Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi everyone! I've been on this site before but forgot my password and username lol
I'm Amanda from Ontario Canada!
I was diagnosed with ASPS in 2001 at the age of 17.
My primary tumor was in my right upper inner thigh,
Metastasized to my lungs.
I had my primary tumor removed along with 2 thoractomys on both lungs, where they resected over 50 mets. This was all done within the first year of being diagnosed.
I underwent over a year of celebrex and vinblastine (clinical trial) at Toronto sick kids,
I Became septic and came off the clinical trial.
My parents were told to take me home and give me quality of life at that time.
My parents reached out to Dr.Kellar in Ottawa,
He put me on traditional chemotherapy,
Doxrubicin and Ifosfamide.
I only did a couple rounds before I said no more.
I am now 33, almost 34,
With no sign of disease,
I have 2 beautiful boys who are 11 and 12,
I was told I would never have children.
Life is good!
I go for scans yearly but have since been thinking maybe every 5 years would suit me as there has been no sign of disease for so long.
Just wondering what your thoughts are.
I don't think the oncologist I have now has ever seen, or experienced anyone with asps,
Would be nice to hear everyone's thoughts on how often I should be seen, and scanned.

Xo

I wish there would be a simple answer, but there isnt. All other sarcomas are very unlikely to grow after 5 years ned, but asps is different, it is possible that you still have microscopic tumors that cant be seen in scans. These possible micromets can stay microscopic for years even decades and then start growing, if mets are found small they can be treated lot easier and cheaper compared to if nets are found symptomatic. So scanning is vital for asps. Most common metastatic sites are lungs, brains and bones. So brains should be monitored with mri at least once a year, but there is no radiation in mri, so you can have it as often as you want, if your kidneys work and you are not allergic to contrast. For lungs mri is accurate enough, it will detect over 1 cm mets with 100 percent sensitivily. This is ideal size for cryoablation, so if you cant have another thoracotomy then ablation is my favourite treatment. Lung ct can miss smaller mets and it is slower than ct, so it is hard to convince doctor to order annual lung mri instead of ct, but because of asps slowly growing nature you need scanning for the rest of your life so radiation exposure must be minimised. For abdomen mri is better than ct, so I would have that as well. Bonescan is possible, but I havent used it, because bones are not vital organs and bonescan has lots of radiation. In order to detect possible mets before they became to large to treat you must be scanned at least annually. If possible tumors are found when they are too big for local treatments ( surgery, ablations stereotactic radiation) then you need immunotherapy pd1-inhibitors, because immunotherapy is very expensive it actually is lot cheaper to invest money in scans, so tumors are found early. I know that having scans is scary, but it is something that asps-patient must learn to deal with.

Amanda, here are people that remember you very well.
it is really fantastic to hear from you again, still NED.
For scans - I would get a CT scan for lung mets yearly and MRI brain once a year. We had the cases of the very late relapse, like after 10 years of NED a patient found a huge lung tumour...or after 15 years the brain met was found...

Hi Amanda

When we were talking earlier , I was curious about the trial that you had been on with the Celebrex and vinblastine.
Knew of vinblastine being a chemo however hadn’t known the thought behind using Celebrex ?
It’s what is referred to as a NASAID( non-steroidal anti-inflammatory drugs or NSAIDs) and is also a cox-2 inhibitor.

I found am article on Celebrex and cancer that explains this thought behind using at least the Celebrex . : )


https://academic.oup.com/qjmed/article/ ... 11/1602554

So good to hear from you on the icureasps site.
With your story comes hope! It will bring this hope to ALOT of people.
Thank you from the bottom of my heart for posting .

Love to you

Dear Amanda,
What a special joy it is to hear from you on this Forum!! Thank you so much for reaching out to re-connect with the Board and to share your inspirational ASPS story. With our families having started this roller coaster ASPS journey together 17 years ago, we have truly shared some of Life's greatest heartaches, challenges, and joys, and we Hesses are so deeply grateful for your and your family's treasured special friendship and continued support and encouragement as we continue on this challenging journey. We are so grateful too for the rare sustained disease stability and NED status that you achieved following your courageous harsh traditional chemo treatment so many years ago, and for all that you have been able to do and accomplish since then, now as a wonderful young mother with two beautiful children. You are an incredible young woman dear Amanda, and a source of great inspiration and Hope to Brittany, Jim, and I, and to all of those fighting this insidious disease. Regarding your question about possibly changing from once a year monitoring scans to only once every five years, I strongly agree with all of the pre-cautionary advice and concern that has been expressed by Olga, Jussi, and Debbie about that plan. Unfortunately, this cruel disease is very unpredictable, and anecdotal experience by other long term ASPS survivors has shown that it can heartbreakingly suddenly reappear (and often rapidly progress) after numerous years of long term sustained stability and seeming to be completely gone. At this point in time and until a definitive way to prove that the ASPS is permanently cured, Lifelong vigilant and regular scans are essential in adequately monitoring and managing the disease. Brittany's extremely knowledgeable Cediranib Clinical Trial oncologist has told us that even though Brittany has now had nine years of sustained disease stability with no new mets, and all of her previous innumerous and widely disseminated mets have shrunk and disappeared from the scans, that she may be macroscopically tumor free, but sadly probably not microscopically. We are painfully aware that she could develop disease progression at any time which is why we continue to have her brain, spinal, and bone scans done once a year, and her chest/abdominal/pelvic done scans done once every four months in order to detect any possible new mets at the smallest most treatable size. Since you said that your current oncologist is unfamiliar with ASPS, he is probably like too many doctors who are naiive about the nature of this unpredictable disease, and based on your long term NED status he may become complacent and overly confident regarding the critical importance of continued appropriate and adequate scans which should definitely include an annual brain and spinal MRI, an annual full body bone scan, and an annual chest CT and abdominal/pelvic MRI. You DO NOT want to wait to become symptomatic, but if you do develop any concerning symptoms in any of those areas, be sure to schedule an appropriate scan as soon as possible. I don't want to worry you, and it is my greatest Hope that you will remain NED dear Amanda, but it is much better to be pro-active and safe rather than complacent and sorry. In the meantime, please take good care of your incredible beautiful self, tell your dear mother "Hello", feel the embrace of my special hugs, enjoy your precious sons, have a wonderful summer, and keep in touch as you are able.
Heart to heart with deepest gratitude for your thoughtful sharing, and with special caring thoughts, much love, and continued Hope,
Bonni

That's fantastic news, Amanda :) We were diagnosed at almost the same time, but our courses of treatment have been quite different LOL.

The good news is whether there's ever a recurrence, there's some great options available now that weren't available back then.

Hi Amanda,

I'm reading your story for the first time and believe me it is a great motivation for me and all here that there is always hope..

thank you for sharing

Your story is such a motivation for all of us.It gives hope that miracles do happen :) I hope all asps patients get their miracles <3