Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Dear ASPS Community Friends,

I am Luke from China. I was diagnosed with ASPS in 2013, and have been fighting it ever since. My dad discovered this forum and shared with me many posts from here, which inspired me to stay strong and believe in the future. The past 12 months have been especially difficult for me, but now my disease is stabilized and I want contribute to this forum by sharing my own experience, especially the treatments I've gone through, to hopefully help fellow patients gain confidence and hope.

I was diagnosed in April 2013, a year after i graduated from college. I was in the US at that time and have been receiving treatment in the US from then on. Here's a brief timeline of my treatments:

• April 2013 - Surgery to remove primary tumor. CT scan found small mets in both lungs (all < 1cm)
• June 2013 to August 2013 - Radiation to the primary tumor site
• September 2013 to April 2015 - disease mostly stable
• May 2015 - found a bump on my neck, ultrasound and CT scans showed it as a blood clot
• June 2015 to September 2015 - the bump kept increasing in size despite I've been taking blood thinners.
• October 2015 - biopsy of the neck revealed that it is a recurrence of ASPS. At this time the tumor on my neck was already 8.8 cm AP x 2.8 cm transverse x 4.3 cm SI. The small mets in my lungs also started growing rapidly.
• January 2016 to February 2016 - radiation to neck & jaw & shoulder
• March 2016 - suddenly developed double vision. MRI showed a tumor inside right cavernous sinus. Treated with radiation again
• April 2016 to May 2016 - the residual tumor on my neck & jaw still kept growing. The tumor was very visible and I couldn't go out without wearing a patch on my neck. Under the advice of my oncologist, I went to University of Miami Hospital for a immunotherapy clinical trial (https://clinicaltrials.gov/ct2/show/NCT02636725)
• June 2016 - Started the clinical trial with 6.7 x 3.9 cm tumor in neck&jaw and >10 lung mets with the biggest one measuring 1.6 x 1.7 cm. Taking 5mg Axitinib twice a day, and a Pembrolizumab infusion (200mg I believe) every three weeks.
• September 2016 - neck tumor decreased to 4.7 x 1.8 cm, lung mets also decreased in size to various degrees (biggest one decreased to 1.1 x 0.7 cm from 1.6 x 1.7 cm)
• November 2016 - neck tumor decreased to 4.7 x 1.4 cm, lung mets decreased in size again

I am really grateful that my oncologist introduced me to this immunotherapy clinical trial. It has a focus on ASPS patients and Dr. Breelyn Wilky, the doctor who initiated this trial, really cares about ASPS. It is an extremely rare disease, and I think it makes a huge difference to have a doctor who is determined to find a cure for us. I think currently there are only 13 patients on this trial, and many of them are of different sarcoma types, but I heard from doctors and nurses that most patients have improvements, and some of them very significant.

I think we are in a very exciting era when new treatments pop up every day, especially immunotherapy. I use the google alert service which searches the whole internet for me everyday for keywords Sarcoma and ASPS. And literally every day I get news about new progress in clinical trials and new drugs coming out. I wholeheartedly believe that we're not far away from a cure. And I would definitely suggest patients like me to give immunotherapy a try.

This is my first time posting on this forum. I hope the formatting is correct... And I wish this post could be helpful for everyone. Feel free to ask me questions.

Thank you and stay strong!

Hello Luke
My name is Debbie and my husband and I have a son Josh that was diagnosed August of 2012, with ASPS. His primary was on his right upper thigh front side. A little under 7cmx5cm
Here's his personal story
http://www.cureasps.org/forum/viewtopic.php?f=4&t=750

Thank you for sharing your story with us as I know there has to be times when it's so overwhelming to think of, for the patient.

It sounds like you've been on Pembrolizumab about as long as Josh has been on nivolamab (Opdivo )

http://www.cureasps.org/forum/viewtopic.php?f=82&t=1297


Where was your primary located before it was removed in 2013? Radiation was performed because it wasn't able to be removed completely?

Your story is one of true bravery and you are so right when you talk of the time we are living in as being a truly great
era.

Olga , whose son Ivan was dx'd in 2003, will let you know where she'd like your story

Here's Ivan link
http://www.cureasps.org/forum/viewtopic.php?f=4&t=66

Sincerely
Debbie Pearson

Hi Luke,

Thank you very much for sharing your story with us. I was just recently diagnosed with ASPS and it is very encouraging and helpful to see other patients' stories and what they have tried. The new clinical trial in Miami seems to be promising. I truly hope that the medications will continue to work for you. Have your doctors recommended surgery for the neck mass? How big was your primary and were they able to achieve negative margins? Please keep us updated with your progress when you are able. Take care.

Your post really saved my day thanks. I am on keytuda myself.

Hi Luke,
thank you so much for coming out and posting your story. The information you gave us just priceless - I personally found few things that I did not know about ASPS:
- that a soft tissue ASPS met can occur in the neck area, we did not have a case like that before;
- that in a small size its can be mistakenly identified by the ultrasound and CT scans as a blood clot (so MRI is necessary to perform or at leat an interval scans to rule it out);
- that the soft tissue ASPS met can grow very rapidly - to 8.8 cm x 2.8 cm x 4.3 cm from almost nothing in just few months, even if we assume it was there earlier than you noticed it - it took like 6 months to grow that big;
- that the sinus met can manifest itself by the double vision, I would suspect a brain met intead. We already have a case of the sinus metastatic tumor so we knew it can go there;
I have few question if I may:
- where in US was you primary care performed when Dx, what hospital/oncologist?
- are you in US now or are traveling from China for the trial participation? Do they enroll international patients?
Thank you in advance.

Hi Debbie,

Thank you for your fast resply. My primary tumor was at the base of my tongue. I was told by my surgeon that I'm probably the 3rd person in history to have this sarcoma started on that location. He believed it was a benign tumor until he resected it and sent it to a pathologist, so he didn't do a safe margin during the surgeon. As a result I had to have radiation to clear that area.

I hope Josh is doing well. Stay strong!

Hi ntran,

My doctors did consider a surgery. But after they reviewed the MRI images, they believed that a surgery would be too damaging (they said at the very least I would not be able to speak again), and there's no guarantee that they would be able to remove all of it and prevent it from coming back. So I went with radiation.

The primary tumor was at the base of my tongue. It was about 2 - 3 cm and they didn't achieve a safe margin when resecting it because they thought it was something benign..

Hi arojussi,

Hope you'll have great results! Stay strong buddy!

Hi Olga,

Thank you for your reply and I also wanted to thank you for creating this forum and making it possible for patients to share information.

The tumor on my neck actually managed to deceive a MRI test too, along with the CT and ultrasound test. Sorry I didn't make it clear in the post. I think for some reason ASPS is really good at pretending as hemangioma. In my case, when I first went to a doctor in 2013, he had me do a series of scans and he concluded that it's hemangioma. The same thing happened again in 2015, when the doctors examined the scans and believed that it's just "something weird with my blood vessels, probably a bloodclot". It fooled me twice, so shame on me. I hope other patients could learn from my experience and request a biopsy whenever "something weird" pops up, even if your doctor says it's unnecessary.

I was in Boston in 2013 for a Master program, so I went to Mass General Hospital for both the surgery and radiation. In 2014 I moved to NYC for work, and started going to Memorial Sloan Kettering Cancer Center. In June I joined the clinical trial at University of Miami Hospital and I'm commuting from NYC. I do believe they enroll international patients, as long as you can travel. For the first three months the patient needs to see the doctor every week. After that it becomes every three weeks.

lzx90415 wrote:Hi ntran,

My doctors did consider a surgery. But after they reviewed the MRI images, they believed that a surgery would be too damaging (they said at the very least I would not be able to speak again), and there's no guarantee that they would be able to remove all of it and prevent it from coming back. So I went with radiation.

The primary tumor was at the base of my tongue. It was about 2 - 3 cm and they didn't achieve a safe margin when resecting it because they thought it was something benign..

Luke

Thank you for the response
Sure makes sense as an area that is so vital to not remove the muscled area plus not unusual for doctor to not suspect ASPS. But to see a vascular hemangioma
Not the first doctor to make this mistake

Did the MRI show no recurrence?
Patients need to really be vigilant when they have ASPS to let their dentists know of having alveolar soft part sarcoma so they as doctors can watch for tumors.

How'd you discover the tongue tumor ?
Thank you so much for your upbeat attitude and support of all
Love to you and family

Dear Luke,
I am so sorry for your ASPS diagnosis, but am very grateful that you found your way to our Web site and that you have reached out to graciously share the detailed information about your ASPS journey and treatments. The diagnosis of your primary tumor in your tongue is indeed extremely rare, although I personally know of one other patient who was diagnosed with ASPS in his tongue at about age 5. Thankfully his disease did not metastasize beyond his tongue and he did not have any further disease progression. Your tongue tumor diagnosis documents that this insidious and unpredictable disease can, and does, develop and metastasize to any soft tissue areas of the body as we have learned the hard way through our now 15+ years of our daughter Brittany's very challenging ASPS battle. The erroneous initial diagnosis of your tumor as a benign hemangioma or blood clot also illustrates the difficulty in accurately diagnosing ASPS which happens far too often with this extremely rare and little known disease.
I am so deeply grateful that you found your way to Dr. Breelyn Wilky's very promising Immunotherapy Clinical Trial in Miami and that you are having a very successful and encouraging response to the Axitinib and Pembrolizumab (Keytruda) drugs with significant shrinkage of your neck/jaw tumor and lung mets. The information which you have so thoughtfully shared is critically important and very encouraging for all of us in the ASPS Community who are closely following the promising new immunotherapy treatments and Dr. Wilky's Clinical Trial with greatest Hope that they will prove to be a desperately needed VERY successful treatment for ASPS. Perhaps Olga will move your posts to the Immune Checkpoint Inhibitors (ICI) topic on the Discussion Board where it can be more easily followed. It would be very helpful if you could also share about any side effects that you are experiencing from the drugs. Are you being followed with complete scans including chest, abdominal, pelvic, brain, and bone, or only with scans that follow the areas where you had tumors and mets when you began the Trial as, in our experience, is the common practice with most Clinical Trials? We personally feel that it is critically important to regularly monitor and scan all areas of the body since this unpredictable disease can show up in other areas rather than just the ones being followed by the Clinical Trial.
I will be anxiously awaiting your next update. In the meantime, please take good care of yourself dear Luke, travel safely in your frequent travels to Miami, tell Dr. Wilky "HELLO" from the Hesses, know that you are not alone in your battle, and know too how deeply grateful I and the other ASPS Community members on this Board are for your thoughtful and invaluable sharing. Truly shared anecdotal treatment information is one of our strongest weapons in fighting this extremely rare and very challenging disease.
Holding you very close in my heart and my most caring thoughts with healing wishes and continued Hope,
Bonni Hess, mother of now 34 year old Brittany diagnosed in July 2001 at age 19

D.ap wrote:

lzx90415 wrote:Hi ntran,

My doctors did consider a surgery. But after they reviewed the MRI images, they believed that a surgery would be too damaging (they said at the very least I would not be able to speak again), and there's no guarantee that they would be able to remove all of it and prevent it from coming back. So I went with radiation.

The primary tumor was at the base of my tongue. It was about 2 - 3 cm and they didn't achieve a safe margin when resecting it because they thought it was something benign..

Luke

Thank you for the response
Sure makes sense as an area that is so vital to not remove the muscled area plus not unusual for doctor to not suspect ASPS. But to see a vascular hemangioma
Not the first doctor to make this mistake

Did the MRI show no recurrence?
Patients need to really be vigilant when they have ASPS to let their dentists know of having alveolar soft part sarcoma so they as doctors can watch for tumors.

How'd you discover the tongue tumor ?
Thank you so much for your upbeat attitude and support of all
Love to you and family

Hi Debbie,

The MRI failed to detect the tumor either. The radiologist who read the images believed it was something caused by bloodclot. They only re-read the images after the biopsy. And i agree that we should tell our dentists about our situation so they can watch for tumors.

Luke
In their defense
They weren't trained to know what they were looking at
Such a rarity
We need to educate the medical folks
Josh in 2012 thought he had a pulled muscle in his upper thigh .. not so ASPS journey began

We are so lucky even tho it feels not so to have all the technology , and doctoring on our side to fight our sarcoma today , tomorrow and the days to follow.

Thanks for the touching of base today
Night
Debbie

Bonni Hess wrote:Dear Luke,
I am so sorry for your ASPS diagnosis, but am very grateful that you found your way to our Web site and that you have reached out to graciously share the detailed information about your ASPS journey and treatments. The diagnosis of your primary tumor in your tongue is indeed extremely rare, although I personally know of one other patient who was diagnosed with ASPS in his tongue at about age 5. Thankfully his disease did not metastasize beyond his tongue and he did not have any further disease progression. Your tongue tumor diagnosis documents that this insidious and unpredictable disease can, and does, develop and metastasize to any soft tissue areas of the body as we have learned the hard way through our now 15+ years of our daughter Brittany's very challenging ASPS battle. The erroneous initial diagnosis of your tumor as a benign hemangioma or blood clot also illustrates the difficulty in accurately diagnosing ASPS which happens far too often with this extremely rare and little known disease.
I am so deeply grateful that you found your way to Dr. Breelyn Wilky's very promising Immunotherapy Clinical Trial in Miami and that you are having a very successful and encouraging response to the Axitinib and Pembrolizumab (Keytruda) drugs with significant shrinkage of your neck/jaw tumor and lung mets. The information which you have so thoughtfully shared is critically important and very encouraging for all of us in the ASPS Community who are closely following the promising new immunotherapy treatments and Dr. Wilky's Clinical Trial with greatest Hope that they will prove to be a desperately needed VERY successful treatment for ASPS. Perhaps Olga will move your posts to the Immune Checkpoint Inhibitors (ICI) topic on the Discussion Board where it can be more easily followed. It would be very helpful if you could also share about any side effects that you are experiencing from the drugs. Are you being followed with complete scans including chest, abdominal, pelvic, brain, and bone, or only with scans that follow the areas where you had tumors and mets when you began the Trial as, in our experience, is the common practice with most Clinical Trials? We personally feel that it is critically important to regularly monitor and scan all areas of the body since this unpredictable disease can show up in other areas rather than just the ones being followed by the Clinical Trial.
I will be anxiously awaiting your next update. In the meantime, please take good care of yourself dear Luke, travel safely in your frequent travels to Miami, tell Dr. Wilky "HELLO" from the Hesses, know that you are not alone in your battle, and know too how deeply grateful I and the other ASPS Community members on this Board are for your thoughtful and invaluable sharing. Truly shared anecdotal treatment information is one of our strongest weapons in fighting this extremely rare and very challenging disease.
Holding you very close in my heart and my most caring thoughts with healing wishes and continued Hope,
Bonni Hess, mother of now 34 year old Brittany diagnosed in July 2001 at age 19

Hi Bonni,

I'm sorry for your Brittany's diagnosis. But I really admire her for fighting this horrible disease for such a long time and never give up. She's proven to us that we can live long and have faith in treatments. From the bottom of my heart I wish her well and that in the near future we could all find a cure.

Speaking of the biopsy, I had the same concern too. I got radiation before going to the clinical trial, so I was worried that the neck biopsy might fail and they'd have to biopsy my lungs, which will cause the problem you mentioned. Fortunately, Dr. Wilky told me that if the biopsy failed they wouldn't do another one. But yes I agree that there could be risks associated with biopsy, and it needs to be considered.

D.ap wrote:Luke
In their defense
They weren't trained to know what they were looking at
Such a rarity
We need to educate the medical folks
Josh in 2012 thought he had a pulled muscle in his upper thigh .. not so ASPS journey began

We are so lucky even tho it feels not so to have all the technology , and doctoring on our side to fight our sarcoma today , tomorrow and the days to follow.

Thanks for the touching of base today
Night
Debbie

Hi Debbie,

i totally agree. There's still much to learn about this disease, but technology has enabled us to find our way. Kudos to all the doctors for their hard works. They've made this world a better place.