Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello, my name is Ozden, 26 years old from the Netherlands. One year ago i went to the docter because some pain in the lower abdomen. After some test i was diagnosed with ASPS (august 2018) in the lower abdomen pelvis, with a tumor size about 10 cm. Also the CT scan showed alot of metastasis in both of my lungs. 3 big lumps, 2 of them about 2.3 cm and the other one 3.7 cm, also some small ones. The dr in LUMC Leiden told me to start with pazopanib. There was no difference in the size of the tumor in about 6 months. Because it didnt reduce the size of the tumor, the dr told me to try another medicine called Cediranib. I just started using it. Surgery, radiation wasnt adviced by my dr. because of the size of my tumor and the numbers of metastasis in my lungs.

I Thougt it may be a god idea to get some expert opinion from this forum. What should i do in my position?

Thank u so much

Hello Ozden
I’m sure sorry for your discovery of your pelvic tumor .
Can it be surgically removed with its location ?
Where are the larger lung tumors located ?

Thank u Debbie for your relply,

I'm not sure if its possible to remove the pelvic tumor. Although i really want to remove it, but my doctor told me its very risky since its too big and because i have lung metastasis.

My doctor want me to only take medicine and wait and see if it does something. but I'm not sure if thats the right thing to do. I really want to try everything i can and fight against this tumor.

the lager lung tumors are located at :
upper left lobe parahilair at position 163:23 mm
lower left lobe next to the aorta thoracalis descendes at position 246:23 mm

and the Primair tumor right in the small pelvic at position 526:106 mm
and the last one is the Ossal metastase in the right ilium wing with soft tissue extension at position 528:37 mm



Should i try and ask for a second opinion to remove the pelvic tumor? or should i keep taking the medicine that doctor gave me? Its really hard for me because i know no one with this kind of cancer and i know not much about the treatment of it.

And here are my scan of my lungs and pelvic

Hi Ozden, the tumor load looks very significant and basically it would be hard to find any dr that would agree to operate on a primary or bigger mets although the cryoablation or SBRT might make sense to control the bigger/most dangerously located mets. Also any size of primary or mets can be resected if there is a pain or quality of life problems with them present as a palliative measures. We usually advocate to remove at least few bigger ones as they might reduce the efficacy of the treatments by being immune suppressive and pro-inflammatory.
Th most active drugs in ASPS at the moment are ICI drugs - immune-checkpoint inhibitors- like Keytruda, Opdivo and few others. They are mostly avail. on the clinical trials yet, but some of our patients get them prescribed off label (my son Ivan is currently getting it off label in Canada but it was really hard to get approved). There is a sample application letter in Systemic treatments viewtopic.php?f=76&t=1482
Right now you are getting one of the drugs from the TKI class (tyrosine kinase inhibitor) - there are few others incl. cediranib which was very effective for one of our members here. Usually they show some response that last for awhile. You can try cediranib to see if there might be any response.
Having not removed big primary and other bigger mets might be immune suppressive, which is not a good thing if you start ICI drugs as they basically work by blocking the breaks the tumors apply on immune system by producing the false signals. So for the ICI drugs to be effective, the active immune system is needed - after the breaks are removed it has to have enough capacity to work. But there are strategies how to improve the response to ICI drugs and some of them include the treating the bigger tumors by the SBRT or cryoablation to create some dead tumor tissue in the body and to make it easier for the immune system to discover the tumors. So if you ever move to ICI treatments, you might ask for the local treatment addition in an attempt to potentiate the tumor recognition by the immune system.

Hello Ozdone

Have you determined by the pathology that this is ASPS definitely?
Our family ask 2 pathologist for a conclusive diagnosis .
We were also able to have our sons primary removed .

The usage of cediranib will be a good beginning I feel as well.
How many doses have you been given and when will you be given a scan ?

Olga and Debbie, both thank u so much for ur reply.

I will for sure continue with the cediranib en see with the next scan (16 may) if there will be any changes. I am taking the cediranib for a month now, 2x a day. Also i will discuss with my dr about the ICI drugs. I understand that not removing the primary and the other mets could be immune supressive, so i am in for any kind of treatment to remove at least something. Since i am not sure my docter will take any steps to remove anything, i am thinking about to get a second opinion. Is there any place u would recommend?

The pathologist concluded that its definitely ASPS .

Tumor burden is indeed significant. So convincing any surgeon to operate can be difficult. And honestly with any other cancer surgery might not be best option. Basically if t-cells learn to reconise cancer long lasting response can be achieved. Asps is indeed most sensitive cancer to check point inhibitors. Having access to check point inhibitors can be very difficult, but if you manage to have access to those drugs, there is realistic change,thatasps tumors will shrink radically and never grow back. In theory immunesystem can learn to reconise cancer while using tki-medicines or even whithout anymedicine. Sometimes surgery or ablation to one tumor can create abscopal effect. Rarely meaning, that immunesystem destroies cancer completely. There isnt yet enough scientific data to say, what is best way to activate immunesystem against cancer. But as a asps-patient my personal opinion is, that if surgical removal of all aspstumors isnt possible, then trying to activate immunesystem against asps using check point inhibitors is best.

Hello Ozden,
how are you and how did the latest checks go? I keep my thumbs up for the results.
As this is a worldwide forum I figured we are living relatively close, ;) Europe. I will be on vacation with my mum soon - in Noordwijk which is only like 16 minutes from Leiden. I sent a PM to you already and wanted to know if you'd like to meet in person.

Saskia