Kevin from Mel, Australia dx 7/2016

[kevin.n]

Hi everyone,
I just recently stumbled on this forum and it is good to see some activity on it. I am 21 year old male just graduated from university, living in Melbourne, Australia. Currently, working full time at a consulting firm during the treatment of ASPS.
My story begins in December 2014 where I discovered a lump on my right buttocks. It was the size of a golf ball and was definitely alarming. We did an ultrasound with my GP and was then referred to a sarcoma specialist. Another ultrasound, and a CT scan, the specialist diagnosed me with an Aveolar Malformation. He suggested that we do a biopsy of the lump but I opted not to, after learning about the AVM. I didn’t want to have surgery because I wanted to continue study uninterrupted.
One and half years passed and the lump grew to 5x6x3 it had to be taken out. Almost the size of tennis ball I was referred to an AVM specialist. Surgery took place July 2016, it went well and I had recovered almost fully. I lost some mobility on my right leg but nothing major. After my recovery, I got called in and received the news. They looked at the tumor and diagnosed me with ASPS. They didn’t cut it out with a margin, so it was radiation therapy or have it recut. After MRI, CTs and PET scans they found up to 9 spots spread across my lungs, the biggest spot being 1.5cm in diameter. I was then referred to Peter MacCallum Cancer Centre.
In the month of October, I did radiation therapy on the sarcoma area for 5 weeks. After the radiation therapy, doctors suggested to wait a few months before starting treatment for my lungs. Around January, I had my first talks about treating the lung spots, they suggested Pazopanib but that would require an application to the government to get it cheap. The application was rejected and I had to undergo chemotherapy to be allowed the Pazopanib. Instead of paying, I began chemo in February 2017, I had a low dosage of Doxyrubicin every three weeks. I worked full time through my chemotherapy and soldiered through it. After 4 rounds of doxy, this brings it to the present. I am currently recovering from chemo and PET scans have shown no changes to the size of the spots. Doctors have said to recover for 3 months and take time off treatment before taking the next step.
A quick read of the forums has given me hope and more things to think about. Especially, the scan schedule, which I will raise with my doctor. Any advice is appreciated.


Thanks for reading. I hope to keep everyone updated.

[D.ap]

Hello Kevin

Thank you for sharing your story with us all .
I'm sure sorry that we all need to meet under these circumstances .

It's a truly hard story to share as none of us want to be diagnosed with Alveolar Soft Sarcoma ,ASPS.

Your team of doctors sound like a good group to of found .
The radiation was performed because of not being able to achieve positive margins ?

There are several options for your biggest tumor(s) in your lungs and they would be cryablation or RFA . The Cryo lends itself to less damage.

Your metastic spread to the lungs ,is sure familiar to us all.

Did your pathology report show any bone involvement that you could see ?

We found from the beginning that it's a good idea to start a notebook / scan file on computer , of all that we do in the way of treatments and or consults . Asps is so rare that doctors kinda learn as we do , to a certain degree,of how to approach it.
This forum has umpteen amounts of info and all had been consolidated by Olga, Bonni, and other administrators for over 20 years , I think .
Don't be discouraged by the stats as we have technology on our side .

Love
Debbie
Our sons story


http://www.cureasps.org/forum/viewtopic.php?f=4&t=750

[D.ap]

Damian and Kat have a daughter , Annika whose around 10 years old now?
They live in Australia
You can go to their post and PM them
Damian is training and going to medical school

http://www.cureasps.org/forum/viewtopic.php?f=4&t=822

[kevin.n]

Thanks for the prompt response Debbie. I do have great team of doctors at the moment.
Although I sometimes regret not getting a biopsy done back when I discovered the lump or having my initial doctor push for it.

No positive margins were considered back then. I was misdiagnosed with Arteriovenous Malformation (AVM). It was surgically removed like a plastic surgery.

Cryoblation or RFA has not been mentioned for treatment, I will raise this doctor if it is something for the future. Thanks for the info.

No idea about the bone involvement. I will make sure to ask. Again thank you for the info.

That sounds like a great idea, I often find difficulty in communicating my past and current condition.

Yes the stats do seem stacked against us, but I am an optimist and tackle things bravely.

Regards,
Kevin

[D.ap]

Hello again Kevin
Thanks for the clarification of your surgery .
How often will you have scans to the area?

Unfortunately ASPS even tho it's known as an indolent sarcoma , if at all possible , needs complete positive margins of removal. It can and most likely will
reoccur . Its highly radioresistant --

The success towards curing the sarcoma , lies in successful sugerical and ablative procedures to eliminate the tumors . Even tho doctors see us as stage IV patients , it should never be a reason for them to stop moving forward to remove tumors that are able to be removed .

People have lived long lives inspite of the diagnosis of having metastic disease stage IV diagnosis.



[Treatment and prognosis of stage IV alveolar soft part sarcoma].

https://www.ncbi.nlm.nih.gov/m/pubmed/23336381/

[arojussi]

I agree that surgery (vats, laser assisted thoracotomy) or interventional radiology (cryoablation) are best treatment options. Unfortunately cryoablation is only possible when tumors are smaller. If my memory serves me correctly I think 3 cm is limit. So trying chemo and votrient first can be risky.

[kevin.n]

Hi All,

I hope you've been well.

It's been 5 moths since my last post and not much has changed. Around 3 months back I had my doctors checkup with a stable growth in my spots. I asked questions about surgical(vats, cryoblation, rfa) and ablative procedures but was told it was impractical. They said that I would lose and damage too much of my lungs. With how small my mets were they said there was no point to rush into meds and that another three months they will do a check up. Fast forward three months and I just recently had my doctors appointment where I was given more information into what they treatments they were lining up for me.
Previously, pazopanib was the oral drug they suggested for treatment after my chemo. However, they intend to let pazopanib be a last resort. They see that next and promising treatment would be immunotherapy. New immunotherapy drugs trials that will start soon and see me starting around late January 2018. One of which drug that will be starting soon is https://clinicaltrials.gov/ct2/show/NCT03261011 and there is another in the works. They hope to try one out of the two immunotherapy drug trials. I asked about Keytruda and they said it will come at cost around $10,000 a month. Cediranib was also mentioned and they said it was difficult to secure. They said that they had been looking at different ASPS treatments across the world and considered various options. At this moment they see the immunotherapy trials as the option of choice

I don't know what to think of this plan, it will be another three months of waiting for treatment to start. It's so daunting and always leaves me in a weird place when they say another three months.
I again asked again about how many mets I had in my lungs and they said "a bit more than ten" across my lungs. The biggest one being just over 1 cm in diameter.
I obtained all my lung ct scan reports and the actual ct scans. The ct scans does not require any installation to open them. I can open my scans and scrolls through my lungs.

I don't know what to do in the meantime while I await for immunotherapy. Maybe a second opinion?

Thanks all for reading.

[D.ap]

Morning Kevin ,

From the US
The doctors are correct in the fact of loss lung volumes IF you cryoblated ALL the seen tumors. However the plan should be to ablate the largest 1cm tumor and watch the remaining tumors at this point ?
I’d get a pulmonary surgeons opinion , to map as
precisely ,the amount and location of the lung tumors.
Also educate this surgeon that Asps is an indolent sarcoma typically presentating with multiple small lung tumors .
Some that grow after a surgical removal of the primary.

Has the primary been totally removed with positive margins as of today ?

Immune therapies have been having some success with ASPS patients , so I’d sure keep that as an option .

It’s good to hear from you !

Love
Debbie

[arojussi]

Hello. I am sorry I know what I say will be hard to hear. If primary tumor was removed with positive margins having other surgery to ensure clear margins shoud be first priority. Histologically complete removal of all tumor cells is also called negative margins having this is important, because even small primary tumor can send metastases to blood stream and cause more tumors.

To my knowlegde your doctor is wrong about you losing too much lung tissue from removal of all your lung mets. You will have to exercise a lot, but there is plently of lung tissue. I had around 30 visible mets in my lungs and several microscopic mets all but 3 slowly growing ones removed with laser assised surgeries and cryoablations.

I tried pazopanib in combination with keytruda. Didnt work for me. Pazo slowed tumor growth a little and asps is naturally slowly growing it took almost a year for doctors to confirm that my asps progressed during treatment. As I stopped pazo my cancer grew quickly. So if you start tki treatment stopping it for any reason you will risk faster cancer growth. Pd1 tarceting immunotherapies have indeed worked well for some late state asps patients. I would save keytruda for later and use it only after your asps can no longer be managed with surgeries and ablations. Sorry but I am not familiar with immunotherapy trial your doctor is suggesting.

[kevin.n]

Hi all,

Just a quick update on my treatment. I am beginning a clinical trial over the next few months. Hopefully I wont encounter any problems during the screening process. It will be a combination of a few cycles of a new trial drug followed by cycles of durvalumab. I'll look to share any information of the results over the months. Im quickly writing this, but if anyone wants a detailed schedule or understanding of the trial, Ill do so if requested.

Warm regards,
Kevin

[D.ap]

Hi Kevin
I’d be interested in the trial you are embarking on with durvalumab
We have several patients taking part in a clinical trial in a Texas oncology hospital by the name of MD Anderson, here in the US combinding durvalumab and tremelimumab.
Does the trial require a molecular testing ?
They have been receiving good results .
Will there be any other ASPS patients participating ?
Here is the link to MD Anderson’s clinical trial

http://www.cureasps.org/forum/viewtopic.php?f=88&t=1400

[kevin.n]

It is not the same as MD Anderson trial.

The new drug is as follows: "MEDI5083 is an antibody drug that binds to a protein called CD40". The doctor explained it to me as a drug that will put the "accelerators" on my immune system. A few cycles and injections of this drug, is then followed by the durvalumab.

See https://clinicaltrials.gov/ct2/show/NCT03089645 .

Not sure about any other ASPS patients participating and whether Ill have a molecular test.

[Shamsher Singh]

Hi Kevin
This is Sona, I am also from Melbourne, been diagnosed with asps a week ago. If you don’t mind can we talk ? What you are up to?
How everything is going on??
Please I am hopeless at the moment
If you could please give me some direction?

Thanks in advance

[Olga]

Sona, hi, you should open the New Topi with your name and make the first post with the short history - size, when Dx, was it resected, metastases, scans, problems..then we can explain what can be done and what you should be looking for...regards, Olga.