Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi, my name is Kyle, and I've recently been diagnosed with ASPS. I feel like up to this point I've been able to push my way through the system to receive treatment at a pretty quick pace, but am now starting to feel lost, and don't know where to go from here. I'm so glad I was pointed towards here to seek out help, and advice. However there is just so much info it's a bit daunting to wade through it all to find what best to push for in advocating for myself.

October 20 2019- Noticed a lump in my left thigh. Originally thought it was just a knot in my muscle, but quickly realized that wasn't the case.

October 23 2019- Ultrasound

October 28 2019- MRI

November 7 2019- Needle biopsy

November 15 2019- Received results from biopsy with official dx of ASPS. Had first chest CT showing 10 nodules with largest being 7mm.

November 21 2019- Pet scan showing nothing abnormal except primary tumor in thigh.

November 25 2019- Began pre-op radiation.

January 28 2020- MRI on primary tumor location for preparation of surgery. 2nd Chest CT showing 1 additional 2mm nodule without any significant growth in existing nodules.

February 10 2020- Resection of primary tumor in left thigh measuring 5.3cm x 4.7cm x 3.5cm obtaining negative margins.

May 12 2020- 3rd Chest CT showing growth in most nodules, and one additional new nodule confirming metastatic disease.

And that is where I am now. I received the results of my last chest CT during a follow-up with the orthopedic oncology surgeon that removed the primary tumor. They were suggesting a somewhat wait and see approach going forward. Possibly doing a needle biopsy once the largest nodule in my lungs is over 1cm to %100 confirm mets. They also seem to be of the opinion to not do anything that would reduce my quality of life when I don't have any symptoms. After reading here I'm seeing that most likely isn't the best approach, but I don't know what really is. I've had appointments with a radiation oncologist, and the orthopedic oncologist surgeon, but haven't actually met the medical oncologist they have both been working with regarding my treatment. I've request a brain MRI, and full body bone scan now, but haven't heard back yet. Then I'm going to get an appointment with the medical oncologist they have been working with to see what options he would suggest going forward. I'm also going to book an appointment with oncologists at another organization to get a second opinion about what to do going forward.

I really want to have an idea of what treatment and procedures are out there for someone in my position before these appointments so I can ask the best possible questions, and propose the best possible course of action. Any help or guidance that can be provided would be greatly appreciated. The people here seem to be pretty amazing with everything offered. Thanks in advance.

Hello Kyle ,

Welcome .😊

So glad to hear of your primary resection .
Your perseverance as well as your questions , will get you far in treating this incredibly rare sarcoma .
Not many oncologist have seen it and understand it.
It’s a learning process for all concerned .

You certainly are seeing a lot of what our son saw, back when he was diagnosed in August of 2012. Lung mets after his resection .

Most certainly press for the whole body bone scan ( not just PET) and the brain scan and lower pelvic scan .
They are not only important for what is here and now, but can be referred later if need be , as a base line reference. A change .

Ask for your pathological report , as it will tell of possible invasion and mitotic level .
This is our sons initial journey story .

viewtopic.php?p=4388#p4388

He’s been through the gambit of surgeries and ablations , But is currently on Keytruda and knock knock, is fighting a stubborn liver tumor .

This group has been a Gods send to our family .

Ps
May I ask how old you are?
Sincerely ,

Pss
Kyle ,are you in contact with a Dr. Kevin Jones , of the University of Utah, Huntsman Cancer Center?
He’s been very much involved with ASPS research .

I was 36 when diagnosed, now 37. I'm currently being treated at IHC. Today I reached out to Hunstman to see who best there to get a second opinion from with my specific case, and was waiting to hear back. I appreciate the info about Dr Jones. I'll see if I can get in contact with him.

Hey Kyle
I assume Dr. Jones is still with the University .
If not Dr. Breelyn Wilky is at Colorado university ,
As she has performed clinical trials specific to ASPS. She is very knowledgeable about ASPS .
https://www.cudoctors.com/Find_A_Doctor/Profile/28739

Best of wishes ❤️

D.ap wrote: ↑Wed May 20, 2020 5:50 pm
Ask for your pathological report , as it will tell of possible invasion and mitotic level .

What do you mean by this?

I do have a post surgery pathology report that says "Mitotic Rate /10 high-power fields: 0"

Hi Kyle,
welcome to the board. So far your treatment is processing in a right way. Have you been able to gain the full functionality with the leg use after the primary resection? It is important to focus on this as there has to be the conscious effort soon after the surgery, not to miss this window of opportunity to regain and keep the full functionality. It can be done and was done by many of our members here.
Re. increased growth in the lung mets - it is the usual occurrence that we see somehow faster growth after the primary tumour resection, as the same growth factors our body uses to hear the wound, are influencing the lung mets to grow. It will most probably slow when you get your next scan.
The most promising systemic treatment option for now is one of immune-checkpoint inhibitors - Keytruda, Opdivo or atezolizumab. My son is having a very good response to Keytruda, he was on it for more than 2 years and now he is off the treatment, because he is having some autoimmune side effects and we do not even know if more Keytruda even needed as his body has already formed the tumor specific response, the immune cells that attack ASPS in his body. We do not know how long is it going to last. His name is Ivan, look for his story. He is very active, physically fit despite being initially diagnosed in 2003 and living with metastatic state ASPS since 2005.

ky7le wrote: ↑Thu May 21, 2020 3:45 pm

D.ap wrote: ↑Wed May 20, 2020 5:50 pm
Ask for your pathological report , as it will tell of possible invasion and mitotic level .

What do you mean by this?

I do have a post surgery pathology report that says "Mitotic Rate /10 high-power fields: 0"

Hi Kyle,
haven't been ignoring your request.
still mulling over my response.
I agree with Olga as you have truly advocated for your ASPS care.
Hang in there.
Love,

Hey Kyle ,
Happy Saturday .😊
So the mitotic level that the pathologist reported , was 0?
It appears to me that a lot of our ASPS patients are reporting below 5 as their mitotic levels .
Joshua’s was reported as a 6, from his primary path. report , back in August 2012.

All our other reports that I’ve seen have , his laser lung surgery from Germany only reported positive or negative for ASPS.

Was there any reference to lymphatic Invasion ?
And or bone involvement ?

Here’s Joshua’s pathology report

viewtopic.php?p=8012#p8012
Answer when is good for you.
Enjoy your day .
Love ,

Thanks to the recommendations here I was able to get my doc to order a brain MRI, and full body bone scan so those are now scheduled.

Olga wrote: ↑Thu May 21, 2020 11:22 pm Hi Kyle,
Have you been able to gain the full functionality with the leg use after the primary resection? It is important to focus on this as there has to be the conscious effort soon after the surgery, not to miss this window of opportunity to regain and keep the full functionality. It can be done and was done by many of our members here.

I'm still working on getting up to %100 range of motion. Getting close but still not there. Also working on building up strength and endurance. My physical therapy was unfortunately interrupted when I showed symptoms of Covid even though I never had it. I did get out on a couple hikes last weekend without issue which was nice.

Olga wrote: ↑Thu May 21, 2020 11:22 pm The most promising systemic treatment option for now is one of immune-checkpoint inhibitors - Keytruda, Opdivo or atezolizumab.

Going through the stories on here a lot of the recommendations I've seen have been to do what's possible with surgery, cryoablation/laser assisted, before starting other options. A lot of these stories are going back many years though. Has people's opinion on this changed now to where I should be looking for systemic treatments first?

D.ap wrote: ↑Sat May 23, 2020 10:20 am So the mitotic level that the pathologist reported , was 0?

It seems like it if I'm reading it correctly.

D.ap wrote: ↑Sat May 23, 2020 10:20 am Was there any reference to lymphatic Invasion ?
And or bone involvement ?

It the case summary it says "Regional Lymph Nodes: No lymph nodes submitted or found", and then in the pathologic staging sections says "No regional lymph node metastasis or unknown lymph node status" I may need to reach out and get clarification.

As for the bone involvement, the pathology report doesn't mention it, but the tumor was pressed against bone, and they did remove a section of periosteum around the bone.

Q- Has people's opinion on this changed now to where I should be looking for systemic treatments first?
A - in short - we do not know. Immune checkpoint inhibitors are fairly active in ASPS and many of our patients are having dramatic and long term regression of their metastases but we have no idea how long is it going to last. It is still very new, so our responses are 2, maximum 3 years old.

Hello Kyle
I used the wrong term when asking you for lymphatic involvement.. I was talking about the primary tumor vessels ,and suggested the lymph nodes .


https://staging.seer.cancer.gov/naaccr/ ... /home/1.8/
Excellent news on the bone scraping to obtain good margins .
Another question I have is when the biopsy was performed , was the tumor suspected to of been a sarcoma ?

That is the only part of my pathology report that mentions anything "lymph", and I don't see anything about LVI like in the link you provided. I looked at the thread where you posted Joshua's pathology report, and it seems that his and others may have more detailed info. Maybe the copy I have is just a summary?

Something I'm seeing on here somewhat regularly is the suggestion of having copies of all your scans along with all other records. I think I need to request all my records. Have people experienced any difficulty doing that? Is it a pretty straight forward process?

My surgeon was the one who ordered my biopsy, and I didn't meet with them before so its hard to gauge what they most suspected. However they do have experience with sarcoma so I definitely think it was something they were thinking about.

ky7le wrote: ↑Wed May 27, 2020 8:34 pm That is the only part of my pathology report that mentions anything "lymph", and I don't see anything about LVI like in the link you provided. I looked at the thread where you posted Joshua's pathology report, and it seems that his and others may have more detailed info. Maybe the copy I have is just a summary?

Something I'm seeing on here somewhat regularly is the suggestion of having copies of all your scans along with all other records. I think I need to request all my records. Have people experienced any difficulty doing that? Is it a pretty straight forward process?

My surgeon was the one who ordered my biopsy, and I didn't meet with them before so its hard to gauge what they most suspected. However they do have experience with sarcoma so I definitely think it was something they were thinking about.

Kyle - do ask if there is an addendum to the initial pathology report.

Yes you have every right to ask for the hard copy and or a disc of say your MRI and CT. Keeping a log and or file , with all this data complied , is really important. Asps is a life long commitment and surveillance . With that being said, you will have LOTS to remember and having a log will help jar your memory .😏

Asps is so incredibly rare that the more the patient knows and understands , the more we learn.😊
We certainly aren’t all knowing ,but we need to ask questions which I feel ultimately leads to our whole ,care .

When you have a choice , choose lower radiation scans/tools, for surveillance scans , between sched scans . It helps to keep your radiation exposer down , over your life time.