Cure Alveolar Soft Part Sarcoma International (iCureASPS)

This is the story of my daughter, Jordanne, and her battle with ASPS.
August 2009 Jordanne went to see her dentist for a root canal due to numbness in her mouth. After the root canal, the area was still numb and her dentist saw a dark spot just at the bottom edge of her Xray. He because concerned and sent her to an oral surgeon in Olympia to do a CT scan of the jaw where they found what seemed to be a tumor in her lower mandible. When the surgeon did the biopsy, he commented that he couldn't get it all because there was so much bleeding. They sent the tissue to the University of Washington for dx and we expected results in a week. 5 weeks later we were still waiting and they couldn't figure it out. After they mentioned that it might be some type of rare. I started investigating her symptoms on the internet and saw some mention of different primary tumors that start in the lower extremities. This made the connection that she had a swollen thigh that she thought was a pulled muscle that was becoming more painful and that had been giving her problems for months. So we called the oral surgeon with this bit of information. Low and behold they were able to make the connection and dx that she had ASPS. With further MRI and CT scans revealed that Jordanne had about 45 small lung mets as well

We were able to find this website and another on the internet so we were blessed to be able to find some surgeons back east that had experience with ASPS. One year ago this week Jordanne had her first surgery in Washington DC by Dr. Martin Malawer. He removed her primary thigh tumor which was about 17 cm. We donated the tumor to Dr. Vistica at the National Cancer Institute in Bethesda MD where he was able to put it in mice the same day.
So we hope that he will have some successful research for a cure of this disease. Dr. Vistica's daughter has ASPS so it is obvoiously very personal to him. Dr. Malawer recommended that Jordanne go to the National Institutes of health as they were starting a new clinical trial for ASPS. We met with Dr. Kumar who wanted Jordanne to start on Cedirinab right away and not have the surgeries. Jordanne said "no way" she just wanted the leg and jaw tumors out of her. After consultation with Dr. Malawer and Dr. Delacure at NYU they both agreed that it was better to remove those tumors.

The night prior to Jordanne's first surgery they did an embolization to shut off the blood flow to the tumor. This was a very painful night for her until they were able to take her down to preop and put an epidural in. A week after her successful surgery, her foot was still numb. Final determination was that possibly the embolization caused damage to her sciatic nerve. It was very disappointing to find that she had this surgery to improve her situation and she was still in excruciating pain with no feeling or movement in her foot.

2 weeks after her leg surgery, we travelled to New York City for the surgery to remove the tumor in her jaw which had grown through the bone. The surgeon, Dr. Mark Delacure removed part of Jordanne's jaw and replaced it with a titanium plate. The surgery seemed to be successful with clear margins. After 10 days returned home to the great state of Washington. We had gone to the East coast for a one week consult and ended up staying 6 weeks for 2 major surgeries as well as our first consult with the staff at NIH.

After returning home it took at least a month to find a pain specialist to try and find a solution for the extreme pain Jordanne was having due to the damaged nerve. All of the neurologists seemed to be of the same mind that the damage was probably permanent but there was a very slim possibility that it would start to regenerate. We finally chose to have a spinal cord stimulator implanted that would interrupt the signals to her brain and she would feel the pain more as vibrations. This surgery was done at the end of December 2009. This did help relieve some of her pain combined with strong pain medications. Unfortunately, she did not have the use of her foot and had to learn to walk with a leg brace that went to the top of her thight and crutches. Jordanne is 5"10" and all legs, so this was a very large brace which she hated and avoided using whenever possible.

In January, Jordanne had healed enough from the 3 surgeries to enter the Cediranib trial at NIH. Flying from the West Coast to the East Coast every two weeks was a real strain. Every trip involved getting a wheelchair at the airport, getting bulkhead seating on the plane and trying to get to the DC early enough to catch a shuttle to NIH. The blessing is that Jordanne is under 25 so she qualifies to stay at the Children's Inn on the NIH campus which is free. This is a very special place for children that are being treated at NIH and thier families. Most of our visits we have been able to stay there. We were so happy when Jordanne was able to get on the one month schedule.

Being 22 years old at the time of diagnosis Jordanne has not let this slow her down too much. The worst was always the pain and inconvenience of not having the use of her foot. Then in February she noticed a clicking in her jaw. She told me" I think I broke my plate." Of course my answer was that she couldn't have because it's titanium. So a trip back to New York confirmed her suspicions that the plate had broken. We received permission for her to go off of the cediranib while she had surgery. Dr. Delacure ordered a part from Switzerland to try to fix it and scheduled another surgery in March. When we went back for the surgery, he had to break the news to her that he was unable to get the part so he was going to try a couple of different fixes. Unfortunatey, he was not able to fix the plate so had to make the decision to just cut it back so the ends wouldn't rub against each other and leave it as is. Jordanne by then had started to see some shrinkage of her lung tumors and so he recognized that getting back into the trial was more important. She is able to chew most foods even with only one hinge of her jaw. God always makes a way.

Jordanne has had about 35% shrinkage of her largest lung met. It started at 1.6cm and is now measuring between 8 and 9mm. The rest of her lung mets are all about 1mm or smaller. None are in any locations that cause problems so she has very little problems on that end. Currently she is showing stable disease.

Most recently, around August 2010, Jordanne noticed that she could wiggle her toes and move her ankle a bit. We made an appt with her neurologist and she confirmed that the nerve was regenerating. Praise the Lord! It has now been about a year since it was damaged and we can expect about another year of nerve growth so there is a great potential that by October 2011 she can throw away her latest leg brace which is a below the knee brace that is easily hidden under pants. Since the first movement in her foot she has continued to gain strength in her leg and is now able to walk with just the small brace, no crutches, and just a slight limp.

You can read more of her story under the Cediranib clinical trial section of this site.
We are really happy with Jordanne's currently physical progress and the stable disease while on this drug. Of course, she still battles the side affects of Cediranib, primarily diahrea, headaches, high blood pressure seem to be the worst of it for her. We are just happy that she has not had any new tumors in the year since she has been diagnosed.

Dotty - what a story. Every ASPS case is unique but metastases to the jaw are so rare and you without any knowledge of sarcomas being able to make a connection and to find the primary in the thigh - it is just amazing. The nerve regenerating a year after it was though to be dead - it is so amazing too and will give such a hope to hundreds people that will eventually read this story here on the board by performing the relevant search on the web.
I have a question re. the scanning - Jordanne should have once a year MRI for the possible brain mets surveillance, but since there is a metal plate in her jaw she can not have it, right? Does she get at least a brain CT scan at times?

I think you did the right thing with aggressive surgery. Sure, it didn't go perfectly - but I strongly believe that it was the best solution.

I hope so much that she'll get her foot back. I'm guessing if she exercised, it would stimulate the nerve? Swimming, and biking come to mind

Cediranib couldn't have come at a better time for your daughter. When I got this disease back in 2003 there weren't that many options. Now we have sunitinib, cediranib, better ablations, and Dr. Rolle of course. One could say that these are much better days to be an ASPS patient than even just a few years ago

Olga and Ivan, thank you for the responses. I think everything happens in God's perfect timing and under His watchful eye so we go through this journey with faith and peace of mind. As on all journeys there are many up and down battles.
Unfortunately, Jordanne just got hit with another downside. She planted her weight ( light as it is) on her damaged foot to kick something with her good foot, just goofing off and fractured 2 bones in her bad foot. When they Xrayed the foot they said that she has osteoporosis and that's why she has compression type stress fractures. Unfortunately, I wasn't there with the doctor only the nurse after the fact so don't have all of the information yet. It didn't get Xrayed until Friday afternoon so we have not talked to her doctors yet.
My question to those of you who are in the medical field is "Can you have osteoperosis in just one area of the body?"
Could she have this condition in the foot because she had a very large vascular tumor in her left thigh for probably at least 2 years sucking up most of the blood in that leg? Then having the sciatic nerve damaged and lack of circulation to that foot for the next year. She has had more exercise and circulation in that foot just within the last few months and we had seen great improvement in her movement and much less swelling and pain. Now, of course she is back to pain meds and crutches.
We don't know how this may affect her position on the Cediranib trial.
I will keep you updated once we find out more.
Thanks,
Dotty

I'm sorry, Olga, I did not respond to your questions.
Jordanne has never had a brain MRI. She had a brain CT a year ago when she was diagnosed and nothing showed. I did not think about the fact that she has titanium in her jaw for MRIs. She can't have MRI on her abdomen because of her spinal cord stimulator. She gets CT of her jaw, chest, abdomen and thigh every 2 months at NIH. They also do PT of the entire body. She was unable to have the PT this last trip because the machine was not working.
I'm not sure how bone scans work but assume they are more like the CT?
It seems that ever since she was diagnosed we went right into surgery mode, complications from surgeries and then directly into the Cediranib trial. We have had very little time to actually dwell on ASPS itself. Since her lung mets are so small and not located wnywhere near anything major, it has just been this lingering thing that we don't think about too much. I keep hearing the word vigilance but don't think we have been as much as we should be.
I definitely appreciate the wealth of information that I glean from this forum. I could only find one reference to osteoperosis and that was on Brittany's experience after being on steroids. So, I can only assume that Jordanne's case is as unique as she is.
As a mom, I am continually amazed at her strength and perserverance. She just seems to roll with the punches and comes back with a joke and a smile on her face. God has given me such a beautiful gift in this young woman for a daughter.

I am so sorry to hear about this new development.
I do not know much about this osteoporosis situation but there are few leads I would investigate:
1. Find out the bone density in the other leg - if there is an osteoporosis as well it would mean she in fact has systemic osteoporosis - blood work for calcium needs to be done as well. There are other causes of bone loss (disuse) which are not the results of the osteo, but other factors such as prolonged bed rest.
2. If she was not putting the weight on that leg for a long time - may be this is the reason for the osteoporosis, as doctors always say that the best way to avoid it for example for the post-menopause women is to have the weight bearing exercise in addition to having enough calcium and vit.D, the same goes for the long term flying astronauts - they develop low bone density because of the no-weight bearing conditions. They loose about 20% of the bone density during the flight.
3. Find out from the people on Cediranib trial if they have other cases of the osteoporosis, may be this is the less known side effect of it. There is a role of parathyroid hormone in osteoporosis and since it is being lowered in the people by the cediranib, can it also cause the osteo?
4.Was she on the long term steroid meds at any time of her treatments?
5. What are the side effects of the long term use of the spinal cord stimulator, does it use any slow release meds or is just a block? Could this device cause the spinal cord injury?
I found this article and there are lots of hints:
http://emedicine.medscape.com/article/322204-overview

Crap, that's just terrible. As far as I know, bone density decreases in astronauts due to the fact that the bones receive no load in space. I think it's definitely possible to have localized osteoporosis, similarly to localized muscle atrophy. Also, because her muscles in the leg are probably very weak right now, much more load and stress is placed on the bones.

When the fractures heal in some months, you should get a physiotherapist who is familiar with recovering from this type of injury. Perhaps, someone who has worked with older people who tend to suffer from osteoporosis? Jordanne would need to do gentle, low-impact exercises such as water running, swimming, etc to build up the strength.

I think this should have no effect whatsoever on the cediranib trial.

Jordanne sounds like a wonderful young lady, Dotty.

Sorry to hear about the setback, but hopefully they will also be on the mend soon and also this alerts her doctors about the need for managing her osteoporosis. I went to my regular doctor physical this year and was surprised to find I was vitamin D deficient (surprisingly common where we are - in Seattle) - Vitamin D (sunshine) is also good for fighting cancer and there are supplements of course - but the docs can also check a blood level. 'K' doesn't like being out in the direct sunshine, but we made more of a conscious effort to put her out in the sun (with sunglasses) since we discovered low vitamin D in our family.

To my knowledge, osteoporosis is increased by disuse and denervation and maybe that is what contributed to her situation.

We're keeping Jordanne and you in our prayers,

'F'

p.s. To my knowledge, most surgical plates put in the body are Titanium these days, which shouldn't interfere with MRI scans too much (sometimes it sets up an echo artifact - but you can still get pretty good pictures). With different doctors and trials and surgeries, it is a good idea for every patient to be as vigilant as possible - mark on their calendars the need to check brain MRIs at least once a year etc. The earlier you can detect things, the easier they are to treat. Each oncologist has so many patients they keep track of that it is easy for them to get lost which screening tests need to be checked on a routine basis. Clinical trials doctors usually only check what they need to for their trials.

thanks Ivan and 'F',
Being a Northwest girl Jordio loves the rain and clouds. She does have big skylights in her house but probably not the same. I'm starting her on some calcium with vitamins D and K until we hear from the doctors.
So sounds like the titanium shouldn't give her trouble with the brain MRI. We have been going round with her oncologist about the need. He thinks if she doesn't really have any symptoms it's not really necessary. However, he'll recommend whatever we push for. I don't know how many actual patients he sees these days. He took Jordanne on as a favor to the family because he doesn't take new patients. She really likes him but I'm getting the distinct impression we need to start pushing a little more in certain areas.
Thank you so much for the prayers, that is the best thing anyone can do.
Dotty

Dear Dotty,
I am so very sorry to hear about Jordanne's new setback just when her foot was beginning to heal and she was no longer needing to use her crutches and pain meds. It is so heartbreaking and unfair that just innocently "goofing off" and doing the normal things that young people like to do resulted in an injury, but with her incredible resilence and detemination I am sure that she will be on the mend again soon. I don't know if osteoporois is a possible side effect of Cediranib, but I do know that Dr. Sawyer has advised Brittany to try to do as many weight bearing exercises as possible since Cediranib can cause muscle loss.
I have to strongly disagree with Jordanne's oncologist regarding his seemingly nonchalant attitude about not needing a brain MRI if the patient isn't having any symptoms. Brain mets need to be diagnosed at the smallest possible size because by the time there are symptoms, the brain met may be too large to safely resect or to successfully respond to Gamma Knife. Given the nature of ASPS to metastasize to the brain in some patients, it is essential to have a brain MRI at least once a year,and if the patient has a history of previous ASPS brain mets, brain MRI's need to be done every three months. Tragically, we lost a patient (who had communicated on the now defunct TAAASPS forum) because after having one brain met resected, his oncologist didn't schedule him for another brain MRI for five months during which time he developed more brain mets which were by then too large to be successfully removed or treated. I don't want to frighten you, but I just want to emphasize the critical importance of being vigilant with regular scans. In addition to yearly brain MRI's, full body bone scans should be done once a year also. Unfortunately, this disease can, and does, play evil tricks, and even if mets are stable in one area of the body, they can be growing in another area.
With the exception of Jordanne's painful and debilitating new injury, I Hope that she is doing well, feeling good, tolerating the Cediranib, and continuing to gain some weight. Please tell her that all of us Hesses are thinking of her and sending her gentle hugs and best wishes for a very speedy and full recovery from her injury.
With special caring thoughts, healing wishes for Jordanne, and continued Hope,
Bonni

From what I read about the availability of the Vit.D from the sun exposure - the article was from Boston done by Harvard's people - it is impossible to develop enough vit D there from the Nov till Apr. They are located at the 42.4°latitude, since we are here in Vancouver are located at the 49 °latitude (with Seattle being pretty close to it), we live in the situation when this interval is even longer. By some studies the vit.D from the supplements can not completely substitute the sun effect, there is probably more in it then just vit D. In the situation when we are able to produce vit D (summer months, sun vacations) we only need to make a few 20 min exposure periods during the day when 30-40% of the body exposed - its like people in shorts with the covered head will do just fine, although previously unexposed parts of the body are thought to be doing better job in producing the vit D (newbies people on a nude beach are probably doing better job then...).

I wish I had found out that I was vitamin D deficient at the beginning of summer rather than the end. Also we had been rather compulsive about sunscreen and that may not have helped either! Sun through skylights won't be enough either.

I told to take supplements and the doc will recheck a vitamin D level in 6 months.

Maybe this is one of those good things to find out - having good levels of vitamin D is supposed to help the body's natural immune response against cancer.

Hello again,
Additional information regarding the importance of brain MRI's for ASPS patients is that Brittany's last three brain mets were found during one of her routine three month brain MRI's with her having had no symptoms to indicate that they were there, and a fourth met was found during the very specialized MRI that was done prior to the Gamma Knife procedure once her Gamma Knife frame had been attached. Because the brain mets were found and treated before they became symptomatic, the Gamma Knife procedure was thankfully apparently successful in killing the four mets which are now no longer visible on Brittany's MRI scans.
With more special caring thoughts and continued Hope,
Bonni

Hello all,
New update from my little china doll. She was doing quite well with the fractures in her feet, off the pain meds in about a week and getting around with a walking boot. NIH said the osteo showed up on her scans as being all through that leg and even in the knee cap. They were concerned that she could break a bone ver easily. Bingo!
As a young woman in her twenties wanting to live as normal a life as possible, Jordanne went out with a friend for Halloween weekend. Someone knocked the stool over she was sitting on and the boot got caught on the rail of the stool resulting in another fracture. This time in her tibia. She will see an orthopedic specialist tomorrow for a decision on what we can do immediately to improve her situation. She is currently in a cast and NIH has asked her to go off of Cedirinab until she sees the doctor here. Although she is currently in a lot of pain, she is still in positive spirits and positive that it won't take too long for this to heal.
One step forward two, three or more back. Even in this we are thankful that God has a plan. Also thankful that it wasn't a large break or in her thigh.
Dotty

Dotty, I am so sorry to hear that. This is a big dilemma with the young adults with the cancer - one one hand they want to live their life as normal as they could and for the young people being normal means to be careless and on the other hand they can not afford to be careless. Is osteo only found in this one leg, did doctors compare the situation in the other leg. I mean did they figure out is it systemic or local.