Kevin from California, US (Dx Feb 2012) - RIP Oct 17, 2014

[wangcns]

Yeah, the anomaly in his sternum turned out to be a cystic legion. Common enough, but the doctors panicked, of course, because of the ASPS.

I mean, if there is a good thing, it did make us seriously think about the possibility of systemic treatments, or what other options we would pursue if resection was not an option...but yeah. I'm glad it's not ASPS in the bone. This whole experience has accelerated our timetable for returning to Germany to clear out the left lung. Our oncologist has said it's better not to be too aggressive with this disease, that sometimes it's necessary to wait and see what move it makes, but it doesn't change the fact that Kevin still has innumerable tumors in his left lung. If his right lung had at least 129 tumors (and I'm not kidding myself that Dr. Rolle got every single one of them, no matter how good he is) how many are in his left?

If this disease is to spread (and of course, I'm hoping it won't) I think we would both like to know that his tumor load has been reduced, if at least buying us some time (and not lying awake at night worrying about his current tumors growing with every passing day) to consider our next option. Or maybe, just maybe, a few months without worrisome developments... Kevin's diet has been pristine for the past two months and we're hoping and hoping that this, along with the resections, will slow down the progression of this disease. We're prepared to do systemic treatments if we have to...but even our oncologist has said he has mixed feelings about what's available out there.

[Bonni Hess]

Dear Connie,
Thank you for the additional diagnostic information on the benign anomally in Kevin's sterum. Is any treatment required/recommended for a cystic legion, or will it eventually dissipate and disappear? I am so happy that Kevin and you will now be able to move forward with scheduling Dr. Rolle's laser resection of the numerous mets in Kevin's left lung. As you have said, this will help to reduce the tumor burden in Kevin's body and strengthen his immune system to enable it to better fight this challenging disease and Hopefully prevent disease progression and the need for a systemic treatment until a permanent treatment and cure can be found. Regular scans, pro-active treatments such as laser resection of macroscopic and microscopic lung mets, being as knowledgeable and well informed as possible through research and shared anecdotal treatment information remain the most effective way to manage ASPS at this time, and you and Kevin are to be commended for all that you are doing in this regard.
Take care Connie, give Kevin my best wishes, and please keep the Board updated as you are able.
With special hugs, caring thoughts, healing wishes for Kevin, and continued Hope,
Bonni

[wangcns]

No treatment required for the cystic lesion, the doctors said it wasn't an issue.

We've heard back from Dr. Rolle and we will be going back to Germany in three weeks to do Kevin's left lung. However, it looks like new mets have already appeared in the right lung that was operated on in July. When Kevin is recovered from this next surgery, we're think we are going to try system treatment, as it appears the mets are regrowing, even with surgery. At least the current tumor load will be cleared out from Kevin's lungs and even if the new mets are growing slowly, we'll have some time to see if any of the TKIs or similar drugs will have an effect in slowing the growth and spread of mets. Our oncologist has suggested Pazopanib as a starting point, but we're going to wait until after the surgery to make any decisions.

Heavy heart, but this isn't anything unexpected. And at least we're not going to Germany in the dead of winter, which is some comfort to me since I'm California-bred and dread the cold. Gotta keep fighting the good fight...

[Olga]

First you have to make sure that the reading radiologist understands the physical aspects of the laser assisted surgery and the shape of scars that it lives behind, they may look as a progressing small mets Kevin had before.
As for trying something from the systemic treatment field (versus local treatment), you have to educate yourself on the subject and a terminology they use more and be really clear in what exactly are you planning to achieve with the treatment.
For example, there is a new article re. Pazopanib phase III trial:

Pazopanib: In Advanced Soft Tissue Sarcoma.
http://www.ncbi.nlm.nih.gov/pubmed/23072642
Where the benefit they define is the significantly prolonged median progression-free survival relative to placebo, but the benefit for the median overall survival was very slim, people lived 2 months longer with pazopanib than with placebo, although this between-group difference was not statistically significant.

[wangcns]

Actually, it was Dr. Rolle who looked at the CT scans and saw the growth of tumors, so unfortunately it's definitely not some radiologist misreading the scans. If only. Our oncologist had not specifically told us tumors were showing up in the operated lung, he had simply encouraged us to return to Germany as soon as possible and then recommended we begin discussion about systemic treatments when we get back and Kevin is completely recovered from this second thoracotomy.

We've talked with a number of people about going on the drugs. Our first inclination was to try and stick with surgery, but it looks like we may need to pursue other options. Definitely will have that discussion with our oncologist about what exactly we want to try and do in terms of treatment. Thanks for the link, Olga. I will definitely read up on systemic vs local treatments before we pursue any action. We started having that discussion with our oncologist, but since Germany is coming up, I think we've decided to deal with one thing at a time and not get too aggressive with treatment, until we see how the ASPS reacts to this second surgery. Best case scenario, we'll see a slowdown in met growth, but it looks like it's continuing with a 1-2 mm growth every 2-3 months. Granted, we've only had 1 CT-scan since the first surgery, so maybe we'll see measurable slow down after this second surgery. *shrug* At this point, I don't know. I guess we just have to wait and see what this damn disease does.

[Olga]

Connie, how is Kevin's pain level and lung capacity, is the operated lung completely expanded?

[wangcns]

Yes, as far as he can tell he's recovered from the surgery. It's been roughly 3 months since his surgery? He's been running and exercising with no discomfort. The only pain he ever experiences is if someone shoves hard against his surgical scar (a dog we were babysitting dived into his side, painful!). He's lost nearly 30 pounds since before his last surgery, so I'm a little worried as I know the surgery takes its toll, but otherwise he says he feels good.

[Olga]

Good to hear that his horrible after thoracotomy pain Is gone. It looks like he still eating healthy, Dr.Rolle is going to be impressed:(

[wangcns]

Hey guys,

I'd love to hear everyone's opinion on this. So as of today, we are considering delaying our left lung thoracotomy to try drug therapy. We've already booked our flights to Germany and have confirmed a surgery date with Dr. Rolle, but for a variety of reasons, which I'll list in just a bit, we want to delay the surgery and incorporate a six month course with TKIs.

This is from our oncologist:

"There are three TKIs that would be reasonable (Sutent, Pazopanib and sorafenib). They all hit roughly the same targets to one degree or another and have similar side effect profiles. I would recommend sutent as the best responses have been seen with this drug, but if there are issues with insurance we could always do pazopanib as it is FDA approved for advanced soft tissue sarcomas."

This is a very difficult decision for us, so let me list our reasons why and why not. I would love to get input, any advice, references to additional reading, anything to help with this decision.

Factors:
-Mets already regrowing in resected lung
-Mets all seem to be growing at a slow and steady pace, roughly 1-2 mm every 2-3 months
-None of the current mets in lungs are in dangerous locations and are relatively small, just like in his right lung. Nothing over 10mm, if even that.
-Brain and body scans are clear of other mets

Pros:
-Opportunity to compare resected lung with left lung treated only with drug therapy
-Window of opportunity to compare treatments with relatively little risk in terms of timing, we may not have this chance again as the disease progresses further
-Sooner or later, we need to explore our drug options...the earlier we establish what works for Kevin and what doesn't, the better
-Preserve left lung tissue visibility, at least for right now (right lung has a great deal of scar tissue, difficult to see new growing mets)
-Resection does not appear to be slowing down met growth in lungs, roughly 3 months since right lung thoracotomy and mets are growing/regrowing at same rate
-Both our surgeon and our oncologist favor a course of TKIs before returning to Germany. Considering our oncologist was the one who encouraged us to reach out to Dr. Rolle in the first place, we are taking that into consideration.
-Kevin doesn't have to get another thoracotomy so soon

Cons:
-many risk factors to drug therapy
-dealing with side effects; currently Kevin enjoys excellent quality of life other than the expected discomfort and pain from undergoing a thoracotomy, drugs may change that
-possibility of more serious side effects
-rebound and other risks of getting off the drugs, including the possibility that he may always have to take it
-no proven effect on long-term survival, many drugs still experimental or in the clinical trial phase
-surgical resection has more data indicating correlation between multiple resection of mets and long term survivability with this disease

I think that's about it. Obviously, there are other pros and cons, which I've separated as I feel when faced with the question of Kevin's health and survivability, are lesser considerations, such as the impact of the Germany trip on our lives in terms of work, expense of the trip, recovery time (both physical, mental, and financial) from the last trip only 3 short months ago...etc.

Again, please share whatever thoughts you guys may have on this course of action. And I may need to call Air Berlin and see if they will refund our tickets or at least let us delay the trip >.<

[mikko]

I would go to Germany now or may be a little later ( if Dr. Rolle says it´s ok). I would NOT start systemic treatment at the moment.

[wangcns]

I think we would still do the surgery, but in this case, not for at least another 6 months. I think we want to try a 6-month course of TKIs to see if it will help with the regrowing of mets, before we go back to Germany.

[Olga]

Hard choice. We had to answer all these questions ourselves at some point as well, and we are going by the surgery/ablation route since 2005 with the great quality of life and only few nodules left - 1 is proved and active and a few that are not growing at all so they are might be the scars. It does not makes us an experts or give our opinion more weight than other people or your oncologist, but it is my belief that if you guys take your time to answer to all the questions to yourself, then the choice becomes obvious.

Some random notes that I would post to clarify the subject:
1. What is the goal in "trying" TKI? There are no known cases when any of the above mentioned drugs worked indefinitely and the resistance always developed. When resistance develops, the mets start to grow faster so there is no overall survival advantage. It si not like you tried something and if it doesn't work, you just stop it. The consequences of the developed resistance are huge. So there are 2 most possible outcomes in trying TKI:
- it may not work at all (i.e. the factors they aim to shut down are not expressed in Kevin's tumors), then the time is lost and the tumors grew, but at least there is no faster growth;
- it may work for awhile (i.e. slows or stops tumors from growing for awhile), but the resistance develops at some point and the tumors develop the ability to grow using the diff. routes and escape, it usually means faster growth.
The interim outcome like Brittany is now having is very rare and she is using cediranib, which is the most potent TKI known.

2. Kevin may loose the possibility of the second surgery because if the mets start to progress faster in both lungs or if there are any other mets appear elsewhere besides of lung Dr.Rolle will refuse taking Kevin for the surgery, I know many cases when he could not take the patient for the surgery anymore. There is also wash out period before of the surgery after TKI, it increases the length of the time Kevin would not have the surgery and therefore the probability that he misses the chance to have one.

3. Kevin can always try above mentioned drugs after the surgery, but there is a possibility he would not be able to have a surgery after the "trying drugs" (whatever it means). In my opinion, phase 2 trial is more appropriate for after the surgery if there would be no other means of dealing with the mets locally. Scarring will resole as the time goes and the picture is going to be more clear.

4. The surgeries with multiple lung mets are rarely able to achieve the mets free status but they most definitely could be a first step toward it. They also buy him a time until the new combination of the drugs would go into a trial. It is already known that TKI are the drugs that are active in ASPS and are able to induce the temporary slow down, the combination trials are needed to compound this effect to provide a permanent result that would build on this ability. If he tried (and failed) any TKI, he can not go on cediranib or other TKI trial according to inclusion criteria (at least for awhile).
I hope it is somehow helpful and adds you more food for thoughts.

[Ivan]

Systemic treatment (which will likely not work) should be the LAST option. We've had people die or become unresectable because they were messing around with clinical trials when they had other options available. Beware of this possibility. Wishful thinking is not something an ASPS patient should ever engage in in any way, shape, or form.

You have an option between something that works for sure, and something that probably doesn't. Furthermore, second surgery is usually much simpler than the first.

I think the best philosophy with this disease is cut early, cut often (if necessary).

[wangcns]

Thanks everyone for your input. It's been very, very useful. We're still trying to make a decision but your responses have given us a lot to think about and consider, as well as do more research on. We asked our doctors for clarification on what the goal of going on a six-month course of TKIs would accomplish and the answer we received was that it would be nice to get an idea of the response prior to resection, to use as comparison data. Dr. Rolle recommends having the resection first, and then go on TKIs afterwards if we want to pursue systemic treatment...

Ugh, so hard to know what is the right thing to do. Haven't called the airlines to cancel our tickets yet...

[Kkgriffith]

hey all,
Thanks for all the responses and support, this forum is giving me the tools to make a decision that I feel more confident about.

So I have decided to continue with the planned surgery of my left lung by Dr. Rolle.

Why I made this decision:
-Delaying this surgery in hopes that the TKI's will work perfectly and save me right away is not the way to go
-having less tumors if and when I decide to go on TKI's will buy me more time if the drugs have a "rebound" effect.
-the longer I wait, the bigger the tumors get and the more damage will be done to my left lung during surgery
-(please correct me if Im wrong)I also haven't heard of an ASPS patient that has had long term success with TKI's (Noah did say there are other Sarcoma patients that have had continued success, but that's all I know of)
-guys like Ivan who has had multiple procedures and still maintains a great quality of life gives me strength!

I haven't discounted the use of TKI's all together, I might just hold off until surgery is not a viable option for me anymore.

Thanks again to everyone for all their posts, I will also continue to post on our blog during the trip.
-Kevin