Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Why Hello there!!!

I'm not quite sure how this works or what I'm doing but I found this forum yesterday and it was quite revealing and reassuring. I have spent a lot of time trying to find out more about this condition and as it is so rare this has been difficult. So I suppose using this forum to tell my own story will benefit me and others alike. Reading through all your stories is constantly making me feel stronger and more able to process this diagnosis, you are all extremely brave, lovely and upbeat people xxx

So, here goes!

I am an 18 year old female student, living in England. A couple of years ago I felt a small lump on my back, I thought nothing of it and it wasn't until summer 2015 when I decided to go to a doctor to check it out, having realised it had grown quite significantly. The doctor told me it was a cyst, and they could remove it there under local anaesthetic. Just before the surgery a different doctor informed me it was a lipoma, and they were going to go ahead with the surgery to remove it. After they put in the anaesthetic I freaked, and disallowed the operation to go ahead. They referred me to a hospital for an ultrasound to find out whether I could get the 'lipoma' removed under general anaesthetic.

October 2015 I had an ultrasound which came out as 'concerning' and I was advised to have an urgent referral to a suspected cancer clinic. I went to a different doctor, who didn't pick up on the urgency as sent me for a MRI scan. The scan (now 6 weeks later) came up as worrying, again, and finally I was referred to an orthopedic hospital. Here they told me it was most likely a vascular-malfunction, but a biopsy would be the only way to find out for sure.

Sure enough, the biopsy results I received a week earlier than planned on 21st January 2016, and I found out the lump was in fact an alveolar soft part sarcoma. Obviously devastating news, but I got the mass removed four days later on the 24th January, it was deep in my left shoulder but luckily not touching any bones, and measured 4 x 3 x 2 cm ("nodular tumour consistent with a grade 3 alveolar soft part sarcoma. This has been stages as pTB1 Nx Mx grade3"). No radiotherapy was needed.

My bone scan after the op came out negative for disseminated bone metastases; however, my CT chest showed a 5mm lesion in the right lower lobe (i.e. abnormality in my lung). I found out these results on January 26th, and it was thought best to keep an eye on the mark on my lung with further CT scans. My next one is on July 5th and of course I am hoping the scans will come through clear or with no changes however I'm not feeling too optimistic about the results considering how common it is for ASPS to spread to the lungs.

I am not very well clued-up about this type of cancer, and have just been trying to focus on my studies and enjoying university life since recovering from the excision of my shoulder, for which I was in hospital for 5 days and off from lectures for 4 weeks.

I'm sorry this was sort of long, I'm not sure if anyone will actually read this... But I will sure continue reading through everyone else's stories and will keep updating on here because writing everything down helps me to process what has happened a lot.
Thanks for this forum, stay strong all of you!!!!

XXXXXXXXXXXXXXXXX

Hi Mandy,
thank you for the detailed report of how your ASPS case was Dx and managed, it is unfortunately very typical story for ASPS to have a very slow diagnostic course as it is grows slowly and is very non-specific. But it is also its good side - only its slow growth allows us to take actions and to fight for the lives efficiently giving enough time for recovery between the treatments and surgeries.
You did not say specifically when in England you are, but you have to know that the biggest expert on ASPS in UK is Prof. Ian Judson (Google him), you should insist on referring your case to him if something starts to go wrong. There is an UK specific problem though - they are very insisting on treating metastases only when they are symptomatic (we are here insisting on treating all mets as soon as possible so not to allow them to became symptomatic as they are most treatable on a smaller size and when symptomatic the optimal/less traumatic local treatment options are often lost). I suspect that the UK positions has to do with the government sponsored health care and financial constrains. But it is to early say if any of these problems are going to apply to you, so the proper scanning is your friend and goal for now. Good luck with the scans.

Olga,
Thank you for your quick reply! I am based in London, with the Royal National Orthopedic Hospital and the UCH Macmillan Cancer Centre.

I just googled professor Ian Judson, and if it comes to such I will definitely mention him to my doctors.

My doctor was very vague about what treatment will be used if the cancer has spread, hopefully they will remove them as soon as possible but I'm not sure. The uncertainty is difficult to deal with but I guess I will find out what's next at my next scan.

Thank you!!
xx

Hi Mandy

I sorry to have to meet under these conditions with all of us having alveolar soft part sarcoma (ASPS) or knowing someone with it..I'm the latter

But Olga is right on the money with being thankful for our indolent sarcoma.

So your primary tumor was on the back of your shoulder?
as well to show what internal images you have currently so they can keep track and make note for your future scans. These are called base scans as they are a starting place to either note nothing to report or something to follow in the future.

How was the grading described to you?

I'm not able to figure out? "pTB1 Nx Mx grade3"
Did it have to do with the suspect lung image?


Sure was nice to meet you

Much love
Debbie

Our son Joshua.
dx'd in 2012 at 32 years of age

Hi Mandy,

Just for your information the next meeting of Connective Tissue Oncology Society will be held this November,
and my local doctor (Ilkyu Han) told me this year they will discuss about ASPS.
I don't know whether it will resulted any new cure method or not but let's just wait and see.
Link: http://www.ctos.org/Home.aspx

Meanwhile, you could read some of success stories to ease your mind and study about this disease as well.

Best regards,
Daniel

Debbie,

Thanks for your reply!

It was on the back of my left shoulder yes. I'm not sure about the grading system, maybe it's different here, I think it's called the TNM staging system. So, "TB1" Means 'the tumour is deep in the body'; "Nx" means 'the lymph nodes were not examined'; "Mx" I think means they're not sure if the metastasis is present. And Grade 3 is the usual - 'high grade', the definition at the moment being 'the cancer is of any size, superficial or deep and of any grade, but has spread to at least one lymph node but not to any other parts of the body'.

If I'm honest, I'm not sure how significant the grading is but I presume that if they decide with another CT that the cancer has spread to the lungs I'd be classed as a 'stage 4' which is when the cancer has spread...

I wish you continued good luck and the best wishes for you and your son <3
xxx

Hi Daniel,
thank you for your response, it really is appreciated.
That sounds hopeful, all we can do is wait and see really.

I have read some and they are definitely helpful, this forum is honestly amazing.

best wishes to you xxx

Hi Mandy,

Very good that you have found this forum that none of us whats to join. Unfortunately it is as it is and luckily there are people here that have helped a lot with their advises gained from their long term battle with this disease.

I would recommend and agree with what Olga wrote. Prof Judson is one of the best. Last year was in Royal Marsden hospital in London. I guess you as British citizen can get to him much easier.

Be very vigilant as this is of great importance.

Hi guys,
So in July I had a chest-CT and it came out the same. The spots on my lungs hadn't grown and we weren't as worried.
However I had a scan yesterday (29th Nov.) And it turns out the spots have grown, and there are more. The biggest being 7-8mm.
So i definitely have secondary cancer.
However they are not treating me straight away, and are waiting for another CT in January...
Dr said the drugs aren't easy to access and surgery is not always effective as mets can keep appearing afterwards.

Feel at a bit of a lose end, bit surreal that I'm carrying cancer in my lungs and doing nothing about it.

Thought I'd update, maybe it'd help give me some ideas or help

Thank you )
Xxxx

Mandy

Hi again
The report sounds right as ASPS trucks on as it is a systemic disease . I sure can relate to your helpless feeling of knowing that the mets are there and slowly growing . Be assured that it's on all of our minds every day . I remember having the same talk with olga back in 2014 after we had both of Joshua's lungs treated in Germany . We experienced the same urgency to do something to control the growth .

Would you be able to travel to Germany for lung mets removal ?
Have your doctors talked immune therapy?

Have you had bone scans and how is your primary surgeried area looking ?

By contacting the Dr in Germany he could give his opinion on the likely hood of doing the procedure at this time.
http://www.cureasps.org/forum/viewtopic ... as+retired

It's good to hear from you today
Take care
Love
Debbie

Dear Debbie,

I think my family are decided on waiting until my next scan as we would quite like christmas to be as peaceful as possible.

My doctor said drugs may be an option, however since the drug trials ended a few months ago (for drugs like cediranib etc) these drugs are very difficult to access- partially due to our NHS system in the country.
We wouldn't be able to afford a trip to Germany ourselves, but if it came to it i'm sure my parents would find a way.

Primary area is fine.

Have had no other scans apart from chest-CT since about February.

Xxxxxxx

Dear Mandy,

I agree with Debbie. You can send your new CT on CD to Coswig and wait for their answer.
best regards from Finland
mikko

Dear Mandy,
Thank you so much for your thoughtful update. I am so sorry, but given the nature of this insidious metastatic disease, unfortunately not surprised that your most recent scan showed increased growth of your existing lung mets, and the development of new ones. I understand you and your family wanting to have as peaceful a Christmas as possible while waiting for your next scans in January without pursuing any type of immediate treatment. However, I am concerned about you not having had any scans since February except your chest CT scans, and I strongly encourage you to request that your doctor schedule you for a pelvic/abdominal MRI or CT, a brain MRI, and a full body bone scan as soon as possible as these are common metastatic areas for ASPS and it is critically important to find any mets at the smallest possible size to try to ensure the best chance of a successful outcome to resection/ablation/or radiosurgery. You DO NOT want to wait for the mets to become symptomatic!! as is inexplicably/inexcusably/negligently a common practice for many oncologists. Vigilant, adequate, and appropriate scanning is essential in managing and fighting this challenging disease. Please know that you are not alone in your battle, and that those of us on this Forum are here to provide shared researched and anecdotal information and strengthening support and encouragement. Take care dear Mandy, have a beautiful Holiday season shared together with your family and friends, let the bright light of Hope lead you through each day, and keep in touch as you are able.
With special caring thoughts, healing wishes, love, and continued Hope,
Bonni

Dear all,

I'm going in tomorrow to have my check-up chest CT. After some talking, my consultant agreed to give me an abdo/pelvic CT, but not a brain MRI. Instead having a head CT; it's not exactly what I wanted but hopefully it'll be enough.

Debbie, there hasn't been mention of having another bone scan since the one I had in February was completely clear.

We're not sure what to expect from the scans tomorrow, but I'll try and keep you updated...

Best wishes,
Xxxxxx

Hi Mandy
As future reference try to alternate with mri of pelvic area as ASPS is a long term commitment of scans to catch it at its smallest size. Will your doctors agree to perform Mri's of the pelvic area as a precaution to preserve fertility for you if you choose?
The machine being used as a brain Ct scan, how small of pictures /slices will it show the oncologist radiologist ?
We use Mri's because of the detail it shows

The bone scan I feel needs to be an annual schedule as well if possible .

It's good to hear from you
Take care
Love
Debbie

Dear Mandy, Thank you for your thoughtful update. I am sorry and very frustrated that you encountered resistance to your totally justfied request for an abdominal/pelvic CT and a refusal for a brain MRI which is really inexcusable. A brain CT is inadequate to detect ASPS brain mets at the smallest most treatable size!! Your consultant/oncologist seems to lack the necessary knowledge/caring about the critical importance of adequate and appropriate scans for managing and fighting this challenging disease. I would personally seek another doctor who is ain experienced Sarcoma specialist and who is willing to take a more pro-active approach in helping you to manage and fight your ASPS. I don't want to frighten you but we had another English ASPS patient on this Board who received VERY complacent and inadequate scans and care for her ASPS, and tragically ultimately lost her very courageous battle. It is essential that you be very pro-active, insistant, and that you seek treatment from a doctor who is knowledgeable and experienced preferably with treating ASPS, but at least wirh Sarcoma. My very best wishes are with you for very good results on your tomorrow's scans and I will be anxiously awaiting your next update when your time allows. With special caring thoughts, healing wishes, and continued Hope, Bonni