Cure Alveolar Soft Part Sarcoma International (iCureASPS)

I write to you today excited to have found such a resource as your site and heartbroken that it is because of the diagnosis given to my two year old daughter Brinley. I am hungry for knowledge and any personal stories and experiances that may guide us through this new journey. Thank you in advance to any of you who care enough to help me gain an understanding of this disease. Here is alittle about us and how we came to learn of Brinley's ASPS.
My name is Crystal, I am a respiratory therapist (which means I have just enough medical knowledge to allow my thoughts to run away with my mind sometimes). I am a wife to my husband of 19 years, and proud parent of three amazing children. Two teenage sons and a beautiful two year old daughter Brinley.
On June 4th while eating lunch my husband felt what he thought was a chunk of food under Brinley's shirt. We lifted her shirt and found a pea size bump under her skin about 2 inches below her ribs on her belly. The next day was Monday and I was able to get her in to see the pediatrician, who in turn sent us to ultrasound. At ultrasound they discovered it was a vascular tumor and scheduled us an appointment with a pediatric surgeon on the 6th. After an exam of Brinley and a look at the ultrasound the surgeon scheduled surgery for the next morning, June 7th. The surgery went well. They removed the tumor and sent it down to pathology. After receiving some results as to the make-up of the tumor the surgeon returned to the operating room and removed the muscle tissue that surrounded the tumor as a precaution, before closing Brinley's incision. 3 days later we were given results from pathology that the surrounding muscle tissue was testing clear and that they were sending the tumor to Seattle as they had been unable to identify it at our hospital. On June 25th we received the diagnosis of ASPS. We were then scheduled for a CT scan of Brinley's lungs on the 2nd of July in Montana and sent to Seattle Children's Hospital on July 5th. Dr.Hawkins, a Sarcoma specialist, met with us and answered our questions to the best of his ability. He told us that because of the rareness and the number of variables it is a difficult disease to study. He believes Brinlry to be the youngest patient diagnosed with ASPS thusfar. (which is unsettling to me).His youngest paitent treated was 4 years old. He wants us to come back to Seattle every 6 monthe for CT scans of her lungs or possibly just x-rays sometimes to minimize the amount of radiation, and MRI's of her belly which was the origination site, but nothing more at this time. We received a call from Dr.Hawkins a few days after arriving home from our Seattle trip and he explained to us that he had been reviewing the CT scan of her lungs(Which was clear) and had been in touch with the pathology lab that had Brinley's tumor. The tumor was not encapsulated (as he had assumed or been led to believe) and the cancer was to the margins of the tumor. He wanted us to hurry back to Seattle for revision surgery. After much discussion I was able to persuade him to call the pediatric surgeon that had preformed Brinley's surgery and obtain the surgical report where indeed it stated she had removed enough of the surrounding muscle and revision surgery was not necessary (Thank Goodness!) We did donate Brinley's tumor to ASPS research whenthey were done with it!
I am surprised that there is no further testing to be done now??? Is it just the mama bear in me that wants to pretty much scan her whole body to make sure we have a clean slate and have not missed anything.... I really think I want a second opinion....I was thinking about the cancer hospital in Phoenix, I have heard amazing things about their work there. I am meeting with our pediatricians office tomorrow to find out how a person goes about getting a second opinion. Are scans every 6 months adequate? Maybe I should fill you all in on one more thing about Brinley. Her initials spell BOB for her uncle Bob whom we lost to Ewings sarcoma 4 years ago. He fought hard for 7 years before losing the battle. The sarcoma specialist says there is no corralation betweenthe two of them having these two sarcomas, but they seem pretty simular to me, and that much more scary to me and my family.
If anyone out there has any information (good or bad) about Seattle Children's hospital or the one in Phoenix please let me know. I would love any input on treatments, simular stories of children close to her age or tumors originating in the belly, really open for any and all correspondence just to gain a greater understanding for what we are up against.
Thank you so much to the folks out there who post on and maintain this site. You are wonderful and I am so happy to have found you. Good luck to all of you out there affected by ASPS please stay strong.
Sincerely,
Crystal, Brinley and family

Hi Crystal, welcome to the board.
I am really appalled on the primary surgeon actions - we see it again and again, in fact it happened to my son as well - when the ultrasound has shown that the nodule is suspicious, they proceed with the surgery instead of doing an MRI and FNA to find out what the nodule is and later take it into the surgery plan and resect it with the appropriate margins right away.
I would not be comfortable with the scans every 6 months right from the beginning. This schedule is adequate after 2 years of the clean scans but in the beginning they should not be that far apart, I would say 4 months interval should be used for at least a year - I am not saying 3 months as it is known that ASPS grows slowly - although I would make the first one 3 month just in case. ASPS tumors are known to metastasize very early regardless of their size and after the primary tumor removal significant number of the patients that had clean scans start to see their dormant mets to surface as the surgery stimulates the growth factors.
I am sure that the second opinion is allowed based on the rarity of the disease, it can be arranged trough the COG if your or Seattle hospital is a member there. MDACC, MSK and Mayo clinics would have seen the most of the patients.
The treatment plan proposed - surveillance - looks about right to me, there is really nothing to do now.
I found another similar case at the Pubmed:
http://www.ncbi.nlm.nih.gov/pubmed/21317811
and with a diff. localization http://www.ncbi.nlm.nih.gov/pubmed/17964622
read the articles we have in the library on the main page and look on the Pubmed.
If you do not mind, I'll open a new topic for Brinley in the Patient's Updates later and move this conversation to that topic to keep all the info organized.

Olga, thank you so very much for responding so quickly. Yes pleas do whatever you feel is best with my post as I would love any input possible. I will indeed research the article you posted the link to. Are there any registries or othersources you would reccomend as well? Thank you again for your compassion and all you do for the ASPS community. Have a wonderful day.
Sincerely, Crystal, Brinley and family

Dear Crystal,
My name is Bonni Hess, mother of now 30 year old Brittany who was diagnosed with ASPS at age nineteen, eleven years ago in July 2001. I am so deeply sorry for your precious two year old Brinley's heartbreaking recent ASPS diagnosis and all that she and your family have been through the past two months. I am grateful that you found your way to our Web site and that you have reached out for information, input, and support from the members on this Board. I admire your great strength, your very pro-active approach, and your efforts to be as knowledgeable and well informed as possible, all of which are so critically important in fighting this extremely rare and challenging disease. Thank you for having the foresight to donate Brinley's tumor for desperately needed ASPS research. Was the tumor donated to the ASPS research being done at Dana Farber or by David Vistica? I am familiar with Dr. Hawkins at Children's Orthopedic Hospital (COH) in Seattle because he was ASPS patient Adam Beatty's Sarcoma Oncologist until Adam turned 21 and could no longer be treated there because of his age. I know that Adam's family highly respected Dr. Hawkins, and COH is certainly a highly regarded children's hospital, but I understand and agree with your wanting to obtain a second opinion. The Sarcoma Alliance will provide funding for travel to obtain a second opinion if you are interested. I agree with everything that Olga has said, and think that Brinley should not only receive scans at least every 3 to 4 months for at least the first year, but that she should have complete scans now including chest/abdominal/pelvic CT scans, a brain MRI, and a complete bone scan if they have not already been done. The best currently available treatment for ASPS is to resect/ablate/or treat with radiosurgery any metastatic tumors at their smallest possible size, and this requires vigilant scanning to diagnose tumors when they are very small and before they become symptomatic.
My heart breaks for the devastating loss of your dear brother?/brother-in-law? Bob to Ewings Sarcoma four years ago, and I know that your experience with his courageous seven year battle must increase your fears, but it is important to remember that each type of Sarcoma is different, each patient may respond differently to different treatments, and, as per my personal observations during our eleven year ASPS journey, it seems that the younger the patient at diagnosis, the less aggressive the disease. I don't know if there is any data to confirm this, but Olga may know if there is.
Our primary Home is in the Seattle area, but we also have a second Home in the Bigfork area of Montana. Where in Montana are you located Crystal, and where is Brinley receiving her pediatric care? If you are in the Bigfork/Kalispell area I would be glad to meet with you to try to better answer any questions that you may have. Please know that you are not alone in this battle, and that those of us on this Board are here to help you with shared information and strengthening encouragement and support. Take care, let Hope lead you through each day, and keep in touch as you are able.
Reaching out to share special caring thoughts, gentle hugs and healing wishes for precious little Brinley, and continued Hope,
Bonni

Bonni,
You have no idea how much your kind words mean to my family and I. We so appreciate both you and Olga sharing your knowledge and compassion. I am sorry to say I am not sure which organization received Brinley's tumor. That day at Seattle Children's hospital was overwhelming to me, and I had so much guilt sitting in that waiting room surrounded by gravely ill children while my Brinley was squealing and playing. It was a very humbling experience and some of the details I am afraid did not stick to memory. I am very happy to hear that Dr. Hawkins is highly thought of...comforting to know she is in good hands even though we believe there should be more legwork done right now in the early stages.
Brinley's pediatrician is in Missoula out of Community Medical Center we are still meeting with her Monday the 6th to discuss how to go about getting the second opinion. I printed off both yours and Olga's responses as well as some articles from this website for her to review and familiarize herself with ASPS before our appt. I do hope she will take a look at it all. So in answer to your question, we live just east of Missoula. I would absolutly love to drive up to Bigfork/ kalispell and visit with you if you are sure it would not be an imposition. I know it would be very reassuring to speak to someone who has walked the walk, and I would luv for you to meet Brinley.
i need to look more closley

Sorry, Bonni,
Typing on my cell during my lunch break and it submitted before I was through
Anyway, I was trying to ask how Brittany is doing now? I will search the site and read your families posts.
Thank you again for your response.
Sincerely,
Crystal, Brinley and family

I hope your little girl grows up without having to deal with ASPS. This annoying disease is not suitable for a child.

I think that CT scans every 4-6 months with an occasional brain MRI should be sufficient follow up.

Dear Crystal,
Thank you for your thoughtful response. My special thoughts and best wishes will be with you tomorrow when you meet with Brinley's pediatrician. If you have any questions regarding things that you want to address and discuss at your appointment, please feel free to call me on my cell phone at 425-829-3210 or e-mail me at BonniHess@aol.com. It would certainly not be an imposition for you to visit me in Bigfork, and I would love to meet you and precious little Brinley when your time and busy schedule allow. Just call and let me know when you can come and we can work out a date that works with both of our schedules. I have always felt that shared information is one of our most powerful weapons in fighting this extremely rare and little known disease, and I am glad to share any of the information and knowledge that I have gained on our eleven ASPS journey that may help other ASPS patients and their families in fighting this challenging battle.
In response to your question about how Brittany is doing, she has now thankfully had 39+ months of disease stability with dramatic shrinkage and disappearance of her multiple and widely disseminated mets and no new tumors since beginning a Cediranib Clinical Trial in Edmonton, Alberta in April 2009. You can access and follow her Cediranib treatment experience and results in the Cediranib topic on this Forum which I keep currently updated. Brittany's next Clinical Trial scans are on August 24th and, as always, our scanxiety is growing as she could potentially develop resistance to the drug and disease progression at any time, but we are holding very tight to Hope that she will continue to have a successful and sutained response to the Cediranib and continued disease stability.
I must close for now, but I'll look forward to hearing from you when you are able. Take care dear Crystal and enjoy today's beautiful Montana sunshine together with dear little Brinley and your family.
With special hugs, deepest caring, healing wishes for precious Brinley, and continued Hope,
Bonni

Thank you Ivan. I hope that we can gain a good second opinion so that we can remain vigilant and address whatever comes our way, good or bad! I commend you on your courageous battle and look forward to your insight on our journey forward. Stay strong!

Bonni, thanks again. I feel so blessed to have found you, and this site. I will keep you posted as to our next steps in this new land. Hoping to make some headway this afternoon at our pediatric appointment.

Best wishes everyone!
Crystal, Brinley and family

Hello again everyone,
I am writing to you all today to ask if anyone has suggestions of where to seek our second opinion for treatments. It seems to be challanging enough finding hospitals familiar with ASPS, but then adding the factor that Brinley is only two years of age seems to throw another loop into this already complicated mix. Any input from anyone knowing of good pediatric care would be greatly appreciated. Our pediatricion is currently placing calls to St.Judes and the Mayo hospitals has anyone been to to either of those for care?
Thank you in advance for any replies. Our family is still trying to get our feet back under us so that we can journey down this new path.
Sincerely,
Crystal, Brinley anf family.

I know that MDACC and MSK see most of the sarcoma patients, you need to find out if they had enough younger cases to be able to figure out stats for that specific age.

St. Jude's Children Research Hospital is a great facility.

http://www.stjude.org/stjude/v/index.js ... ri%20spunt

The link above has all the info on Dr. Spunt.

She has been my oncologist for close to 6 years and she is Awesome!!!!

Dr. Spunts Nurse Practitioner's name is Joan Harper also if that helps.

If the link doesn't work let me know I can send it to you via PM.

Hope this helps

Mario

Thank you Mario! I have heard a bit about you and that you were under St.Judes care at one time, but are too old now??? So happy to hear a good hospital & oncologist referral all in one. The link did work and I sent it to Brinley's pediatrician to look over the details. Once again thank you for taking a moment to help our family research the best care for Brinley. May I ask how you are doing now? Stay strong! Best of wishes to you!
Sincerely,
Crystal, Brinley and family

Thank you Olga for your response. I passed that info along to our pediatrician. We have read articles posted by MSKCC and they do seem very knowledgable. We really appreciate your input, thank you for taking the time to respond!
Sincerely,
Crystal, Brinley,& family

Dear Crystal,
I am grateful that Olga and Mario have provided you with referral information for facilities that have experience in treating Sarcoma where you might be able to obtain a second opinion/treatment for your precious little Brinley. I would like to add Dr. Steven DuBois at UCSF Medical Center and Children's Hospital in San Francisco to the list of possibilities. Dr. DuBois is a very kind, caring, and compassionate pediatric oncologist who treated Brittany and Mario during their ARQ-197 Clinical Trial experiences. Unfortunately the
ARQ-197 was unsuccessful for both Brittany and Mario in stabilizing the progression of their diseases and shrinking their tumors, but Dr. DuBois continues to faithfully follow Brittany's ASPS journey and treatments with e-mail communication to us even though he is no longer treating Brittany which demonstrates to me a level of special caring and dedication which is not always found in some Clinical Trial follow-up. Dr. DuBois can be contacted by phone at 415-476-4764 , and the website address is www.ucsfhealth.org/childrens.
UCSF has a wonderful family housing facility located very close to the hospital. Please let me know if you have any questions regarding Dr. DuBois or UCSF that I might be able to answer, and keep the Board updated as you are able.
With special hugs, deepest caring, healing wishes for dear little Brinley, and continued Hope,
Bonni