Ellie from England - Dx Sep 2016 at 12 yrs old

[Disco76lover]

Hi my daughter was diagnosed with ASPS in September 2016. She's 12 years old. Her primary in her leg was removed swiftly from her calf muscle (3cm) with clear margins. She had a brain scan, bone scan and lung scan in September when diagnosed. Everything came back clear except one speck on her lungs which was tiny. The next lung ct scan, 3 months later, showed a total of 7 mets. All of which were approx 1mm and too small for an op. The following scan, a couple of weeks ago, identified a few more mets and slight growth on the original ones. This page is uplifting and gives me hope at times. She is on no treatment at present but we are seeing the oncologist tomorrow to discuss options. Reading comments, immunotherapy seems to be the best option? But we live in England and I'm not sure what is available. I will check this tomorrow. Is cedirinab an immunotherapy drug?

[D.ap]

Hi Disco 76

Hello my name is Debbie and our son Josh was diagnosed( dx'd ) in 2012 at the age of 32.

I'm so sorry to hear of Ellie's dx'd

However it's so good to see she has a good advocate for her care ,asking for advice . Stating her case.

Yossi, Olga and Bonni have been my go to folks since 2013 and have not let me down

Here's our sons time line
Our son is currently on Opdivo since July 2016 with great

http://www.cureasps.org/forum/viewtopic.php?f=82&t=1297
results .

However he has had multiple surgeries / ablation before embarking on chemo . Sutent
Prior to the 2016 sutent , Josh had lung laser removal of tumors , and liver cryoblation
We choose not to embark on any Med till we were backed against the wall so to speak ..

Personal section -

http://www.cureasps.org/forum/viewtopic.php?f=4&t=750

Cedirinib is a chemo

Cediranib
Drug
Cediranib is a potent inhibitor of vascular endothelial growth factor receptor tyrosine kinases. The drug is being developed by AstraZeneca as a possible anti-cancer chemotherapeutic agent for oral administration. Wikipedia

https://en.m.wikipedia.org/wiki/Cediranib


Tryosine kinase inhibitors (TKIs )
alter cells and will need to be followed up with additional meds if the tumors are shown to progress ,is what my limited research understanding tells me .
So once the decision is for chemo the " alteration process", the onc team and family need to always be vigilant with the plan B.


I know Olga and Bonni will follow up
My love and hugs to you , Ellie and family .

Ellie's primary tumor was small and in her leg and that is a good prognosis .

There are a host of parents on the forum with young kids that you can PM and ask advice as well
Damian and MJ as well as Bridget ( Joe )to name a few.

Love
Debbie

[Disco76lover]

Ellie's primary scan on her lungs in September, showed one tiny speck which the oncologist didn't think was related to her tumour on the calf or anything to worry about, due it being on its own and so tiny. However, the following scan in December, proved otherwise unfortunately with it having grown slightly and more mets showing up on the scan. The next scan in March, showed more growth and a few more mets. This is the reason for them starting Ellie on treatment and an op not being an option yet due to them not being large enough for the surgeons to see. However, because of the growth and in order to stop the spread elsewhere, as well as the lungs, the targeted therapy is due to start shortly.

[Disco76lover]

Hi Debbie, I've just been reading about Opdivo and it sounds really promising. I am so pleased your son has had a good response to this. I understand you not wanting to start tablet therapy also until you had to and that was our goal too but the increased growth and worry of it spreading to other organs / bone and brain was just too risky not to start her on something. God bless

[Olga]

Hi welcome here.
I am very sorry to hear about your daughter diagnosis. Who is the treating oncologist for your daughter? The most experienced and knowledgeable in specific ASPS area in UK is Pf. Ian Judson, you should ask for a second opinion - if you already did not have consulted him.
I will now tell you what you have to know about ASPS and I am very sorry - because most of it is very hard to hear - at least it was for me 13 years ago when my son Ivan was Dx in Nov.2003.

About the treatment line. I would really recommend you to start asking your oncologist a direct questions for the evidence proved course of action not for the good intentions. Every step has to be evidence based - published good source articles, studies or at least the single cases. Educate yourself to be able to understand the mode of action of the drugs, and their current limitations and problems.
In general, there is no drug that is shown a long term befit for ASPS patient's survival. There are drugs that show activity and temporarily positive effects like reducing the tumor load and even leading to a disappearance of the metastases. There are single cases when people are surviving for the long time after the chemotherapy treatments being in remission but there are many more cases (like 99%) when the response is temporarily termed and resistance to the drug develops as the time goes. In some people the faster then before of the treatment growth resumes in all known classes of the drugs (traditional cytotoxic chemotherapy, TKI (cediranib and sunitinib), immonotherapy etc.), sometimes it is so fast that the advantage of having the slow or no growth is lost and people are having a negative effects for the chemotherapy treatment. Yet there are the single cases of the complete response and event free survival for a long time - we are lucky to have a single member that is having a complete response to cediranib for years - but she is lone from all the people tried. Even the immunotherapy drugs have a very big problem - some people (like 5%) with some specific mutations can have a double or triple speed growth instead of the stooping it.
All the chemotherapy treatments for ASPS are considered to be investigational (no proven benefit) and should be only used when there are no other options avail. as the long term effect is unknown and can be harm instead of benefit. None of them are proven (or even reasonably noted) to be stopping the metastatic spread, most of them are even proven not to cross to the brain.
About ASPS metastatic spread. It spreads very early in course of the disease. 30 mm primary tumor means that the primary was already there for few years at least. All the new mets that are seen in the lungs were there already there - single dormant cells brought there by the blood flow. They stay trapped in the smaller blood vessels until they suddenly grow in. The growing activity is bigger after the major surgery as the grow factors are elevated, the growth has to slow in the next few months (at least it is usually the case). There are might be dormant mets in the brain etc. elsewhere, they might not survive. The mets in the lungs are to small to be able to metastasize - to have that ability they have to be vascular and ASPS mets smaller than few mm are not vascular, they don't even lit up on the PET scan.
So my question is - based on what information the chemotherapy treatment is planned to start? Ask for the supporting articles and lets discuss them.
In UK the laser assisted surgery is available so when the mets are few mm they are the good size for it (Mr George Ladas, senior consultant thoracic surgeon at the Royal Brompton Hospital has stared it in 2010 so should be pretty experienced by now). Some people survive with the help of the lung surgery alone without any further metastatic events.

[Bonni Hess]

Dear Melanie,
Hello again following our Facebook conversations, and welcome to the CureASPS Discussion Board. As with everyone on this Board, I am so sorry for the reason for you being here on this site, but I am so grateful that you accepted and followed my advice to register and participate on this Board, and I Hope that you will find it to be a source of very helpful shared information, experience and research based input and advice, and strengthening support and encouragement on your ASPS journey. Olga and Debbie have offered some excellent information and input and have both tried to tell you the same thing that I shared with you on Facebook regarding the critical importance of weighing all treatment options before just beginning a systemic treatment which may or may not be successful in stabilizing the progression of dear Ellie's disease since there is unfortunately currently no available treatment which can/will provide a permanent cure for ASPS. This is not to say that there is no Hope, as there are several promising new treatments and much progress is being made in ASPS research so Hopefully some day VERY soon there WILL be an effective treatment and permanent cure for this extremely rare, little known, and very challenging disease. In the meantime, it is essential to be as knowledgeable and as pro-active as possible in making any treatment decisions, because as Olga has said, some treatments can cause more harm than good. Unfortunately there is no definitive answer as to which treatment is the best, so treatment decisions need to be made on the basis of researched and anecdotal treatment information obtained from research, documented treatment results data, networking with other ASPS patients/families, and proactive discussions with Sarcoma/ASPS oncologists. Olga's recommendation for a consultation with Professor Judson at the Royal Marsden is a very good one as he is internationally known and respected as one of the most experienced and knowledgeable ASPS oncologists in England. Olga is extremely knowledgeable and has done extensive ASPS research during her son Ivan's 13 year ASPS battle which has incredibly been successfully managed thus far without any type of systemic treatment, but instead through vigilant scanning and proactive resection, laser resection, and ablation of new mets. As Debbie said, there are several young ASPS patients on this Board, and I Hope that their parents will reach out to you, especially MJ whose young daughter Prairie is currently and thus far being successfully treated with Cediranib in a Clinical Trial at NIH in Bethesda, Maryland. You can read about Prairie on this Board if you scroll down to her Personal Updates topic, "9year old, new diagnosis, pelvic primary w lung mets" and contact MJ by posting a comment on Prairie's topic or by private message to MJ if you are interested in communicating with her about Prairie's Cediranib experience and treatment results. Ellie's disease seems to be following "normal" ASPS progression with the diagnosis of lung mets following resection of the primary tumor. If Ellie has not already received them, I strongly encourage you to request complete scans including chest CT scans and an abdominal/pelvic CT or MRI every three months and a base line brain MRI and full body bone scan to be repeated at least once a year unless mets are found earlier. I know that you must be numb and overwhelmed with all of the information and everything that you are going through right now dear Melanie, but I commend you for being so proactive and for reaching out to obtain as much information as possible in the midst of all of your shock and sorrow. You seem to be a very strong, intelligent, and resourceful woman. I Hope that you have a strong support system of family and friends which is so very important in this difficult battle. Please know that you are not alone and that those of us on this Board are here to help you in any way that we can with shared researched and anecdotal treatment information, input and advice, and support and encouragement. Please take care of yourself, and keep in touch as you are able.
With special hugs for you and dear Ellie, caring thoughts, healing wishes, warm friendship, and continued Hope,
Bonni, mother of now almost 35 year old Brittany diagnosed at age 19 in 2001

[D.ap]

Melanie

Here's the info on folks and children who have been dx'd


MJ ( daughter Prairie)

http://www.cureasps.org/forum/viewtopic.php?f=4&t=1108

Bridget ( son Joe)
http://www.cureasps.org/forum/viewtopic.php?f=4&t=1158

Kat and Damian ( daughter Annika )
http://www.cureasps.org/forum/viewtopic ... &start=105
Hugs coming your way as I know how over whelming and daunting this can be.
But be assured we are always here to talk to night or day

Love
Debbie and family

[Disco76lover]

Thank you for your responses. Unfortunately our oncologist has advised now that she doesn't think an operation or radio ablation to remove the mets is an option. This is due to the fact that if they did that, more could appear even if they are large enough to operate. This is why she is preferring to go down the targeted therapy route. However, we need to liaise with her to discuss this as I am feeling that we need to discuss this with. Please can you Tell me a little bit more about radio ablation and thoracic surgery? Also how big do they need to be for them to be removed? Thank you

[Disco76lover]

Prof Judson has retired and he didn't deal with paediatrics

[Olga]

Most of the treatments that Ivan had were done against the advice of the local surgeons and sometimes oncologist. Our long time oncologist retired last year but she was pretty good in sense that she recognized that they often were not able to offer a better treatment plan locally - and would never offer something to do like a chemotherapy of sorts "just because something needs to be done", I always appreciated that in her - a clear understanding of the ASPS being a different slow growing sarcoma and giving a different set of opportunities than the rest of them - i.e. you can have a surgery and yes more lung mets appears and you can have a next one and more mets appears and you can have them cryoablated etc. - and it buys you years of the life time and you might be able to wait till the real cure is found. All the treatments have to be evidence based proven to be beneficial overall - for most sarcomas even temporarily slow down or stop the growth is beneficial as they are fate grown/aggressive but not for ASPS - you need to be a good chess player with ASPS as our thoracic surgeon from Germany Dr.Rolle said once to me in the beginning of our ASPS journey.
To answer your questions:
- re. thoracic surgery. Only laser assisted surgery would be a good fit for the tiny mets as they do not resect them but burn on spot on the open lung palpating it to find the smallest ones. The minimal size is based on the surgeons skills, Dr.Rolle was able to find the 0.5 mm ones - the number of the mets rescted was usually *3-5 times bigger than the one found on the CT scan as it misses the small ones. Consult the surgeon directly.
- re. ablation - we prefer the cryo over the RFA, it is less damaging to the lungs, the size to ablate is about 10 mm and it is only good for the few mets. I suggest you to read the info we have in the relevant topics of the board and ask us any add. Qs.

[arojussi]

My oncologists also advised me against surgeries, but after multiple surgeries they were forced to admid that surgeries were best way to go. This was time before immunotherapy. For ablation I think tumor need to be around 1cm in size.for traditional surgery skills of the surgeon is key factor. Rolle was able to find tumors as small as half millimeter, but nobody is as good as he. So discuss with surgeon and wind out how small tumors he can find and remove. Tyrosine kinase inhibitors are rarely permanent solution so I wouldnt use them at this point. After one thoracotomy more tumors most likely will appear but they can be treated with other surgery or ablation if neccessery.

[Olga]

Also - are you sure that Pf.Ian Judson retired? It would have been a big loss for ASPS community - although we were not always agree with his totally "systemic" approach point of view on ASPS treatment.
http://www.icr.ac.uk/our-research/resea ... ian-judson

[Bonni Hess]

Dear Melanie and Olga, I just spoke with Professor Judson at the Miami ASPS Workshop in September and he seemed very actively involved in ASPS patient treatment and did not mention anything about plans to retire so this is very surprising and disappointimg news to me which will Hopefully prove to be erroneous as Professor Judson is an extremely knowledgeable ASPS oncologist!! I will respond to the post about Ellie"s oncologist's continued recommendation for systemic treatment when I get back to my computer as we are currently aboard our boat with limited Internet access. With special caring thoughts and continued Hope, Bonni

[Disco76lover]

Thank you Olga, jussi and Bonnie for your responses. I will discuss all of these recommendations when I meet with the Oncologist in a couple of weeks.
I am on this emotional rollercoaster which is very new to us and some days I have lots of hope and others I struggle to find it. I am sure I am not alone with these feelings and because of this cancer being so rare, with not much information, this site is invaluable.

Yes I was told at the Marsden that he had retired from there. This does not mean that he has retired from everything but certainly from there.

[Disco76lover]

Please can you send me the hospital name for dr role so I can inform the oncologist in case she needs to send Ellie's lung scans there? Thank you