Martin from Macedonia - Dx Jan 2015

[MartinBube]

Hello everyone

first of all I'm really glad that I have found this forum where people exchange opinions and it is much more informative than all the stuff you can Google online.

My name is Martin I live in Macedonia and just few days ago I was diagnosed with ASPS.First I have noticed it end of September when I felt pain in my lower back, the pain remained for a week and than stopped completely, I did 2 MRI one with and one without contrast however all the prognosis were that it is something not serious at all. I refused to do punctuation Biopsy just in case it is something that can cause further complication and I removed it on 16th Jan completely with surgery. a week and a day after I got this diagnosis that struck me from nowhere.

my stage is conditiona IA because of size 3*2.8cm and no N0 and M0 were removed on surgery, which I suppose were not to be found on the spot (low back) however starting from next week I will do full body scan to check possible spread.

Since this is very rare I'm not sure that even the best doctors here would know much to help in case further treatment is needed, so anyone that can give any suggestions is highly appreciated. I already tried to contact one hospital in Turkey, and searching now Germany and Austria, possible UK even USA if necessary. I'm ready to go anywhere and do anything just to stop any further progress and hopefully be one of the success stories.

thank you in advance for any help and suggestion and hope to stay in touch

[D.ap]

Hello Martin

My name is Debbie and our son Joshua has Alveolar Soft Part Sarcoma. Such a mouth full everyone here calls it ASPS
I am sorry to hear of your diagnosis however welcome .

The size you were able to finally find the tumor was a nice small size.
The doctors were able to remove it all with what is called negative margins?

There are times that muscle and or spinal cord makes it harder to totally remove

Surgery should be used as much as possible as this sarcoma is very resistant to radiation
There is a Dr Judson in England that may know of a Ssrcoma doctor in your part of the world
I'll try and get the link and post here

Until then
Stay positive and be hopeful as there is much hope to be had !

Sincerely
Debbie

[MartinBube]

Hello Debbie,

thank you very much. Regarding the surgery my surgeon has advised that it has been removed completely and in healthy tissue however I will continue ongoing checkups and maybe do another one.
I already asked if necessary to do another surgery immediately but they said seeing what she saw when it was removed there is no need.

I do have already asked for contacts from UK, Germany, Austria and Turkey. I do have a cousin in the UK that is also looking for me so we can contact the doctor you know in case needed also.

thanks again for the help

[Olga]

Hi Martin,
welcome here.
I am Ivan's mom, his history can be also found here. He lists all his treatments in the first post of it.
ASPS is a very rare disease but in some sense it is a well known rare disease with some clinical studies and even ASPS specific clinical trials. Some bigger cancer clinics managed to get stats during the course of decades. We have some library with the link on the main page. If you are going to look for more articles, use Pubmed dot gov - the articles there are pier reviewed and screened for the proper level of evidence.
About the treatment. So far your treatment is done well, the surgery is done with the clean margins without delays.
Now, before contacting any other sarcoma expert for the second opinion, you need to have the following scans: CT of the chest and abdomen, bone scans and MRI of the brain. Get a copy of the scans on the CD disks and keep them signed at home.
The question if you can to do some treatment to stop any further progress has no answer as of now. In about 70% of the ASPS patients their diseases eventually moves to a metastatic stage, it is suspected that many cases that appear to be not metastatic in the beginning are already disseminated but the micro metastases can not be detected. ASPS usually metastasizes via blood stream. The micrometastases (clusters of few cells) can sit dormant for awhile. In some situations they start to grow. The chemotherapy could be given in attempt to kill the floating/dormant sarcoma cells. There was one Italian study when they were given chemotherapy to all the kids/teens with the non-metastatic ASPS. As I saw from the results, the metastatic rate in these kids short term future was somehow lower than usually, but it is also possible that the kids were not followed long enough to find out that they have found the mets later in their life. It was also speculated that kids may have more chemosensitivity. The author of the study was Andrea Ferrari from Italy and she has probably retired by now.
So as of now, there is no evidence that any chemotherapy given to an ASPS patient on a stage without any visible disease reduces his chances to progress to a metastatic state. You have also take into consideration that "doing something" is not necessarily better than doing nothing - in some cases treatments were found to shorten the patient's lives.
To summarize the situation - as of now you still have a hope to remain in a non-metastatic ASPS cases, but you need to do all the scanning first.

[MartinBube]

Dear Olga

Thank you very much for your valuable information. Starting from next week I will do all the tests necessary, however I'm not still sure although the surgeon has said that it was removed very well since at the time of removal they were not aware of the diagnosis, however I will meet her on Monday and will discuss regarding possibility to have additional surgery done if necessary.

At the moment I hope for the best but as I have read until now this is rare and no one can predict what and when can happen.

as soon as I have any results from my scans I will post the results.

thank you

[Olga]

When you meet the surgeon - obtain the official report from the surgery for your records. There has to be the name of sarcoma (pathology diagnosis), the note if the resection margins were negative (no tumor cells found at the margins) and if there was a vascular invasion.

[D.ap]

Hello again Martin

Olga's instruction is very important because any doctor you talk to will need the report and or scans performed.

Were you able to keep a slide of your tumor?
Anytime you have surgery and have an oppurtunity ask for the tissue for your doctor to archive. It can give a history and or current makeup of the existing tumors and possible drugs that can help in your cure.

This is one of Professor Judsons current trials-

Email at the end.


Important Announcement: CASPS – A Phase II Trial of Cediranib in the Treatment of Patients with Alveolar Soft Part Sarcoma

26th April 2014


OPEN NOW: CASPS – A Phase II Trial of Cediranib in the Treatment of Patients with Alveolar Soft Part Sarcoma

Sponsored by: The Institute of Cancer Research / The Royal Marsden NHS Foundation Trust

Information provided by: The Institute of Cancer Research – Clinical Trials and Statistics Unit

ClinicalTrials.gov Identifier: NCT01337401

Cediranib (AZD2171) is a new, unlicensed drug that has been studied in the laboratory and clinic, and researchers think it could slow the spread of ASPS. CASPS is a small clinical trial investigating cediranib in the treatment of ASPS, which is currently open to recruitment in the UK, Australia and Spain. Further information on CASPS and a list of hospitals involved in the trial can be found at http://cancerhelp.cancerresearchuk.org/ ... coma-casps.
Objective: The aim of this study is to see if cediranib (AZD2171) can help people with alveolar soft part sarcoma.

Eligibility: Individuals 16 years of age and older who have been diagnosed with alveolar soft part sarcoma. Please note that other eligibility criteria do apply – for further details please go to http://cancerhelp.cancerresearchuk.org/ ... coma-casps.

Contact: Professor Ian Judson: casps-icrctsu@icr.ac.uk

Locations: United Kingdom, Australia, Spain


Talk to later
Debbie

[MartinBube]

Thank you Debbie,

I will contact the doctor for sure. Yes I do have possibility to send samples anywhere needed, the complete removed tissue is in the Local institute of pathology and I can take samples when I need for anything.

Starting from Monday I will take all the results and continue with getting the full body scans, and than see what happens.

in the meanwhile I will be sending samples to my cousin in the UK where she will get it to a lab for second opinion and detailed diagnoses that can help in getting proper treatment.

[D.ap]

Martin

Great job in diligently doing your home work and looking to someone else to make doubley sure this sarcoma is ASPS
Second opinions are important as there are simaliar sarcomas that appear to be ASPS

God's speed to you and yours

Love
Debbie

[MartinBube]

Hello everyone

First of all the piece was taken with a negative margin but not radical margin. So now in this week there should be a decision on whether they will remove more healthy tissue. I said that I would definitely do it. just waiting on tomorrows discussion between by oncologist and surgeon. unfortunately here I'm the second case she had with ASPS. Fist one was a woman that had it removed 5 years ago and still living with no consequences reported until now.

just to update on my activities today:

- talked to the pathologist that gave the diagnosis. (took samples that I will send to the Uk for additional analysis, he informed me that the splitting of the cells inside was extremly slow however this is a slow growing thing and it is not very surprising to me, maybe it is slower that the slow) He is not for additional surgery, however he is not the most relevant for that. S

- talked to oncologist (as I mentioned above this is the 2nd case for here so not much experience. Did not even suggested MRI on the head said only CT on the chest. she is the only one treating ASPS here so I must seek help abroad definitely long term. he proposed radiation treatment on larger spot that I'm honestly not sure if will help.

- talked to the surgeon (if necessary she said no problem to do another surgery to remove more muscle and tissue. I'm sure that I will undergo another surgery but it is a must as I can see.)

I will do tomorrow CT Chest and abdomen, MRI head and neck. after that I will do additionally bone scan

it was strange to me the oncologist mentioned PET scan and I told her that cell of this do not react very well but she was not sure, and I'm sure that she is not well informed.

That's all for now, as soon as I have any preliminary result I will update.

thanks everyone for the support and post it has been very helpful to learn more of this disesase since here there are not to many people that know it and understand all the treats.

I'm pretty sure that I will spend some time abroad in a specialized clinic (Austria, Germany, UK or Turkey) because I'm not really sure of the local knowledge. not that people will not help they just do not know how

[D.ap]

Hello Martin

My response to your first post

My name is Martin I live in Macedonia and just few days ago I was diagnosed with ASPS.First I have noticed it end of September when I felt pain in my lower back, the pain remained for a week and than stopped completely, I did 2 MRI one with and one without contrast however all the prognosis were that it is something not serious at all. I refused to do punctuation Biopsy just in case it is something that can cause further complication and I removed it on 16th Jan completely with surgery. a week and a day after I got this diagnosis that struck me from nowhere.

Asps is indolent.. slow growing

Scans are performed on a schedule depending on where the sarcoma is located and if it intrudes and enters the-

1) the bone
2) the spinal cord in your case?

I may be totally off in my observation but this is what I understand in your individual case.

The margins are negative for the ASPS cells however there MAY OF been a "highway " of vascular travelels been establisblished from the primary by the primary tumor in the retriperium area ?

If no sarcoma detected in the shaved tissue ask your radiation oncologist what are the chances of this sarcoma showing back up compared to the the chances of a secondary cance happening with radiation treatments on the surgical area.

Lots to think about

You are doing a great job..

Good thoughts to you and yours

Debbie

Ps Getting the basic scans to begin with is very important

[Bonni Hess]

Dear Martin,
Welcome to the CureASPS Discussion Board and apologies for being so slow in responding to your post but I was away from the computer on vacation in Mexico for ten days. My name is Bonni Hess and my daughter Brittany has been fighting ASPS for the past 13 and a half years and has thankfully had disease stability for the past five years and nine months on a Cediranib Clinical Trial in Canada. I am so sorry for your recent ASPS diagnosis but am grateful that you found your way to this Board, that you reached out to share your information, and that you are so pro-active in researching and seeking information and advice about fighting your disease which is so critically important. It sounds like your oncologist unfortunately has limited knowledge about ASPS, which is certainly not unusual due to the rarity of this disease, but I agree that it will be very important to seek advice and treatment from a sarcoma specialist who has more expertise and experience. My very best wishes are with you for good news results from your this week's scans which will Hopefully show no ASPS mets in any other parts of your body. I will be anxiously awaiting your update on the scan results when your time and the situation allow, and in the meantime, please know that you are not alone in your battle with this extremely rare disease, stay strong, and let Hope lead you through each day.
With special caring thoughts, healing wishes, and continued Hope,
Bonni

[MartinBube]

Hello Bonnie

thank you for your kind words. I'm trying to be as strong as possible although I do have tough moments that in a way put me back in this not wanted reality.

Yesterday I've done a Brain and Neck MRI with contrast. On the first look there was nothing but the final result is not yet published. I will have my CT on Chest and Abdomen tomorrow morning. Afterwards with the result I will see what happens, hope for the best.

I've made contact with a radiologist that is very interested to follow my case and I will be insisting with her and the surgeon to do another radical surgery although the result is to a negative margin but if seems that the negative margins should me more widely cut off to prevent reoccurring.

I did not do a biopsy before surgery because divided opinion whether it is good or not. maybe in that way the surgeon if new what it was going to be was going to cut more, but on the other side I would have made a whole in the encapsulated tumor. Any experience on this?

It is how it is and I'm anxiously waiting to see the results of the scans.

trying to stay strong as much as possible

thank you very much

[Olga]

Martin- did you have an MRI before of the surgery? It is usually pretty definitive in finding sarcomas and its finding would render the biopsy unnecessary.
Could you please provide a more detailed description of the location and what was said in the surgical report. The redo surgery is a very serious matter as there is no tumor now so they can not aim on providing the margins around the tumor. When the redo surgery is done, they usually take the compartment muscles out, it could lead to a disability. Sometimes it is even not possible without the loss of function or at all, if the tumor was pressing against the blood vessels or major nerves.

[MartinBube]

Hello Olga,

this is the MRI report that I had read on 2 different places:
"In the right sacral region just underneath the lipid tissue there is a soft tissue formation which is izo-signal in T1 and hetero-signal in T2 with predomination of the hyper-signal. The formation appears to have rough contours. Towards the dorsal side, the formation appears to have a definite margin without a reaction of the surrounding tissue. Towards the medial and the ventral side the formation shows invasion of the distal parts of the thoracolumbar fascia, and partly in the muscle fibres of gluteus maximus, gluteus medius and the multifidus muscle.
On the diffusion sequences of the MRI the formation shows restriction of the diffusion.
The formation has an appearance of an abscess but other soft tissue formations cannot be excluded. It is necessary for other scans and biopsy to be performed in order to fully differentiate the formation

than this is the report after surgery
"31 years old patient is admitted for surgical removal of tumor malformation in the area of right gluteal region retrosacralis. Removed tumor malformation size egg, encapsulated, with uneven surface and hard consistency. The tumor malformation is removed completely without damage of the capsule. Surrounding tissue was macroscopically with normal structure. The wound is closed in layers.
The tumor malformation is send to pathology for HP analysis. Received HP results no.1092496.
The patient is referred to additional diagnostic examinations: MRI brain, CT chest and abdomen. Is referred to Oncology institute for review and further treatment."

I'm doing the CT today, hope for the Best