Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi everyone
I am Jussi from Finland. My dad has been more active on this site in the past, so I have been able to focus on my studies. I am now 25 years old and was diagnosed with ASPS in the summer of 2008. I had my main tumor in my shoulder and several metastases in my lungs. At first I was treated with vai-pai-chemo before surgery, because my doctor didn´t have any idea how to treat ASPS. This treatmet shrunk my main-tumor a little but had no effect on my lung metastases, so they changed the chemo and then changed it back to vai-pai again, but at this point it stopped working, so I left the universal hospital to try experminetal treatment with oncolytic viruses. At this point i haden´t had any surgeries. Virus treatment had very little effect.
6/2009 I had my main tumor removed from my latissimus dorsii muscle in Finland. After this surgery I still had the full use of my arm. 2009 -2010 I had 2 thoracotomies performed by Prof Rolle in Germany. 6/2010 PET scan of the whole body revealed that my leg pain was caused by 5 cm tumor in my proximal femor. This was treated with RapidArc- radiation with very small dose only 30 Gy. Radiation didn´t work so we had to remove the tumor with a surgery in Finland. Tumor was very vascular, so they blocked it´s arteries before the surgery, which went very well. My proximal femor was removed and so was the tumor. I now have endoprosthesis. I still limp, but it is mostly a cosmetic problem. 3/2011 I had rethoracotmoy of my right lung.
6/2011 I tried experimental interferon alfa treatment in Finland. First with 3 million units 3 times a week. later with 6 million untis 3 times a week. 1/2012 my situation was stabile for the first time. After six months with interferon I had psychosis. I knew from Roozendaals case srudy that this was a possibility. Luckily 5/2012 I was able to return to my studies and stop antipsychotic medication. After I stopped interferon cancer advanced again, so 7/2012 I had rethoracotomy of the left lung. In the 6/2012 Prof Rolle saw 4 – 5metastases in my right lung. At first RFA was considered. I had it before in 2011. However one tumor was crowing close to the heart, so heat was not the best option. Instead we decided to try cryoablation. (RFA and cryoablation are radiological operations) We contacted Dr littrup, who works in America. 9-10/2012 he was able to freeze 3 metasteses from my right lung in the first operation and 2 more in the second operation. Cryoablation was very simple operation with little pain and I didn´t stay in the hospital more than a day or less.
I hadn´t have any operations after 2012. I had over 100 metastases removed by Prof Rolle´s laser-surgery. I think 112 was the exact number. There is still some suspicious nodules in my lungs, but they haven´t gown, except one, which is growing in my right lung. It grows very slowly. It is now around 8mm. I can´t say with certainty, because after 2014 my situation had been monitored only with MRI-scans. MRI is at least as good as CT for brains and abdomen, but for lungs CT is more accurate. MRI should still detect tumors that are bigger than 10 mm, so i think it is enough, because 12 mm is ideal size for cryoablation. I have MRI-scans of my brains, lungs and abdomen in every 4 moths now, because there is no radiation. Before I had my Ct-scans every 6 moths. I still life high quality life. i have completed first five years of medical school. (Training is 6 years in Finland) I can still run with a speed of 7-8 km/h. I go
to physical therapy to treat muscle tension, so my pains are very tolerable. I survive with ipubrofein once or twice a week. My lifestyle is not the heathiest, so it doesn´t explain why I hadn´t have any new tumors after 2012.

Jussi - I am so happy that you finally got your story here, it takes awhile to get used to that stupid sarcoma Dx when you are so young, isn't?
I am also so proud to be able to help you with some decisions that were made at time by your parents - hard choices made that allowed you to survive. The effect of IFN is very intriguing - it stopped the progression and probably arrested smaller/dormant mets - my idea is that this is the cause that you have no progression since 2012 - in my (unqualified) observation IFN works better the the very small mets so if the rest (palpable size, big enough to detect by Dr.Rolle and on the CT scan) are surgically removed, the micromets are still arrested or dead. I know another case when the IFN treatment was done for a year and stopped due to psychotic issues, and then the multiple bigger lung mets were resected but the smaller ones were left there as the surgery was done in a conventional way (not Dr.Rolle so the surgeon could not remove the tiny ones without taking to much lung tissue) - the girl doing pretty well as well. And the smaller mets in the lungs are still detectable but stable. So the IFN is an interesting option initially, when there are multiple small mets suspected (and they always should be suspected even when not visible). We have seen evidence that it does not work on bigger tumors, but worth trying in the beginning for sure - may be staying in a low dose makes more sense to avoid the psychosis (although Ivan is going to say that it is incorrect to make any assumptions on a few non-comparable cases).

Yes it is possible that interferon is the reason why only one tumor is still growing and not more. At first I didn't believe it, but it has been over 3 years, so it might not be just a coincidence. If I remember correctly interferon has small antiangiogenic effect, but it is very difficult to say if interferon's immunomodulative effects work on microtumors. Also there is no way to know correct dose.

P.S: I wouldn't have survived without you and Ivan, so thanks.

Hello Jussi
You are truly a inspiration to all here! We sure needed a positive statement here on the forum today to let us all feel hope in having ASPS and seeing a light at the end of that proverbial tunnel.

It was truly good for my soul to hear from you

What are your feelings on the PD meds that you are hearing across the big pond ?
I'd love to hear when you get a moment.
What kind of medical profession are you perusing ?

Also I'm not sure you knew but along with the PD drugs friends in China with ASPS are using interferon to aid in "boosting" the immune system

http://www.cureasps.org/forum/viewtopic ... eron#p8807

I did not know about about a PD drugs, before I was diagnosed 20 brain tumors 2 weeks ago. I hope I can try them combined with Votrient after my radiation with gamma knife ends. 2 tumors are most likely too big to destroy with radiation, but if we get the rest of them out I hope I can find a surgeon willing to operate the last 2.

About my medical interests: I really love old people and severe diseases. I was considering becoming hematologist, but recent advancements in oncology got me to reconsider. I was planning to start working on PhD about cancer this year, and then maybe specialise in oncology. Now I obviously not gonna do any of that. I maybe could still work as a doctor with my current condition, but not after my symptoms worsen.

Hi Jussi

I truly sorry to hear of the brain mets
I would definently consult a surgerion.
So you've had no treatments since your Inferon?
Cyro and surgeries have been your primary treatments thus far?
How many tumors will the doctor be focusing on with the gama knife?
Let us know how you are doing when you are able

Sure will be thinking of you

Keep that optimistic attitude going
Love
Debbie
Ps what is vai-pai-chemo?

No I did not have any medications after interferon. I think they try to radiate them all. I am thinking about intermittent fasting during radiation, but I am not sure if it only worked on mouses or is complete nonsense. Vai pai is routine chemo used in sarcomas.

Jussi - the side effects from radiosurgery to multiple brain targets could be severe - it depends on the individual ability to deal with the intracranial swelling effectively and if there is a radiation necrosis. You do the search on the Pubmed to read on the subject but in general there are few things you can do to prepare for the effectively deal with the side effects AFTER the radiosurgery:
1. When you get the radiosurgery date, notify the neurosurgeon who already consulted you and knows the case before and right after it is done with the details (your parents can do it after). Even if he against of it, so if you get emergency hospitalized he is able to perform the decompressing surgery effectively or even remove the bigger treated met if it is accessible (it will not be that vascular anymore after the treatment). Tell him that in case there is an excessive brain swelling or an intracranial bleeding you or ER might contact him.

2. Notify the family doctor before and right after the radiosurgery. Make sure there is a record of what is done in the electronic patient file if you have it in Finland and all the paperwork at home with you at all times so if something happens they can read it immediately.

3. Find out and educate yourself and the parents about the acute side effects and signs of the collapse approaching. You should always be under the supervision after the radiosurgery or implement the system of active feedback checks when you contact the parents at the preset intervals and if they do not hear from you they contact you, there is probably some app avail. to help with it.

The number of brain mets the docs can treat by the radiosurgery at once has increased and now they talk about the overall planned treatment volume not the number as a cut off, but by any means a radiosurgery for 20 mets is an extreme plan and you have to be prepared.

Hi Jussi,

very sorry to read about the latest updates regarding the brain mets and I sure hope that you are on a good way to start dealing with them.

maybe this is a stupid question but seeing that you are doing a brain scan every 4 months, did all these 20 mets showed up suddenly? were not they noticed before when you could have treated them all one by one?

Stay strong as you are
Martin

The tumors were already present in march scans, but they did not actually took brain scan then. They only took mri of torso. The mri had my brain visible, but with very poor quality and radiologist missed the brain tumors. I was an idiot and imagined they had looked the brain and ignored my symptoms.

Sadly they can only radiate 13 tumors. After that I hope to ad Keytruda, if any doctor is willing to write prescription. I am already on Votrient, but no response in two weeks. Lets hope it will start working. Two tumors are too big to be destroyed with radiation, but if miracle happens and all the rest get destroyed they are theoretically operatable with surgery. Sadly I know that there is 99 % change that I only have few months left
P.S. I had very minor symptoms after first radiation day. 3 days left.

Jussi,

is it possible that you try to get in some Cediranib trial?

maybe it will have some effect to reduce the size to treatable also on the bigger mets

I dont know how to get in ceridanib trial. if We can find a way to get it. I would love to try.

Morning (state side) Jussi

How big, other than the 2cm tumor ,is the other tumor ?

I hope today brings you closer to some more comforting answers.

Our son Josh had a 16mm ablated over a year ago by way of LITT. It continues to shrink thus far . It was treated by SRS initially and a follow up treatment with microwave heat aided the tumor to break down more efficiently

http://www.cureasps.org/forum/viewtopic.php?f=8&t=1031
Love
Debbie

THANK YOU SO MUCH. If they agree to treat 2 big ones and we can come up the money and travel there I could survive way longer. Of course I am getting ahead of myself. I need to talk to my dad first. He is napping, because he is tired of me having radiation.

Jussi - so you are saying that two bigger mets are going to be left untreated by the radiosurgery? You need to keep pressing - work with the neurosurgeon to have them resected. There are other neurosurgeons locally who could agree to resect them even if the one refused, especially if the bigger ones are symptomatic. You need to fight for it, it they are accessible, it is better than LITT now in a brain already overloaded with the necrotic tissue from the radiosurgeries - talk to the brain surgeon again as soon as the radiosurgeries are over.