Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello everyone, my name is Trice. I am 33 and was recently diagnosed with stage 4 ASPS with the primary tumor located on my right shoulder about the size of a grapefruit with innumerable lung mets on both sides. I noticed the primary tumor about a year and a half ago and was unfortunately misdiagnoses twice, first as a bone and later as a lipoma.

First, I want to say I'm really grateful I found this community. I've learned so much in the past few weeks reading all the existing posts.

I've met with an orthopedic surgeon and medical oncologist last week and was referred to a sarcoma specialist at Stanford who I will meet with this week so I can move forward with treatment.

I have a few questions for the group --

1) Opinions on the right time to resect the primary tumor -- The medical oncologist we met with seemed well informed and gave us information that aligned with what we learned here and other forums, which was really assuring. His suggestion was to start off with systemic treatment (Keytruda + Axtinib on the basis that clinical trials show the best response rates when a TKI and antiangiogenesis medications are combined) and then if there is response to wait for the primary tumor to shrink than remove it. Is this consistent with what worked well for others? Is it risky to wait and see if I respond to the immunotherapy? The primary tumor is in a spot where a resection would be pretty straight forward and research seems to suggest surgery is the best way to combat ASPS.

2) Is a clinical trial appropriate for the first line of treatment? I was referred to the specialist at Stanford because well he's a specialist :) and also the medical oncologist believed Stanford would have access to clinical trials I could be a good fit for. Currently, it looks like Stanford is running a trial on Anlotinib (https://stanfordhealthcare.org/trials/a ... 16819.html). Based on what the first oncologist said, better results are seen in ASPS patients with the combination of TKI and antiangiogenesis immunotherapies, so I have skepticism of if this would be the best option for where I am currently.

I'm looking forward to getting to know you all better and learn on this journey together!

Thanks, T

Hi Trice,
welcome to the forum no one want to be the part of, voluntarily. Anyways we are happy to be of service to our fellow worrier.
I am Ivan's mom, he was Dx in 2003, just to put it in perspective for you. Now after numerous surgeries and lung/brain/pancreas/adrenal metastases resected in a course of 15 years and Keytruda treatment after that for the unresectable mets in his heart he is doing pretty good, it was a life saving treatment for him.
To comment on K+A clinical trial. Its result was pretty good but you have to know that the trial for K alone was never done so there is no substantiated claim that K alone is less effective. There is some theory behind that though, but not proven. Ivan had K only as a single drug. But he did not have any bulky tumor that might be immunosuppressive and can interfere with immune system actions after the breaks are taken down by K.
How large is the primary now? You can try the K+A for starter with the promise from the drs if there is no reaction from the primary, no shrinkage, then you stop for the surgery, to resume after. In our experience the chances for the good response can be reduced by the bulky primary due to its immunosupressive actions.
Re. clinical trial with Anlotinib - we do not know if it is going to be more potent than K+A or K alone. There are limitations for clinical trials versus prescribed treatments as you will have to be limited by their protocol that might have some disadvantages. Are there any financial advantages for you to go to clinical trial versus a treatment with K+A?

Hey Olga thanks for the reply,

That's great to hear that your son is doing well. My primary tumor is about 7cm. With regards to the clinical trial, there are no financial advantages that I can think of. It was a suggestion by the sarcoma specialist (meeting later this week) to the oncologist that I spoke with initially. Before he spoke with the specialist he wanted to start my treatment with K+A, but after their discussion, he suggested I have a chat with the specialist about the trial of Anlotinib to give me more options. Aside from the potential benefit of Anlotinib being more effective than K+A alone, I can't really think of any more advantages of doing the trial, but I also don't know what I don't know. That's just my train of thought at the moment.

Usually being on a trial takes more visits to the clinics. Which is a disadvantage during Covid. Besides there are numerous limitations if you need to stop the treatment or to have a surgery or radiation during the trial. We can not make any meaningful comment re. expected efficacy one versus another too.

Hello Trice!😊

My name is Debbie and our son Josh was dx’d in 2012 with a 6cm upper right leg tumor . Also stage 4 at the age of 32.

I’d certainly visit with Dr. Kummar and staff about the Anlotinib as I believe Dr. Kummar with being involved prior with the cediranib trial of Asps patients , has considerable knowledge under her belt.
https://pubmed.ncbi.nlm.nih.gov/23630200/
So she’s knowledgeable about the success of tyrosine kinases inhibitors and asps.

With the size of the primary and with the possibility of a potential removal ,having been on either of the therapies prior could give them a clue to if it’s working thru the pathology from the possible future surgery .

Also trying Keytruda and axitinib could give you all the same clue.
I’d make sure that surgery is on the table with the trial though .
I assume the Keytruda and axitinib are off label ?

Being stage 4 how big is your biggest lung tumors?
Review all your scans brain lungs and arm as well as a bone ,as these are now what’s referred as your base scans .
Keep copies of the scans as asps is a lifetime disease to be combated .

Good job in educating yourself on this difficult pursuit.
But remember there’s much hope and knowledge to be had !

Olga - I hear you on the pros and cons. Doc let us know we can stop at anytime we'd like which is reassuring

Debbie - Thanks for the info on Dr. Kummar, we will ask about her! The Keytruda and axtinib would be off label, luckily the specialists at Stanford said they don't typically have any issues with getting approval. The biggest nodule in my lung is 2.5 cm and the lung mets seem to be the biggest concern to all the doctors I've seen so far, including specialist at Stanford. Bone and brain scans are thankfully clear.

Here are results from the phase 2 trail on Anlotinib if anyone is interested: https://clincancerres.aacrjournals.org/ ... /5233.long
Pretty promising response rate from ASPS patients!

The general feedback from the specialist was that everyone is different and basically there is no right or wrong treatment to start with. While the first oncologist we met with suggested K+A as a first line of treatment, the specialist suggested either the clinical trial or a double immunotherapy treatment to begin with.

I think the biggest question I have is if the rate of metastasis is higher while the primary tumor is still present, there doesn't seem to be conclusive evidence to prove if it does or doesn't.

Because of the promising response rate from ASPS patients and the reassurance I can stop the trial at any time, I am learning towards starting systemic treatment with the anlotonib trial.

ASPS disseminate early, but in general both, the primary tumor and the lung mets are capable of ongoing additional dissemination. The observation of the fact that brain metastases are almost always are preceded by the lung mets, supports that notion. Usually the tumors have to be bigger than certain size to disseminate (for ASPS it is a pretty small threshold as we know from the experience). I would resect the primary if possible in all cases and then have the immunotherapy with the simultaneous local treatment to one of the biggest and safely (for the treatment access) located lung met to improve the chances of the immune system recognizing the tumor. Bulky primary tumors produce the immune suppressive effect. Either way is a relatively equal to proceed though in absence of the hard evidence.

Trice,
I agree with Olga as surgery is known as the most effective procedure for ASPS.

The primary tumor removal could result in the most beneficial results , in my opinion as well.

The lung tumor could be used as an immune therapy response gage, with it being a 25mm size.

Keytruda was originally developed for lung cancers ,as you well know. It would be in my opinion an easier Med to tolerate compared to Anlotinib.

Thanks for checking in and wishing you the best !

Hi all,

I've reached my first scan results since starting my treatment of Odivo (nivolumab) and Yervoy (ipilimumab) and I wanted to provide an update for you all. The TLDR version is that my lung mets are still innumerable, but have reduced in number and size significantly. My primary tumor has also reduced in size. Overall, great news to hear from the doc!

For those more interested in more of the details, I've had 3 infusions so far, they're typically 3 weeks apart and I actually have my 4th session tomorrow. The worst of my side effects happened between my 1st and 2nd infusions and it was essentially a result of my thyroid being affected by my immune system, causing it to release a high concentration of thyroid hormone (i.e. hyperthyroidism) and all kinds issues resulted from that such as extreme fatigue, vomiting, nausea, sustained heighted heart rate, complete loss of appetite, etc. During an attempt to receive my 2nd infusion, the nurse administering my medicine was so concerned about my weight loss (45+ lbs in 2-3 weeks) and my thyroid levels from recent blood labs that she had an on-site endocrinologist take a look at them and they decided that I should reschedule the infusion and head straight to the ER for further examination in hopes of preventing me from experiencing a thyroid storm. I had to stay there overnight for tests and have my heart rate managed down as it was pretty high (95-105 consistently). After, the side effects from the hyperthyroidism subsided, I've been feeling much better ever since. One thing to note, however, is that generally after hypothyroidism is caused by immunotherapy, hypothyroidism generally follows and could be permanent - which would mean I'd potentially need to take thyroid hormone replacement daily for the rest of my life. This hasn't happened yet, but we are noticing my levels dropping lower than normal in my blood labs so it's still a real possibility. It shouldn't be a problem if managed well.

Besides that incident, I haven't really had any other issues that we've directly attributed to the immunotherapy or ASPS. My most notable tumor shrinkage in my lungs was around 40%, my primary shrank at around 30%. I'll continue to keep you all posted on my status and as I learn more. Thanks for all of your support and best wishes to everyone.

Hi Trice,
this is an excellent response to the ICI combo. The primary rarely shrinks completely so keep in mind that there might be a good window of opportunity to safely resect it at some point.
Re. autoimmune side effects. When immune checkpoint inhibitors cause the endocrine autoimmune side effects ( IrAEs) there is usually because the immune attack is very strong after the brakes are taken off. From the oncology point of view it is a good news because the ppl with the IrAEs get the best long lasting benefit from immune checkpoint inhibitors.
There are numerous publications re. IrAEs management please educate yourself.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7576644/

Hello Trice,

Checking in with you to see how the Opdivo yervoy treatment is going ?
Is the weight stabilizing yet and how are the thyroid levels going ?

Hi Debbie, thanks for checking in on me! Sorry I've been absent on the forum.

Since my last post, I've had positive progress. Thyroid issues have resolved and levels are back to normal, I am currently taking thyroid replacement for the foreseeable future. My weight is back normal, even higher than pre-diagonosis. Currently upping my exercise to try and get back to my comfortable weight.

Scans have consistently shown shrinkage. My primary, located on my shoulder has melted down to almost to only a few centimeters. At diagnosis it was so big you could see it through a t-shirt. Lung mets have also shrunken considerably - last time my onc & I reviewed scans he remarked that when we started there were "spots everywhere" on my ct scans. Now only a few are still visible.

On one of my past scans some calcification was seen on my iliac bone - radiologist said it resembled a healed met from the immunotherapy treatment. That was news to me, because at diagnosis they said bones were clear of mets. Recently did a full body MRI to get a full assessment on any bone metasteses and everything came back clear. It makes me wonder, have others experience small spots of bone hardening that aren't related to mets?

My kidney enzymes have been elevated for the past few months, though they were stable during my last bloodwork. Nephrologist is keeping an eye on it. Not sure what made it stabilize but I'm happy that its remaining under control. For reference, I'm about 8 months in to yervoy & opdivio treatment


- Trice

Trice, thank you for the update. Overall the news are good for you.
Some random comments.
If you put to much weight it might be from the over-replacement of the thyroid hormones? In our experience with the adrenal hormones replacement for Ivan's adrenals damaged by the immune system under Keytruda treatment, the replacement has to be very carefully individually tailored in both, dosage and timing, to much that of the patient own. In your case the weight might be a good indication to use as a mark.
The kidney function might be affected by the immunotherapy as well as by the contrast in the scans. The list of the immunotherapy toxicities is very extensive and it is hard to name any aspect of health that can not be affected as this effect is very person specific. It can attack bones as well, but on the other hand there might be occult bone met that had very low activity and was missed. And now it was attacked by the immune system and noted by the remains? I would say just watch it closely and compare the progress between the scans to figure out what is going on. What was the scan type when the calcification of the bone was noted?

Thanks Olga. My endocrinologist is watching my levels monthly via blood labs and we're basically trying to remain at baseline. We've reached that level now, however, I feel that maybe my original baseline may be lower than what I need it to be currently as my diet hasn't changed much and my weight gain seems excessive at the moment. With regards to my kidneys, I'm doing urine tests monthly to check eGFR levels. For my bones, we first noticed the sclerotic portion on a chest/pelvis CT approx. 2 months ago, then opted to do a full spine MRI since it was the first time it was noticed and the spot was essentially at the very bottom of the CT imaging.

Thanks for everything and I hope all is well your way.

Hello Trice,

Good to hear from you.😊
So you are hyperthyroid ?
What treatment are you using for your hyperthyroid ?
Or are you fluctuating from being hyper and hypo thyroid ?

Also with your weight gain , you’ve been given council on what to watch for being and to report back to the doctors in the advent you gain an inordinate amount of weight ? Ie kidney issues ?