoughts, healing wishes, love, and continued Hope, Bonni

Deb, I'm not sure I'm getting what I should get out of the link, everyone dies?
All, yes, multiple institutions have verified the pathology as ASPS.
Thanks for the info on cryoablation first... And dr names. Her brain mets are about 1 mm so there is some speculation they may be something else, not cancer.
We will see the radiologist next Tuesday and talk more, as well as get set up fir radiation of primary site. Initially, she thought if they were 1mm that we would need to wait for them to get bigger before gamma knife would be effective due to its margin of error? Also, to be sure they are actually mets.
Has anyone ever heard if novalis radiation therapy system? Better than gamma knife?
Thanks, mj

No, 16 out of 19 died, but only 1 patient had ASPS and all the other sarcomas are much more aggressive. Probably the one with ASPS survived, it is usually the case.
1 mm is not the treatable size - can not be targeted, the radiologist will probably set up the schedule of the follow up scanning to see if there is a progression that would be the proof they are mets.
Novalis is a subtype of the Linac based targeted radiation (X-Rays) versus gamma rays based GammaKnife. For the brain location GammaKnife is still probably better although Novalis and CyberKnife are getting used more and more now and more drs get skilled enough for the results to be comparable. They all are diff. types of the radiosurgery - high intensity focused radiation with the use of the dedicated computer software to plan the delivery to achieve the max dose in the target but to try to spare the close located structures. The most important thing is to have a very experienced dr - the one who is done more than 100+ procedures, preferably 300+.

Mary Jo

We hope Prairie is feeling better today.

Love
Debbie

Dear MJ, I too was very perplexed and upset by the purpose/rationale for the posted link for the distressing report on pediatric sarcoma patients' brain met prognosis as it does not offer any positive recommendations for successful treatment outcomes which is, as Olga has appropriately noted, not relevant nor accurate for ASPS. In my experience and observations, when ASPS brain mets are diagnosed and treated at small sizes and vigilant brain MRI monitoring is maintained, the disease can be managed and there can be, and ARE, successful outcomes in terms of survival. I understand that Prairie's suspected brain mets are still too small for Gamma Knife or resection, but I would encourage you to request another brain MRI in a month instead of the currently scheduled three months to ensure that the lesions (if they are confirmed to be brain mets) don't grow too large for successful Gamma Knife treatment. In our personal experience with Brittany's ASPS brain mets, 4 mm. was the maximum size that could be successfully Gamma Knifed. I Hope that dear Prairie is continuing to recover well from her surgery, and that you have received a positive response from your request for her to be able to meet Taylor Swift at the concert. Take care dear Mary Jo. Heart to heart with deepest caring, healing wishes for precious Prairie, much love, and continued Hope, Bonni

Thanks to all, perfect info!
May I offer a distraction? We made the local TV station with our quest to meet Taylor swift.. And go on local radio station 94.1 out of Omaha ne tomorrow at 8:20am to continue our campaign... Trying to keep it light. Please all take care!
http://m.ketv.com/news/little-girl-batt ... t/35611496

This is wonderful - I am sure that Prairie is happy to meet Taylor swift and it will at least distract her from pain after the surgery. Please try to say the name of sarcoma often when talking about it during the interview or at least do not let them to say "cancer" instead of "sarcoma", to increase the awareness of the people about it. It is so often that this opportunity to make more people aware of sarcomas is lost in attempt to make it easier for the auditory to listen.

Mary Jo and Prairie

How cool is that !!
Have a great day
Love
Debbie