Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello, Cureasps.org members! My name is Josh and I was recently diagnosed with ASPS (September of 2012). Here is a brief personal introduction:

I am currently living in the Midwest United States (near Kansas City). I have lived here my entire life, attended some college, screwed around wasting time, just being a general derelict. During this directionless time in my life, I met and befriended a wonderful woman, and our friendship eventually evolved through dating in to a wonderful marriage. We have a 4 year old daughter, and the two of them have given me every reason I will ever need to stop wasting time and make something of my life. I am now 32. Two weeks before my diagnosis, we had just bought the house of our dreams. As you can imagine, this disease has really brought a lot of stress in to our lives.

Now, a brief history of my case:
My primary tumor was on the front/right side of my right thigh, and I mistook it for a muscle knot for quite a long time before seeking a doctor's opinion. Luckily, he directed me to a skilled sarcoma surgeon who ordered an MRI of the site and then removed it completely with no damage to my leg to speak of. For this I am very grateful. He followed that with a PET scan of my whole body, which revealed suspicious lung nodules. A CT scan verified this.
A month later, a VATS was performed, and 3 nodules were removed, the largest of which was 6-8mm (this was also the largest nodule shown on the CT scan). Biopsy revealed ASPS mets. I have several remaining mets (oncologist has stated he has not counted how many, but thoracic surgeon said that there were too many to remove and still preserve lung function).
Now I am learning about treatments and figuring out how I want to attack this disease. My wife and I traveled to NIH/NCI in Bethesda, MD for a consultation on joining the Cedirinib trial. My onc found the trial for us. He also wrote me a prescription for Sutent in case I did not want to participate in the trial.

I just got a second CT scan done on Friday (2 months after initial CT scan) which will tell us the progression of the disease, and am awaiting results. NIH has said that eligibility for the trial is contingent upon measurable disease progression, or the presense of a met greater than 1 cm. None of my mets are > 1cm. I had an MRI of my brain done also. The report says that there are no mets present, but they did note bilateral lesions consistent with possible multiple sclerosis. Which brings me to my first question: could these lesions actually be mets?

After reading several stories on this site over the past weeks, I am hoping that I can consult with Dr. Rolle. I do have very good insurance here in the States (again, I am inexpressibly grateful as there were extended periods in my life where I was entirely uninsured), but I am imagining that they will still not cover a procedure performed in Germany. If anyone has any advice on getting insurance approval for this procedure, it would be very much appreciated. If I have to pay for it, well, we will find a way. I am absolutely determined to live to see my daughter graduate from college. Amazing how quickly your goals can change from saving up and eventually retiring with money in the bank to just staying alive to see your kid grow up.

I am very grateful I found this site and thankful to the founders who set it up and maintain it. As terrifying as this diagnosis is, I am glad to read survivors stories and see that I am not the only person battling this.

I would very much appreciate some advice about recommended treatments from fellow patients. My onc was assigned to me by my surgeon, and, while I am sure he is just busy with all of his patients in addition to having never treated metastatic ASPS before and feeling overwhelmed, he seems fairly unhelpful. He doesn't really satisfactorily provide us with useful info (for example, I asked how many mets they saw in my lungs, and he didn't know) and his personality doesn't really mesh well with mine (he always seems somewhat inconvenienced by my questions and in a hurry to be done with our meetings). If anyone knows of an onc experienced with ASPS in the Kansas City area, we are very open to suggestions.

I will update with a more accurate timeline of my scans if that would be helpful, and if anyone has any advice, please share.

My current plan is to go on the trial if accepted. I would like to consult with Dr. Rolle, but will he be willing to review my scans remotely, or will I have to travel to Germany for a consultation? I have used all of my vacation and sick time at work and am trying to avoid more time off if possible. If Dr. Rolle and the trial are not options, then my last resort will be a Sutent prescription, or, if the doctors think it is a good idea, do nothing for a while until progression can be measured. One important detail is that if I start Sutent, I will be permenantly ineligible for the Cedirinib trial.

Feeling a little overwhelmed right now, and our onc is pretty distant and difficult to talk with, so I could really use some advice right now. I should have more info in the morning once I get the results of the newest scan. Wish me luck

Thanks so much for reading, and offering any advice you can.

Hi Josh, welcome to the board no one would like to be on:(
You are doing fine in how well you already navigate the situation and taking charge of the treatment planning, some people spend months and years in denial loosing the valuable time.
To answer your questions:

- do not rush into the cediranib trial yet. We recently discussed the problems with the TKI trials and off label treatment in Kevin's topic here:
http://www.cureasps.org/forum/viewtopic.php?f=4&t=728
read it and ask again, you have to understand what is the general problem with the TKI accrued resistance, what clinical trial is and what they consider the clinical benefit, you might not like it. There are the situations when clinical trial is the best choice from all available, but you have to really be sure that there are no other options left;

- Dr.Rolle will (and prefer) evaluate the feasibility of the laser assisted resection in your case if you send him a CD with the latest scan - preferably with 2 consecutive scans, all info about how to send it is located in the
Board index ‹ Metastatic Disease Treatment ‹ Lung Metastases ‹ Laser assisted surgery
here:
http://www.cureasps.org/forum/viewforum.php?f=51
some people posted here how they got approved for the insurance paying for the surgery for them;

- you do not have to ask your oncologist what is the situation with your lung mets, you have to get the official written report every time after the scan done, it is your right. Request the report and tell us what does it say, because depends on the lung mets number you might have other options for the local treatments;

- I do not know what the sclerotic changes in the brain look like, I would get another MRI to verify the findings or to have the first one read by the sarcoma specialist, you can ask to get a second opinion at the place with the sarcoma departments like MDACC in Texas or MSK in NY etc. It is very important to move faster with the review as the small brain mets are treated with the high rate of success with the radiosurgery.

Hello Josh,
I'm so glad your case of ASPS was detected and diagnosed and you have options for treatment. I'm also glad you found this board where so many concerned and caring people have a lot of experience with dealing with ASPS and are willing to share.
Prayerfully,
Trixi

Good news. I spoke with a nurse from NCI/NIH about the scan I just had done. They do not see any measureable growth. She also informed me that they can see about 8-10 mets sized 6-7 mm, so now I have a general idea how many there are. I kind of think that there are more tiny ones that don't show up on CT scan--just a feeling I get.
Olga:
Thanks for the information and for the site! I am not eligible for the Cedirinib trial as of now. My other option is to start taking Sutent, but I do not think that I will do that...1) it will make me ineligible for the Cediring trial in the future 2) the side effects sound pretty bad. My biggest concern is that it will metastasize somewhere else, ie brain or liver if I am not treating it somehow, but as I understand it, this can still happen while taking Sutent.
Trixi:
Thanks for the warm welcome!

I will be sending my 2 CT scans to Dr. Rolle to see what he recommends.

I did forget to mention that I have been to MSK in New York, but all they really did was confirm my diagnosis. We talked with Dr. Schwatz there, but he was pretty swamped that day and it was a very quick meeting (it was 2 days before the hurricane rolled in, so maybe they were busy preparing?) I'm not sure if they actually looked at my MRI scan or just read the report, but I am assuming they looked at it since we had it sent.

I am strongly considering going down to MDA in Houston also.

Hi Josh,

Welcome to the ASPS boards, though of course I'm sure we all wish we were meeting under better circumstances. My husband was diagnosed with ASPS earlier this year, with a primary tumor in his left pectoral muscle. It was removed, without affecting any of the surrounding muscles, but the disease had spread to his lungs. He has no other metastases that we know of, though there's always the chance they are too small and haven't been seen yet. His last few tests have come back clear, other than mets in his lungs.

We are currently in Germany right now, on our second thoracotomy at the Coswig hospital. Professor Rolle operated on Kevin this past Wednesday and removed 120+ mets from his left lung. His first surgery, which was in July, was on his right lung, and he had 129 tumors removed. I can only tell you our experience, but so far trying to get insurance to cover this surgery has been difficult. I highly recommend you look at Olga's links; we've been using them to file our appeals, but the brunt of the matter is that the insurance policy my husband's work provides does not cover international surgeries, unless it were a matter of urgent life or death (as in, we were traveling in Germany he got hit by a car and needed a surgery to save his life).

I recently added him to my work insurance, but not before I had a long talk with their benefits manager. They didn't promise they would cover it, but they did say they would be willing to review such a surgery if we were to file a claim. Obviously, it's too late for the two surgeries we've already gotten, but it's worth the added cost if they are willing to consider future surgeries, especially seeing as how currently, my husband's primary insurance is not even willing to look at the case (there aren't even grounds for appeal, because of the plan my husband's employer purchased.)

What we ended up doing was hosting a fundraiser on this website:

http://www.giveforward.com

If you'd like to see my husband's, it's http://www.giveforward.com/ihazcancer.

We've been paying for the surgeries out of our own pocket and we don't expect to see the money back anytime soon, so I understand and share your concern about insurance coverage for the laser aided lung surgery. I know it's not the best answer, but insurance coverage really depends on what kind of plan you have, who you have your insurance plan with, etc etc. But it never hurts to try and use the resources on these forums to make out a damn good case for yourself--though in the meantime I do really recommend holding a fundraiser.

We also had our own dilemma before embarking on this second surgery, which Olga linked to, but we've ultimately decided on surgical resection, at least to clear out both lungs, over systemic treatment. It might be that when we return to the States and Kevin has recovered from this second surgery, we may consider a 6-month course of TKIs, especially since his lungs have been cleared of literally hundreds of tumors, but that's a decision we're deferring on making until Kevin is fully recovered. I don't know what to tell you except sort of repeat the advice that we've collected from our doctors, friends who have gone through similar difficulties, and other ASPS patients we've talked to. Be skeptical about anything being the "one cure" for this disease, whether it be drugs or surgery, or what not. Educate yourself and consider all your options, so when you do make a decision, it's one that you feel good about. There's just not enough data to make definitive judgments about what is the right course of treatment over others, though obviously some treatments have met with more success than others. Talk with people (like on this board, looks like you got this one covered) who have ASPS and listen to what they have to say--everyone's experience of this disease is different, so nobody's story is going to give you the right answer, but it will help you arrive at a decision that you will be comfortable with. As the mother of one ASPS patient told me, the important thing is not to look back and tell yourself, oh I should have done that, or maybe we shouldn't have done that, but to make the best decision possible with the information you have available.

Good luck to you and your family in this fight. You can do it!

Love and healing,
Connie

Luckily, he directed me to a skilled sarcoma surgeon who ordered an MRI of the site and then removed it completely with no damage to my leg to speak of. For this I am very grateful.

REALLY lucky Shake his hand more than once.

I just got a second CT scan done on Friday (2 months after initial CT scan) which will tell us the progression of the disease, and am awaiting results. NIH has said that eligibility for the trial is contingent upon measurable disease progression, or the presense of a met greater than 1 cm.

The bad news is they are very likely growing, just slowly. You should act on the presumption that they are growing, and once confirmed, have a plan in place.

The report says that there are no mets present, but they did note bilateral lesions consistent with possible multiple sclerosis. Which brings me to my first question: could these lesions actually be mets?

Without knowing more, the fact that they are bilateral makes it less likely, in my opinion. I think an MRI of the brain is a rather definitive scan for mets.

After reading several stories on this site over the past weeks, I am hoping that I can consult with Dr. Rolle

Considering the lack of systemic treatment, this remains the best option.

I am very grateful I found this site and thankful to the founders who set it up and maintain it.

No problem. We also benefit greatly by you sharing the information with us.

If anyone knows of an onc experienced with ASPS in the Kansas City area, we are very open to suggestions.

In all honesty, they won't tell you anything that's not already on these boards here. I hold the opinion very strongly that if Dr. Rolle says "Yes", that is by far the best option. Don't even think about starting Cediranib or anything else before sending him the scans..

Dear Josh,
I am so deeply sorry for your ASPS diagnosis which brought you to this Board, but I am grateful that you found your way here, graciously shared your information, and reached out to our ASPS Community for advice regarding treatment. You are to be commended for rebounding so fast from your numbing diagnosis and primary tumor resection, and moving forward so quickly with your research and your pro-active approach, both of which are extremely important in this battle with this very challenging disease. I completely agree with the input and advice which has been offered by the other Board members regarding the wisdom of pursuing laser resection of your lung mets rather than a systemic treatment with Cediranib or Sutent at this time. While Cediranib has thankfully been very successful in stabilizing the progression of our daughter Brittany's disease and dramatically shrinking/destroying her multiple and widely disseminated tumors for the past 43 months, it has unfortunately not shown that type of sustained long term success for most of the other ASPS patients who I am aware of who have undergone Cediranib treatment. The negative side effects of Cediranib are chronic and very debilitating, the risk of rebound and aggressive disease progression are significant, and we know that it is unfortunately not a permanent cure, but due to Brittany's rapidly progressing and aggressively spreading disease and an inoperable, untreatable, and Life threatening pancreatic met four years ago, we had no other option. Sadly, Sutent has not been proven to be very successful in significantly shrinking tumors and providing long term disease stabilization for any of the patients on this Board, and tragically several of the patients who were treated with Sutent have now devastatingly lost their courageous battles. At your early stage of the disease with no other mets than those in your lungs, and with no currently available permanent cure for ASPS, it seems that removing the CT visible and non CT visible lung mets with laser resection to reduce your body's tumor burden is the best treatment option and choice. I am not a doctor, but based on my eleven and a half years of ASPS experience, research, networking, and observation, I personally feel that systemic treatments like Cediranib or Pazopanib (which seems to be showing more success than Sutent) should be reserved for patients with rapidly spreading disease, unresectable and untreatable mets, and/or when there are no surgical, ablation, or radiosurgical options available to remove/destroy dangerous mets.
Regarding your oncologist situtation, I strongly encourage you to seek and find another oncologist who is a Sarcoma specialist preferably with ASPS experience, one who you feel comfortable with and confident in, one who readily provides you with the information which you need and request, and as Brittany says, "one who truly cares about you as a person and not just as a patient and who has your back". Unfortunately, I don't personally know any Sarcoma/ASPS specialists in the Kansas City area, but Dr. James Butrynski at Dana Farber in Boston is an excellent Sarcoma oncologist with ASPS patient experience (including Brittany when he was at SCCA in Seattle), and who meets the critically important criteria which I have mentioned. Wherever and with whoever you receive your oncology care, always insist on personally reviewing the scans with your doctor and on receiving a copy of both the radiologist report as well as a CD of your scans. It is vitally important to be as well as informed as possible and very vigilant in fighting this extremely rare and challenging disease.
Please know that you are not alone in your battle and that those of us on this Board are here to help you in any way that we can with shared information, input, and strengthening support and encouragement. Take care Josh, stay strong, let Hope lead you through each day, and keep in touch as you are able.
With special caring thoughts and continued Hope,
Bonni Hess, mother of 30 year old Brittany diagnosed at age 19 in July 2001

Big news. Dr. Rolle reviewed the info I sent him and has deemed that all of my mets are operable. I have procured funding and am set to leave this Thursday for an operation on the following Monday on my right lung. With work, etc. I had to act quickly or face having to wait until April or later to go for this operation, so here we are
I am quite nervous, having never left the States, and I usually like to have more time to plan, but it is what it is. Another worry I have, and maybe someone with an onc who they are better able to converse with can explain this to me, but I feel that the best option would be to take a round of TKI treatment, and then once that has killed some of the mets, have Dr. Rolle remove the rest. My thought process is that the cancer "seeds" or bad DNA or whatever it is (I am not a doctor/biologist lol) is already in my blood, and I think (perhaps wrongly) that the TKI treatment may kill whatever is in my blood stream so that there is less chance of recurrance. I really wish that my onc was more communicative; I feel somewhat cheated not having someone with a medical background to bounce ideas off of. We are going to see another onc Tues before we leave, but they are far less experienced with sarcoma (and of course, with ASPS).

I plan on reading everyone's posts on what to expect while in Germany. I also posted a question in the financial section about costs http://www.cureasps.org/forum/viewtopic.php?f=40&t=758

Josh, I moved the Costs of Dr. Rolle's surgery topic to a Laser assisted surgery forum:
http://www.cureasps.org/forum/viewforum.php?f=51
and will comment on that subject there.

About the TKI treatments. There are 2 available now off label for ASPS - sunitinib and Pazopanib - but there is very little information about their role in ASPS and how use them best and if they have any influence on the floating or dormant ASPS cells so I see no reasonable ground for the oncologist to assume anything here. At least Ivan's oncologist does not like to speculate based on a theoretically expected mode of action - there are the clinical trials that deal with it, for the rest of the cases the honest answer is "I do not know" when there are no publications of clinical trials results to support any assumption. Saying from our experience, despite of sunitinib or pazopanib being available, she has not given us an advice to use them at least now when other treatment options are still available - surgery or local ablation, we just met yesterday. She is an oncologist specializing in sarcomas.

Very few publications that are avail. about the role of these 2 TKI in ASPS suggest that they can have a stabilizing but temporary effect on an existent tumors with suggested mechanism of action of damaging their blood supply (which is absent in a floating or dormant cells setting). So going from the publications, you could achieve the temporary stabilization in the growth of the lung mets if they respond or progressive disease if they don't as there are cases when ASPS patients did not respond to TKI. If the disease progresses you may loose the window of opportunity to have the lung surgery with Dr.Rolle.

I think this is some of the best news an ASPS patient can hear. Say to hello to Dr. Rolle for me

Josh, my husband just told me that when he was in Amsterdam two weeks ago he could not use his credit card with the chip to pay for the train tickets as the booth - he inserted his usual VISA credit card with the chip and the message came that it could not accept the cards with the chip, it only accepted a gift VISA prepaid card he happened to have on him at the moment. I do not know if this is a typical situation or not, just decided to let you know. I used the credit card without the chip in Coswig and Dresden few years ago, no problem as long as it had 4 digit pin number.

Here we are in Coswig. We flew in to Munich and met my wife's cousin who has graciously allowed us a place to stay following the hospital release. It is very difficult communicating here, but luckily the people are all very nice and patient. We actually managed to get our Vodaphone internet working, despite having broken the SIM card and tons of other problems. The gentleman at the Vodafone store in the Kaufland here in Coswig was a life-saver in that respect, as internet is our primary means of communication back home. One interesting bit of information I discovered is that the Amazon Kindle I brought along to read actually gets free 3G signals anywhere in the world where 3G coverage exists. The browser is super simple, but gmail works in simple HTML view and it doesn't cost $15 per MB to use

The nurses and other staff have been very nice as well and helped us a lot along the way.

Anyway, Dr. Rolle viewed the scan we brought from a week ago and informed me that the number of mets present is now at least 30. I'm very confused as to what is going on, since this wasn't mentioned in the radiologists report that we received, unless I misread it. Also, this seems to be out of character for ASPS. Dr. Rolle said he will review the scan itself with us tomorrow, so I hopefully will then know how to read one and be able to review my old scans to see if there were other things that were missed.

I am very confident we made the right decision coming here, especially if the disease is taking off suddenly like this. I really hope that now is the time that will lead to a control on this disease.

Hope everyone is doing well, and thank you to all the members who answered our questions about travel, etc. I apologize for not responding; we just have been super busy getting ready for this trip, but know that your help is eternally appreciated!

I will update soon. Guten nacht!

Hey Josh,

So glad to hear you and M made it to Coswig a-ok. I totally know what you mean by the bewildering language barrier thingie. I spent a lot of time on the first trip wishing I spoke German and being frustrated at my inability to communicate or understand. Uh, speak any Russian? That will probably get you farther than English in East Germany

Anyway, Dr. Rolle viewed the scan we brought from a week ago and informed me that the number of mets present is now at least 30. I'm very confused as to what is going on, since this wasn't mentioned in the radiologists report that we received, unless I misread it. Also, this seems to be out of character for ASPS. Dr. Rolle said he will review the scan itself with us tomorrow, so I hopefully will then know how to read one and be able to review my old scans to see if there were other things that were missed.

So Kevin had a very similar experience. For example, it was from Prof. Rolle that we learned that the tumors in Kevin's right lung had started to regrow/were growing, also the number of mets identified was much higher than what our doctors told us in the US. We also learned from Rolle, and not our American doctors, that the surgeries have not slowed down the disease at all, and that there is actually marked growth in both lungs despite the surgeries. I don't really know why we had to learn that from Rolle and not our primary oncologist, and part of me is wonders why that is, whether we're deliberately not being told details because they feel it doesn't matter, or because they missed it, I really couldn't say. But what I can guess is that perhaps Prof. Rolle, since he specializes in thoracic surgery, has had decades of experience reading lung CT scans and removing mets/tumors from them, so maybe he's able to give a more accurate assessment?

I don't know if I would freak out just yet about the higher number of mets, most likely Prof. Rolle is just more badass at identifying mets. I don't know if you've read Ivan's story, but apparently pathologists have been trying to kill him for the past ten years I guess that's why they say it's really good to learn how to view your own CT scans and identify and track mets and met growth.

Imagine our shock when we were told Kevin had 30-40 tumors prior to surgery, only to have Prof. Rolle open Kevin up and take out 120+ tumors. This last surgery was 140+. So the possibility is there that visually, they will be able to identify far more tumors than can be seen through a CT scan, which really is still an imperfect mapping tool and subject to user error, etc. Though of course I hope that's not the case, but that's what happened to us.

Isn't Amazon Kindle the best? I realized my 3G still worked in Germany for free on the 1st trip, so I didn't have to worry about loading up on entertainment so much the 2nd time, as I could just buy books through Whispernet. Internetz, foreverz!

Hey, if you see a nurse there named Karla, tell her Kevin and I said hi! She's got short black hair, blue eyes, and is one of the few C-ward nurses that speaks English.

Before my first surgery, I had around 25 mets visible on the scans. Dr. Rolle ended up zapping and removing 86. I wouldn't be surprised if your numbers end up being similar.

The first surgery is the toughest, make sure to read my day by day experience posted in the respective forum to know exactly what to expect. I think being prepared will make it easier.

GOOD LUCK!