Cure Alveolar Soft Part Sarcoma International (iCureASPS)

This is my first time posting on this forum but I have been reading posts since my daughter was diagnosed with ASPS in July this year (2013). Thank you to all who post here as we have not found a lot of information and this site has been incredibly helpful.

Here is some background information: My daughter was a normal, healthy 7 year old. I was cuddling her one day a few months ago and happened to rub the back of her arm and noticed a lump. I took her to the GP who thought it was probably nothing but sent us for an ultrasound anyway. The ultrasound revealed a mass growing inside her right triceps and they gave us a preliminary diagnosis of rhabdomyosarcoma and sent us to Sydney Children's Hospital (about 5 hours away in our state's capital city).

Once we arrived at the hospital my daughter was given a battery of tests; bloods, X-ray, CT scan (chest), MRI (right arm) and PET scan. The tests revealed a vascular mass inside the triceps and hard up against both the humerus and the radial nerve in her arm but no metastases. She underwent surgery (the day before her eighth birthday) to remove the mass only and we had a diagnosis of ASPS about 10 days later. The mass was 4.5x2.? and appeared to be encapsulated with one small focus extending from it.

Approximately 3 weeks later she underwent a second surgery. They removed the middle muscle from her triceps (75% of the muscle mass), stripped the outer layer from her humerus and skeletonised the radial nerve. She also had lymph nodes taken from under her arm. They could not find any microscopic evidence of cancer in the tissue removed.

She has a long scar that is healing and seems to have a functional arm although it is noticeably smaller and shows some weakness, nerve damage and a more limited range of motion. We were told that chemo would be ineffective and they are now recommending radiation therapy at the site of the primary tumour. The reason for this is negative margins could not be obtained due to the radial nerve sitting against the tumour (if it was taken her arm would lose function). I am quite reluctant to proceed as they have quoted a 10% risk of bone cancer in the future and informed us that as she is still growing her arm would not grow and would be up to 5-6cm (they later revised this to 2-3cm) shorter than her other arm. There is also skin sensitivity and a much weaker bone that could be more prone to breakages after radiation and the tissues in general would not heal as well.

On top of the extensive surgery this is not appealing but mostly I am yet to be convinced that radiation will significantly decrease the risk of local reoccurrence. How likely is local reoccurrence after the removal of a well encapsulated tumour? Can ASPS develop inside nerves? How effective is EBRT against ASPS especially when there is no specific target just the site of an already resected primary? Would brachytherapy be more helpful?

They have told us that if it reoccurs locally her surgical options will be very limited and amputation may be our only course of action. I look forward to any replies and hope that you can help me to make a more informed choice for my beautiful little girl.

Hi Kat, welcome to our forum. I moved your question to the personal updates area and opened a separate topic for your daughter case to keep everything organized.
To answer your question. My son's situation was very similar - the location in the left arm - and the redo surgery, but his muscle was taken whole incl. the nerve and the nerve from the finger was separated and reattached to keep the function. He mostly regained the full use of his arm. The cosmetic defect is not visible at all - it was not visible in about a year and of course not visible at all after 10 year after Dx. He did a lot of special movements and exercises to regain the full range of motion and strength and the time immediately after the surgery is the most important. You should also work on her scar flattening, the surgeon could be able to explain you how.
I would probably avoid the radiation given the high probability of the significant damage it can cause and the low probability it would do any good. ASPS is a fairly not radio-sensitive tumor - it can be killed by the radiosurgery - high doses of the precisely targeted radiation - but use of the conventional doses does little damage to ASPS as it is a slow growing tumor. I do not really understand why they are saying that in case of the local recurrence the amputation would be the only choice left. Why not to redo the surgery again if it is needed.

Hi Olga,

Thanks for your quick reply. Just a few questions, were you able to obtain clear margins with your son's second surgery? How big was his primary tumour? Is the radiosurgery you refer to the same as brachytherapy (the surgeon uses catheters to insert radioactive pellets into a specific location enabling precision and a higher dose of radiation).

In answer to your question re: surgical options. The surgeon and oncologist are concerned that a local reoccurrence will give them very little tissue to remove as they took so much this time around and left her with a minimal amount for functionality. The surgeon also wants to do a third superficial surgery to remove the remaining permanent stitches (clearly visible as a blue line beneath her skin) and to narrow the width of the scar at the top of her arm. I'm thinking that this will be very difficult in terms of healing after radiation?

Lastly could you refer me to any articles/research indicating radiation to be ineffective when negative margins have not been achieved after resection? Also do you know if there are any ASPS specialists in Australia?

Thank you so much for all your help and for providing such an informative site.

Kind regards,
Kat

Kat, when our redo surgery was done there was also no cancer found in all surgical specimens so I would say the margins were clear. There is no article re. positive margins consequences in ASPS specifically, there are the general recommendations to have a RT when the positive margins are not achieved in sarcomas in general but since in your daughter case there was no sarcoma found in the re-resection specimens also I would not consider it a case of the positive margins.
There is a relevant article from MSK in NY which is one of a few institutions that see most of sarcoma patients in US. This report is the first to focus only on the specific subset of patients who did not receive adjuvant RT after a negative re-resection and in general seems to support no advantage with the RT in this situation:

Long-term outcomes in extremity soft tissue sarcoma after a pathologically negative re-resection and without radiotherapy.
http://www.ncbi.nlm.nih.gov/pubmed/18429001
they have a full text here:
http://onlinelibrary.wiley.com/doi/10.1 ... .23493/pdf
but they only had 1 ASPS patient in this group.
Was the nerve involved in the primary tumor directly or it was just growing inside of the muscle where the primary tumor was? I think we have few cases here when the nerve was cleaned of tumor, not sure about the local control though.
The radiosurgery is not the same as the brachytherapy - they implant the radioactive seeds that keep emitting the radiation but you need a target for that as the seeds are usually implanted surrounding the target and I am not sure where they would implant the seeds in your daughter case? In any case the cosmetic surgery would need to be done first.

Dear Kat,
As with everyone who is on this Board, I am so deeply sorry for your reason for being here, but I am very grateful that you found your way to our Web site and that you have reached out for information and advice. Your dear daughter is very young to be diagnosed with ASPS since most ASPS patients are in their mid to late teens or early twenties when they are diagnosed, and my heart breaks that she has been through so much at such a young age. However, based on my 12+ years of ASPS experience and observations since our dear daughter Brittany was diagnosed at age 19, it seems that the younger the patient at the time of diagnosis, the better the outcome in terms of disease progression and metastasis so Hopefully this will be the case for your precious little girl. Although I am not a doctor, I personally agree with everything that Olga has said in terms of avoiding radiation to the primary tumor site since ASPS is so notoriously radiation resistant, and there is such a high risk of radiation damage and debilitating post radiation side effects to your daughter's arm that it seems that the questionable radiation benefit does not outweigh the considerable risks. I would encourage you to request additional scans including an abdominal and pelvic CT or MRI (MRI's are best because they don't cause radiation exposure like CT's do), a brain MRI, and a full body bone scan to ensure that there are no other ASPS mets in those areas of your daughter's body. I understand that a PET scan was done, but based on my experience and knowledge, PET scans are unreliable and can give either false negative or false positive results. You are to be commended for your knowledge and pro-active approach to your daughter's disease which is so very important in this difficult battle. It seems that your daughter has received very good medical care and treatment thus far. I don't personally know if there are any ASPS specialists in Australia. Mick, who was one of our patients from Australia who participated on this Board tragically lost his courageous battle in May 2012 after struggling to find adequate care in Australia and not having the financial resources to seek treatment elsewhere. I am sure that Mick's dear wife Sheree would be willing to share any medical treatment resources in Australia that she found with you, and you can contact her by sending her a private message on this Board. Her name is available on the "Rest In Peace" topic under "Mick from Australia" and you just need to click on her highlighted name. Please know that you are not alone in this battle dear Kat and continue to reach out to those of us on the Board for information, input, and strengthening support. Take care, hold tight to Hope, give yourself and your dear little daughter special hugs from me, and keep in touch as you are able.
With special caring thoughts, healing wishes for your precious daughter, and continued Hope,
Bonni Hess, mother of now 31 year old Brittany diagnosed at age 19 in July 2001

Hello Kat

Our son Josh was DX in 2012, September and we have had a whirl wind of surgeries and info as well. Josh is 33 years old.
I am so sorry for this dx of your so very young daughter. You appear to have a great deal under control or as much under control as can be possible.
The tumor size creates a very hopeful picture as I understand the prognosis
The following link I find helpful to do research from


Click here: Alveolar soft part sarcoma: a single-center 26-patie... [Sarcoma. 2012] - PubMed - NCBI
Please be assured that our hearts go out to you. Continue to be brave and positive

Sincerely
Debbie

Thank you to everyone who has replied on the forum and sent me a personal message. Since my daughter's diagnosis I have been unable to find any other families battling this disease and it is good to talk with others who understand (although it would have been infinitely preferable to meet under more pleasant circumstances).

We are continuing discussions re: radiation therapy. My daughter is fortunate to be under the care of a very committed paediatric oncologist who is going out of his way to seek opinions and advice from specialists around the world re: my daughter. I read about Prof. Judson and requested our oncologist seek his opinion and he has done so along with Sheri Spunt and a colleague at the Dana-Farber Cancer Institute in Boston. If anyone knows of any other relevant specialist who is working with ASPS and particularly with paediatric patients please let me know, as I am open to suggestions.

My gut feeling, along with my research and the opinions expressed by forum members are pushing me toward rejecting RT at this stage but I believe in being well informed and making the best choice possible so I continue to seek any useful input as the decision needs to be made soon.

Thanks and best wishes to all,
Kat

Kat

Just a thought. We were given a 15% chance of recurrence on the upper thigh, however we had positive margins. We choose no radiation
We were also told that to monitor the area, upper right thigh, with mri, would show lots of activity as the surgery was still new.
We, as parents, have to weigh the benefits verses the non-benefits of each step.

Hope this day finds you with lots of info and lots of joy!

Debbie

Dear All,

Just a quick update. We haven't said no to RT yet but we are pretty close to doing so. We have had advice back from one sarcoma specialist recommending RT and a further three opinions from specialists treating ASPS suggesting a wait and watch closely approach. They all agree that it is a difficult decision!

Olga, in answer to your question about the nerve, it was not involved in the tumour but was sitting against it. Previously you mentioned scar flattening, I haven't heard of scar flattening before but will ask about it, we have been massaging the scar with "Hiruscar" several times a day, attending physiotherapy, trying to stay active and eat a low sugar/ whole foods diet to assist with healing.

Bonnie, thank you so much for your comments and advice. We will talk with our oncologist about additional scans as well as the proposed scanning schedule as this is an area of concern for us as well.

Debbie, the doctors haven't discussed the impact of surgery on her MRI scans. We will ask about this as we are due for an MRI of her arm soon - thanks.

On a positive note my daughter had her first physio session yesterday and did really well, although we have a lot of work to do to build her strength back up. Meanwhile she is loving riding around on her new scooter, playing with her friends and regaining some normalcy.

Kind regards,
Kat

Kat
Hope this day is bringing you joy
So glad to hear your daughter is doing well
In talking with L , who has a grand daughter just recently DX, I hope that you all have been able to find a good oncologist who is working really hard for your daughters benefit to research and give you the most updated information on ASPS
As the group will tell you, finding a good knowledgable oncologist who thinks outside of the box can be difficult to say the least. Take some time to choose one as this disease is a life time commitment for everyone.

Scans will be the most important tools as they see tumors at their smallest size
Ivan has a software that he can share with you to keep all the scans in files and where you are able to compare them yourself.
Please take care and keep up the great attitude. There is a light at the end of the tunnel. Sometimes it just doesn't feel like it
Much love
Debbie

Kat
Other places here in the USA that are sarcoma experts are NIH,(national institute of health ) NCI ,
(national cancer institute )MD Anderson in Houston Texas and MSK (Memorial Sloan Kettering )in New York .

Debbie

Sent from my iFone

Kat,
Sorry to hear about your daughter's diagnosis and your difficult decision.
Like my mom said, we didn't do RT, but that was due to metastases already being present in my case. However, my first doctors who I saw here in KC all were suggesting RT if the cancer had been at an earlier stage, but none of them were sarcoma specialists.
I have since consulted with Dr. Noah Federman in UCLA's sarcoma unit, but at that point had put RT out of my mind. He may have some insight in to your daughter's case, as he has also seen Kevin and some others who post on these forums, but I doubt he can give you any definitive answer, unfortunately. It's such a rare disease and there is so little info on it.

Good luck, I know it is a hard decision and it goes without saying, but take your time. It is evident you are doing your best at gathering info, and that's all any of us can do. Keep us updated with what you find out.

Dear All,

Thank you to all those who have posted with support and advice, I really appreciate what people have shared. We have said no to RT and have just returned from Annika's first scan (an MRI of her arm). The scan was clear! We will go back in three months for another MRI of her arm and a CT scan of her chest. The oncologist has said that they will not do an MRI of her brain unless there are neurological symptoms and that it is unnecessary for staging as her first CT scan of her chest showed no metastasis and brain mets wouldn't occur without lung mets first? I'm interested to know what others think about this?

Kind Regards,
Kat

Hello,
I would do the MRI to cover all bases...

Kat

Geat news with the clear scan on Anika's arm!
I understand that there are not any tumors in Anika's lungs and I don't know that the lungs are secondary to the primary..then possibly the brain. What I do understand are scans will be the most important tool as they see tumors at their smallest size. As I understand it the scans can show abnormalities and are used to create a baseline for further scans that will need to taken for the rest of our kids life's
I am sure others will also contribute an opinion as well
Take care
Debbie