Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello, my name is Willem, 28 years old and I am from the Netherlands. Two years ago I went for the third time to the doctors with my lump just above my left knee. The last two times they told me it might be a lipoma. They didnt get me as concerned that I went for further research (I had to plan it myself). As it grew bigger I went for a third time to the doctor. When I went to the doctor I said I already had a lump for more than 10 years, so the doctor itself was also not very concerned. Unfortunatly I was totally unexpected diagnosed with ASPS, the end of June 2013. A CT scan showed that I had dozens of metastatis in my lungs. In both lungs I have one big lump, one is about 4 cm and the other about 2 cm.

After 3 months of waiting to get more insight in the growth, I could join a trial of Crizotinib, which i do still do until now. Surgery or radiation was not the most appropriate treatment, because of the numbers of metastasis in my lungs. In the first couple of months the trial seemed to work, it seems stable, however sometimes it was 2 mm bigger and the next time 1 mm smaller. Now, two years later I can say it probably helped to slow down the growth (this is however not sure, because you never know what happened without treatment), but it is not totally stable. In two years the biggest lump in my lung showed growth of about 1 cm. The doctor is quite happy with this result but to me it is still growth and I'd rather see it is stable or went smaller. The doctor still advises me to go on with this trial, because when an other treatment fails it might be not easy to join this trial again, as the study closed already. He advises me to wait till the drug is approved. But he doesn't know exactly when the drug might be approved, that can maybe take one or two years.

I thought it may be a good idea to get some expert opinion from this forum:) In my position, what should you do? Go on till the drug is approved or try something else?

Another thing which I want to get more insight in are the experiences with the lump in the upper leg. Now I can still do everything (I do several kind of sports) but comparing it with two years ago I got more pain from time to time, because of the (slowly) growing tumor. Might it be a good idea to do surgery or radiation? Or is better to wait until I get serious problems (disabled) with the leg, as the doctor said? The doctors said the risk of hitting nerves or blood vessels is quite big, so a surgery might destroy more than it heals...

Glad to hear from you:)

Hello Willem,

My name is Martin. I'm ASPS patient my self. very good that you have found this forum. I've been on the forum less then a year but there are people with stories that you can read with more than 15 years of battle and experience that for sure will post messages.

Form what I read and experience the radical surgery of primary site is very very important. Your doctor seems that is waiting for you to get to a very symptomatic state so that a surgery is done.

Even that you are on a trial, the removal of the primary will reduce the burden of your body to fight with this damn disease. Maybe without primary effect from the trial will be better.

Radiation is not the best option with ASPS as many people will confirm but surgery is the most effective.

Please ask and pursue a surgery on the primary if possible to achieve negative margin.

Willem

My name is Debbie and our son is Josh
We've been with the forum since late 2012

Martin has some very good advice .

Love
Debbie

Hi, welcome here.
Misdiagnosis is very common with ASPS - it is so slowly growing that most drs confuse it with the benign lump.
Re. Primary surgery - if you wait longer it may invade/destruct the bone, I would resect it for sure - the surgery often goes better than expected with ASPS.
Re. lung mets - send the scans to Dr.Rolle in Germany, many of us went there to have their lung mets resected when were told by local drs it is impossible, my son was operated by Dr.Rolle 4 times to clean the lungs, his topic is here:
http://www.cureasps.org/forum/viewtopic.php?f=4&t=66

More info about Dr.Rolle and laser assisted surgery is here:
http://www.cureasps.org/forum/viewforum.php?f=51

Thank you all for the useful advises! I will propose it to my doctor. And I'm going to send the scan results to dr Rolle. I'll keep you updated about further developments.

Dear Willem,
I am so sorry for your ASPS diagnosis and for all that you have been through with the initial misdiagnosis, which is as Olga said, unfortunately very common with ASPS. I am grateful that you found your way to this Web site and that you have reached out on the Discussion Board for information, input, and support from our ASPS Community.
I agree with Olga and Martin that resection of your primary tumor should be/needs to be pursued and done as soon as possible, and I strongly disagree with your doctor that you should wait until the tumor becomes symptomatic. What is the current status and size of the primary tumor and has it increased in size during the past two years and since your participation in the Crizotinib Trial? Unfortunately,because of Clinical Trial protoco, surgery to remove the primary may disqualify you from continued participation in the Crizotinib Trial, but I personally think that it is more important for you to have the tumor removed to reduce your body's tumor burden and help strengthen your immune system so that your body can better fight this very challenging disease. Also, reducing your body's tumor burden may enable any future systemic treatment to be more effective. During my 14+ years of ASPS experience and extensive research, observations, and networking with other ASPS patients and their families, I have found that the majority of the ASPS patients who did not have their primary tumors removed had repeated failed responses to systemic treatments and/or tragically ultimately lost their courageous battles. Additionally, if you are considering laser resection of your lung mets with Dr. Rolle, it is my understanding that he will not accept patients for treatment if they have any other tumors in other parts of their body. I will be anxiously awaiting your next update about your treatment decisions and plans following your consultation with your doctor, as well as Dr. Rolle's review of your scans. Take care dear Willem, stay strong, know that you are not alone in this difficult battle, and keep in touch as you are able.
With special caring thoughts, healing wishes, and continued Hope,
Bonni Hess, mother of now 33 year old Brittany diagnosed 14+ years ago in 2001 at age 19

Thnx for your reply Bonni Hess, it gives me even more the conviction that I have to remove the primary. The maximum diameter of the primary is now measured to be about 10 cm. It has increased with about 2 cm in the last two years.

Hi Willem,

Like you, I knew I had a lump in my thigh for about 10 years but was told not to think anything of it. I agree with the others that removal of primary would reduce the tumor burden on your body, especially since it's increasing in size.

~Jen

This morning I had the first meeting with the orthopaedist. He told us about the risks and the profits from a resections of the lump in my thigh. The lump has grown around a big blood vessel and is lying next to an important nerve. Also it has grown against a bone. Resection is a possibility, but they said it is hard to completely remove the tumor without having too much risk of damaging nerves and blood vessels, muscles and bones.

My question is if you think that resection of the tumor is still the best option if you know that the tumor will not be removed completely. They say that removal of the tumor can possibly improve the effect of medicins, but it has never been proven. Removal increases the probability of being able to walk within a few years. But there is a possibility of complications, which is relatively small if the lump is not removed completely, but bigger if the lump should be removed completely.

Glad to hear from you again:)

WilL wrote:This morning I had the first meeting with the orthopaedist. He told us about the risks and the profits from a resections of the lump in my thigh. The lump has grown around a big blood vessel and is lying next to an important nerve. Also it has grown against a bone. Resection is a possibility, but they said it is hard to completely remove the tumor without having too much risk of damaging nerves and blood vessels, muscles and bones.

My question is if you think that resection of the tumor is still the best option if you know that the tumor will not be removed completely. They say that removal of the tumor can possibly improve the effect of medicins, but it has never been proven. Removal increases the probability of being able to walk within a few years. But there is a possibility of complications, which is relatively small if the lump is not removed completely, but bigger if the lump should be removed completely.

Glad to hear from you again:)

Hi Willem,

The tumor in the leg is truly scary however the chances of it being encased and able to be surgically removed with good margins are good at this time. Good by the fact that it has not invaded the bone? I assume the orthopedic surgeon is an oncologist?

ASPS however is a very vascular sarcoma so the surgeon need to be prepared if removed.
Did the surgeon say anything about vascular feed to the tumor?

If the tumor can't be removed with complete margins the chance of retreating it if growth happens is maybe thru cryoblation? Ask your doctor about how he would retreat the tumor if need be.

How are your lung functions at this time?

Take care
Love
Debbie

Hi Debbie,

The surgeon is indeed an oncologist, he is a specialist in bone and soft tissue sarcomas, so I think he seems the right surgeon. However he never treated someone with ASPS. We didnt especially talk about the vascularity, do you mean he has to be aware of bleeding?

We didn't talk about retreatment, that's a good advise, I'll bring that in.

My lung functions are the same, I can't feel anything wrong, but the biggest lump again shows very little growth in 3 months...

Wiilem

Since he is a oncologist and sarcoma doctor I would guess he already knows the vascular situations however it certainly never hurts to talk about that topic with him.
An MRI with and without contrast will show lots of detail of vascular involvement as I understand.

Love
Debbie

Thnx Debbie. Another question is the use of radiation before surgery, the surgeons (and other specialists) are thinking about that. I saw some topics on that on this site, with not so much responds:) Is there any experience about the effect of radiation of the primary?

Furthermore my onlologist will try to get the Cediranib trial to the Netherlands, which I hope he will manage. We decided to stop the trial of Crizotinib, since the tumor has grown more than 20 percent since the start...

-Willem-

Olga had discussed with Jolie the idea of pre and post operation using radiation.
I typed in "radiation of the leg" in search.


http://www.cureasps.org/forum/viewtopic ... +leg#p5621

When the onc radiologist saw our post op report he wanted to treat Joshua's right lower thigh with radiation . We were given a 25% chance of reoccurrence and we opted not to do radiation. The collateral damage and the possibility of secondary cancer was enough for us to say no thank-you.
ASPS is radio resistant so unfortunately the patient needs the highest , safest dose to even begin to treat the tumor.

Debbie

Types of radiation treatments

http://www.cancer.org/treatment/treatme ... -radiation

Dear Willem,
Thank you for the updated information. I am so sorry that there are so many possible difficult complications with resection of your primary tumor, and am very perplexed/frustrated that the doctors did not remove it two years ago when you were first diagnosed and before it had grown to impact the blood vessel and the bone which now makes resection more difficult. Regarding the doctors considering radiation of the tumor prior to surgery, unfortunately radiation has not proven to be very successful in shrinking ASPS tumors since they are so radiation resistant, and I do not personally know of any patients who have had successful shrinkage of their primary tumors with radiation treatment. Perhaps the majority of the tumor could be removed followed by post op radiosurgery or ablation to try to destroy any remaining tumor tissue, but I still strongly feel that as much of the primary as possible should be resected to reduce your body's tumor burden and prevent the continued dissemination of tumor cells into your bloodstream from the primary. As I have told you, based on my 14+ years of extensive ASPS research, observations, and experience, I do not know of any successful systemic treatment responses when the primary tumor is still present, despite what your doctors are telling you that "it has never been proven that removal of the tumor can possibly improve the effect of medicins". Also, I am wondering if your monitoring scans include a brain MRI and a full body bone scan, both of which need to be done if they haven't been. I am sorry that you have now been taken off of the Crizotinib Trial due to more than 20% increased growth, and think that Cediranib or one of the promising new PL-1 immunotherapy drug combinations might be worth exploring, but not until your primary tumor is addressed and Hopefully successfully resected to try to ensure the most successful response to a new systemic treatment.
Take care dear Willem and keep in touch as you are able.
Reaching out to share special caring thoughts, healing wishes, warm friendship, and continued Hope,
Bonni