Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Merry Christmas,
This is Karen Imm writing. It has been a while since I have posted an update. For those of you who don't know, I will give a brief background. I was dx. with ASPS in Jan. 2000. Had surgery, chemo (adriamyacin and ifosfimide) and intra operative along with external radiation. My leg had never been the same. I am disabled and have fractured my kneecap 4 times. Then I was diagnosed with breast cancer (totally unrelated) and had surgery and radiation for that. Then they discovered lung mets in 2003. Nothing was done at that time. In 2004 the ASPS went to my heart and a lime sized tumor was removed via open heart surgery along with some lung mets. In 2005, I had thoracic surgery to remove a "ping pong ball" sized tumor from my trachea, along with more lung mets. Then in Jan of 2006, I had another open heart surgery to remove more tumor from my heart. Then in June of 2006, they found another tumor in the right hilum (between the heart and lungs) along with continued lung mets. This tumor is inoperable. So we tried IMRT and it seems to have helped. I have also tried Interferon and some other "chemo cocktails" in the past with little success.
I checked into the Dana Farber study (went out there) but they felt that another major surgery is too risky for me considering the open hearts etc... So we just returned from TX where there is another trial. At this point, I do not qualify because my hilum tumor has not grown significantly since Sept. (likely due to IMRT) and it needs to be growing to be in the study. The big concern is that this tumor is covered in arteries and close to major airways. It is currently right up next to the pulmonary artery. So if it grows, it will cut off the blood supply and oxygen to my body. So we are desperately wanted to keep it from growing. And thank God, it is O.K. for now. I will return to TX in 8 weeks for more scans.
I can tell you that I am thankful for every day and love spending time with my family. As you all know, this is quite a journey and I have certainly learned a lot. I am thankful for the overwhelming support I have gotten from friends and family.
I also appreciate this website and want you all to know that you are in my prayers.
God Bless,
Karen Imm

Dear Karen,

Thank you so much for such a detailed update. You are a very strong and positive person!

I wish you a good year. Hopefully the year where we find the cure for ASPS.

Greetings ASPS friends,
I am sorry it has been so long since my last post. Please know that I check the updates regularly. All of you are always in my prayers and thoughts. It is always so helpful and encouraging to know how all of you are doing.
I wanted to ask for some advice. As you know, my metastatic ASPS has "staked it's claim" in my heart, trachea and lungs. I have had 3 open heart surgeries and had my entire right ventricle rebuilt. I have also had a tumor by my trachea removed and another inoperable tumor in the mediastinam (sp) area radiated. I was on the ARQ 197 trial in Boston approx. 2 years ago. It seemed to help with my smaller lung mets, but my larger tumor grew significantly, so I came home and had the lower lobe of my left lung removed. In the past year and a half, we have been monitoring smaller growths (7mm) in my right lung. My latest scan showed that things were growing again. The right lung met had grown from 7 to 10mm. However, my bigger concern is that they noted a tumor behind my heart, by the esophagus. A friend of mine, who is a general surgeon, read my report and said he did not think it would be operable. The scan before this one indicated something "vague" in that area and now it measures 13x9.3mm.
When I met with my local oncologist, who admits to not knowing much about ASPS, he only addressed the smaller lung met and did not even talk about the one behind the heart. To be honest, I don't think he read the report thoroughly. He never looks at the actual scans. When I saw a copy of the report, I called his office back and via his nurse, he said he thinks we should just wait another 3 months to check it again.
This doesn't sound right to me. My last heart resection did not have clean margins, I have a history of tumors in this area and with this new tumor growing relatively quickly (for ASPS) in a short time, I don't want to wait for 3 months. I have been having a few minor symptoms, cough and chest discomfort. When I mentioned these, my local doc said that this is a result of all my surgeries. I have worked with this same doctor for 9 years. He has usually been quite helpful and ready to refer to ASPS experts if necessary. However, with this new tumor behind the heart, I am not sure that I trust his judgment. Waiting 3 months doesn't sound like a good option.
Am I being unreasonable? I have contacted the doctor in Boston and will send him my scans, but I am just feeling kind of "stuck" right now. Not sure what my next step needs to be. I like it so much better when doctors sit down with you and show you the actual scans. My local oncologist has never done that.
Anyway, I am sorry to ramble. I just thought I would put this out to all of you and see what you think. I really respect the opinions of those of you on the board. You truly know what it is like to fight this disease.
Thank you in advance for any suggestions. My thoughts and prayers are with you all.
God Bless,
Karen Imm

Hello Karen,
I am sorry this desease has been such a fight and i am again sorry that you are dealing with a new tumor

I think that you should fallow your feelings and tell him how you feel and get a referal ASAP! I am a true believer in listening to the alarm in the head about things like this and not to ignore it... Call him and telt him to refer you out for treatment.

In healing hopes for all!

Amanda

Karen, hi, the symptoms you have - some cough and chest discomfort - do really look like a result of open chest surgeries you had, but it does not mean that you should just observe that tumor you have growing - you already have two consecutive scans for the comparacement so you should for an independent radiological review of them, to be compared side by side again by the other radiologists and thoracic surgeons, not the oncologist, it might be discussed on a tumor board later. Start from requesting the review (for a valid reason tell them that you want to apply for a cediranib trial and that they request the proof of progression). At the same time request copy of two last cosecutive chest CT scans burned on a CD, you will need it for the consultation, you can burn more copies at home if they are needed.
and If they see a progression, there are a few options that might be still avail. and it doesn't make a sense to wait here:
1. Cediranib trial in Bethesda. Good option but they enroll fast, so at least contact them (all the info is avail. at the cediranib topic).

2. Some type of radiation with the dedicated technology like Novalis might be still possible to use, the case needs to be evaluated by the Okunieff team. I do not remember who did the radiation last time.

3. Cryoablation might be still possible, contact Dr.Littrup he will need to see the scan.
As a substitute for cediranib (if by some reason you do not get on that trial), you can start Sunitinib, it is avail. off label with no trial, regular by prescription.

I think it is enough for a starter?

Hi Karen,

It's great to hear from you. I agree - follow your instincts. It also takes time to get into trials and lots of surgeons may refuse.

As I recall Clare's central tumor was quite large and causing pressure on her breathing - she responded well to Cediranib and has also done well having gone off the drug (important because can rebound). Sutent may also have an effect - but with less shrinkage and though less side effects.

I wouldn't think you would be a candidate for cryo because of its nearness to the heart and vessels - and the anatomy is probably distorted from the prior surgery.

Lucio had a large mediastinal mass that Dr Rolle (Germany) said he could not remove. He went to UCLA and Jay Lee took it out. Jay Lee did 'K''s first thoracotomy. You could consider sending your scans there.

Blessings, 'F'

Hello again,
Thank you all for your words of encouragement and wisdom. I really appreciate the information. I have contacted Boston (Dana Farber) and we will discuss options. I have also made an appointment with a local doc who will sit down with me and review the scans so that we can make some informed decisions.
I appreciate you all and continue to keep you in my thoughts and prayers.
Thank you,
Karen

Dear Karen,
I am so very sorry for the increased growth of your mets and for the diagnosis of the concerning new tumor behind your heart. I am sorry too that your oncologist has been so remiss in looking at your scans and in reviewing them personally with you. This is something which he should definitely be doing, and if he refuses, then I think that you need to find a different oncologist, and preferably one with experience with/or knowledge about ASPS or at least Sarcoma. I frankly don't understand his (inexcusable) laissez-faire attitude about waiting three months, especially with your history of heart mets and given the relatively rapid growth of this new tumor. I am grateful that you were pro-active in reviewing the radiologist report and reaching out to the Board for input and advice. I agree with everything that Olga, 'F', and Amanda have said. I am glad that you are quickly moving forward with seeking another opinion and more information so that you can make the most informed and best treatment decision as soon as possible. Depending on the outcome of your consulatations, I would strongly recommend that you consider a systemic treatment like Cediranib which could Hopefully help prevent progression of your disease and shrink/destroy your existing mets. In the meantime, please know that my most caring thoughts and best wishes are with you, and keep the Board updated as you are able.
With special caring and continued Hope,
Bonni

So what's happening? Did you try to enroll at the NCI for cediranib?

Karen,
Just wanted to add my encouragement in your upcoming treatment decisions.
I agree it is important to have a Dr. willing to sit down with you and discuss your scans.
Praying daily for ASPS cure.
Blessings,
Cindy

Karen,

Since talking to the Michigan cryo guys, I think it might be possible to cryo that one behind the heart obviously it depends on what exactly the scans show. I will email you off list I don't know if you get all these messages from the forum. And that seems to be not a really big deal. With cryo you can see the ice ball by CT so it is possible to control the size of the area affected. The size of that one was also not so big.

Blessings, 'F'

Hello Karen

My name is Debbie and I as I understand it, you've had some experience with heart scans.
We are hoping to understand how your treatment of heart tumors has progressed from your last discovery in 2010 .When you get a chance..

I am so sorry to hear of your maladies and ALL that you've had to deal with in your 13 plus years with ASPS and breast cancer. I really want you to know that I can't begin to feel your pain ,frustration and sadness that you've felt and must be feeling at times.

People ,who have know of you well before me, want to know how you are doing

With much concern and love

Debbie

This is some update I have received from Karen back in March 2013 and that I feel is appropriate to share as it was her intention:

"I was going to provide an update. As you may remember, I have had metastatic disease in my heart in the past. Now I have 2 growing tumors in my heart again. One is involved in the pulmonary vein and making breathing difficult while the other one is in the center of the heart. I have had to have a pacemaker put in and my local doctors have told me that I am out of options. My husband and I will be going back to Dana Farber Cancer Institue in Boston in hopes that a clinical trial might be available. We are just concerned about my heart being strong enough for the trial and also concerned that if the drug works, and the tumors shrink, that it would not create a hole in the pulmonary vein.
Karen."
I have not heard from them since then.

Dear Karen and family
All our thoughts are with you all
Much love
Debbie


Thank you Olga

Thank you for sharing this thoughtful update from Karen dear Olga. Karen's metastatic heart tumors are extremely concerning, and given the negative prognosis of her doctors regarding further treatment options and the insidious nature of this disease, I am very worried that there has been no further communication from her since last March. However, I am holding very tight to Hope that an effective treatment was found to shrink/destroy her heart tumors and stabilize the progression of her disease, and that she will post an update sometime soon.
With great concern, special caring thoughts, and continued Hope,
Bonni