Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Too many people have died or have found themselves in a very difficult situation because they did not follow the proper scanning schedule - myself included. ASPS is an extremely treatable disease wherever it pops up - even in significant numbers - as long as it's diagnosed and dealt with rapidly, and appropriately.

Many oncologists are clueless when it comes to what to scan, so you have to be proactive in making sure the optimal schedule is implemented. We will be compiling a comprehensive recommended follow-up guide for ASPS patients, which will vary for individual circumstances. The schedule will need to be modified, depending on the procedures performed.

General Schedule

Every 6 months: (1) lung CT
Every 12 months: (2) brain MRI, (3) abdominal MRI (preferred) or CT, (4) bone scan, (5) echocardiogram or gated heart CT or MRI
Every 12-24 months: (7) body CT can be substituted for abdominal CT/MRI, and combined with lung CT.

If no mets after a few years, the schedule can be somewhat relaxed. However, it's extremely important to remain vigilant. There have been cases of mets first appearing 10 years after diagnosis.

Patients with suspected metastases

The rest as above, but the suspected site should be observed more frequently. 6 months may still be OK for lungs, but brain or other areas should be re-checked after 3 months.

Patients after surgical treatment of metastases

Operated area followed up 3 months after the procedure. Subsequently, the organ / area should be indefinitely checked in 6 month intervals if it was previously observed every 12 months. For example, brain will be checked via brain MRI every 6 months after the first presentation of a metastasis, even if it was successfully removed. Scheduling for other areas unaffected.

Dear Ivan,
You are to be admired and commended for having the strength and energy to organize and share this extremely important information the day after your abdominal Cryoablation procedure, and your efforts and thoughtfulness are truly deeply appreciated!! I Hope that everyone in the ASPS Community including the doctors, patients, and family members will recognize the validity and critical importance of this scan schedule information which could help to save precious Lives if it is followed. Based on our personal experience during Brittany's challenging twelve+ year ASPS journey, I strongly agree with and support the schedule for scans which you have presented. Additionally, I would like to express my personal concerns about the use of PET scans rather than CT scans and MRI's because we have found from personal experience and observations of other ASPS patients that PET scans can be very unreliable and misleading sometimes showing false positive or false negative results as you and your mother also found out Ivan when a PET scan was done of your adrenal mets and subsequently failed to even show the mets!! This scan error could have resulted in tragic consequences if you had not been so pro-active and knowledgeable. To everyone fighting this insidious disease, PLEASE listen to the advice which is being offered, take charge of and be actively involved in your care, and don't allow naiive, ignorant, complacent, or irresponsible doctors to refuse to schedule regular, adequate, and appropriate scans. Your Life may depend on it!!
With special concern, deepest caring, and continued Hope,
Bonni

Bonni, I would also love to hear your input regarding the schedule. It will need to be expanded for certain other cases and situations. I'll make sure to note the PET's ineffectiveness.

Hi Ivan
This is a question everyone asks and i am glad you posted this

The sarcoma Oncs i have met that know slow growing cancers do not do the PET's from what i have been told.

I wish i could get in a dam MRI machine... I am going to an open MRI and will have to do brain MRI every six months the cuts are 3.5 mm and this is so far the best i can find for an open MRI

Ivan:

Thanks for compiling this list. Sorry to clutter up the thread, but I had a few questions maybe you or someone could answer:

1) Are there any other types of sarcoma where a scan regiment exists? The reason I ask this is that our onc seems hesitant to order scans unless I am experiencing symptoms that would suggest a met in the area scanned. I would like to show him a scan regiment so that maybe getting scans ordered will be less of a hassle.

2) Have you or anyone heard of oncologists ordering CTs without contrast? The reason I ask this is that Dr. Rolle orders all of his scans without contrast. Given, he is a surgeon and not an onc, but I wonder if the contrast is really needed on every single scan, or maybe could just be done annually instead of every single time. In terms of radiation dose to the patient, a scan with contrast is double that of a CT scan without contrast (since it is literally 2 scans). And, unfortunately, there is no law that radiation exposure must be tracked for CT scans (at least in the US), but I have read that a thoracic scan can result in 5-7 mSv of radiation to the patient, so I am assuming a scan with contrast can result in 10-14 mSv. With 4 scans a year this can add up to 40-56 mSv, where 50 mSv is what is generally thought to increase the risk of cancer by 1%. This may seem like a small number, but anything we can do to reduce that exposure is good. The last thing that anyone needs is more health problems.

3) Lastly, I may have asked this before, but is a bone scan different then a PET scan? Would it be realistic to request a PET and use the results to review bone health?

@Josh:
1.Q: Are there any other types of sarcoma where a scan regiment exists?
A: There are some guidelines but in general they are written in a way providing the flexibility to the oncologist so the decision can be made based on the histology and particular situation. ASPS is a slow growing sarcoma and since there are the articles that resection and other treatments for metastases improve survival, finding them on an early more treatable size would be the logical goal. This is the link to NCCN Guidelines Version 1.2013 Soft Tissue Sarcoma (register under Icureasps company)
http://www.nccn.org/professionals/physi ... arcoma.pdf
Read the guidelines and see if you find anything you can use.
of specific interest here they recently added specifically for ASPS the requirement to have a brain surveillance, so if you are having a problem of getting the brain scans use that:
"Central nervous system (CNS) imaging should be considered for patients with ASPS and angiosarcomas. "
more later.

Hi Ivan

After the first i think year my oncs have stopped asking fro contrast.. The contrast shows when things are vascular and i think because we have asps they suspect all things seen in a scan to be asps they also assume it to be vascular.
The only thing they wanted was for me to drink the stuff *gross* that was for the pelvic scan. But, that makes me so sick now i cant use it.

I am going to be requesting MRI Pelvis and abdomen.. What do you think? No more CT unless it is lungs ?

I have never had a bone scan.. they found my asps in my bone thru a CT

Dear Josh,
Regarding your question about guidelines for the scan regimen and your statement that "our onc seems hesitant to order scans unless I am experiencing symptoms that would suggest a met in the area scanned.", I absolutely don't understand how any oncologist with any basic knowledge about any kind of cancer, and especially Sarcoma and ASPS, would wait for a patient to become symptomatic before ordering scans!! I find this attitude and practice to be incredibly incomprehensible, irresponsible, negligent, and totally unacceptable!! METS NEED TO BE FOUND AT THE SMALLEST MOST TREATABLE SIZE FOR THE BEST CHANCE/ POSSIBILITY OF TREATMENT, AND A SUCCESSFUL OUTCOME TO THE TREATMENT!!!!! IF YOU WAIT UNTIL A MET BECOMES SYMPTOMATIC, IT MAY BE TOO LARGE AND CONSEQUENTLY TOO LATE TO SUCCESSFULLY RESECT/TREAT THE TUMOR!!!!! I cannot emphasize this strongly enough!! This fact was recently tragically demonstrated in the case of an English ASPS patient who I closely follow who repeatedly requested a brain MRI from her oncologist for the past year. Her oncologist inexcusably continued to refuse to order the scan for her until she recently developed debilitating neurological symptoms and now following last week's heartbreaking MRI diagnosis of a most probably unresectable/untreatable brain met, the patient devastatingly may lose her precious young Life. Fighting and managing this very challenging and unpredictable disease requires a very pro-active approach which includes vigilant, regular, and complete monitoring scans, and NOT just waiting for symptoms to develop!! If your current oncologist refuses to cooperate on this critically important and possibly Life saving issue, I would personally encourage you to find a new oncologist as soon as possible.
With special concern, caring thoughts, healing wishes, and continued Hope,
Bonni

Dear Josh,
I 100% agree with Bonnie and please find a new Onc as soon as possible! His or her experience or attitude is not safe! I don't care if they change the scan situation! If they are dropping this HUGE ball i can't imagine the other areas they would! Please, post what area you live in or around an lets find you a real Onc that actually deals with preservation an is proactive! You deserve this! <3

I get my (every 3 month) chest CTs without contrast

Per my onc - CHEST CTs for the purpose of tracking mets in the LUNGS don't really benefit from contrast...And it's better to not expose the body to too much contrast especially if we are getting it every 3 months or so.

Anyone - correct me if I'm wrong...

CT with the contrast is really necessary when there is a goal to verify if the local treatment was a success - radiatiosurgery or IMRT, cryo or RFA ablation etc. After all of these treatment whatever is left after the treatment of the targeted met is left in place (not resected and removed as with the real surgery), so it needs to be checked if the tumor is dead or there is a blood flow trough the met so it might be only partially dead or not dead at all (missed by the probe).

Olga

What rule of thumb, time frame ,should be used to do a wait and see if the ablation, treatment was succesful?
In other words , how long before an accuarate scan can tell whether there is success or not.
Maybe several to compare that there is a reduction of the said met?

Thank you

Debbie

The ablated met has to be visible dead - no blood flow - not lit up - on the first post ablation scan done with the contrast, despite being bigger due to the ablation zone always increased by the margins comp. to the pre-ablation met size. The first scan (CT with contrast or MRI with contrast) has to be done in 1-3 months per Dr.Littrup. The size has to start decreasing as the necrotic tissue is getting absorbed by the body and it has to be smaller or at least stable at the 6 months mark (I read an article re. follow up and this is what it said).

Olga
So the CT without contrast only shows a visible size to compare to NOT if it has a blood supply going to the visible met
That would of been determined prior to the CT without contrast
When taking TKI's, is it to a patients advantage to have a contrast as a base line then another maybe not every 3 months but when ?

Debbie

EDIT: sorry I misunderstood. I thought 3 months interval was part of a clinical trial requirement.

If not, that's too often. I would do 6 months, and even compare that not to the previous scan but to the one before that - 12 months or more, if all the scans were noted as "stable". That would allow you to track small growth.