17 yr old daughter from Alberta - Dx July 2017

[gray12]

Ugh... I have been reading and re-reading all the posts here for the past 2 weeks. My heart goes out to all of you and celebrate all your successes. My daughter was just diagnosed with ASPS, she has a 6.5 cm tumour on her shoulder. This process started back in May, when we first noticed the mass. We had ultrasound, MRI on the shoulder and then biopsy to confirm. She had a chest xray that came out clear, but realize that more precise imaging must be done to rule out mets. We are just trying to wrap our heads around it, which I know all of you can understand. We met with RadOnc yesterday, they want to do 3 days low dose 90/ml Doxrubicin and then 10 days 30/grey radiation. If we choose not to do chemo, we would do 25 days 50/grey. She is scheduled for a CT scan to map out the radiation as well as a PET scan in the next week. It is my understanding that they want to shrink the tumour before surgery. We are definitely leaning toward no chemo, and it doesn't sound like they want to do surgery first. My question is who can I call for second opinions and how do I go about it. I have also signed up for the Facebook group and am grateful for whatever advice, help and prayers anyone can offer.

[D.ap]

Hello
My name is Debbie and our son was diagnosed at the age of 32 in 2012. 5 years ago this month .Joshuas was in his upper right leg and was around 6.5cm as well .

I truly remember the utter surprise and the overwhelming feeling when we were told of the 1 in 10,000 chance, that our son was that 1.

Are you all visiting with a sarcoma specialist at this time ?

Olga , one of the administrators of icureasps ,is up in your area and Jessica Boufford , who is on the face book page .
I would ask the cons of the high radiation to your daughters shoulder bone and if it can interfere with surgery ?

With ASPS it typically is encased which helps in getting what is know as clean margins. This can make it much pro than con, for surgery compared to possible radiation collateral damage , if that makes sense .
We luckily found an excellent surgeon as a referral from our sons primary physician and he performed positive margins in light of muscle etc removal .

I hope this helps
Debbie

[D.ap]

Our son
http://www.cureasps.org/forum/ucp.php?i ... ompose&u=4

Olga is in Vancouver
Her son is Ivan
http://www.cureasps.org/forum/viewtopic.php?f=4&t=66

[gray12]

Thank you Debbie, your sons story gives me hope! We are seeing an Orthopedic Oncologist , who specializes in Sarcoma. There is a Sarcoma team here in Calgary and we will be treated by them. We did ask about just doing surgery and they seemed to skirt around the request. Thats why we are seeking another opinion but just not sure how we do that. Im just wondering if every doctor would prefer to do Radiation first. One of the RADoncs that we met with yesterday said that Dr'O'Sullivan in Toronto does radiation then surgery with no chemo. My priority is to get the bloody thing out as soon as possible but if we do radiation only, we are at least 9 weeks away from surgery.

[D.ap]

Hello again
We are hoping that Joshua's story can give all the feeling of hope . We ,as a family ,are feeling a great degree of truly being at a time that brings a hopeful feeling with all the developements and resources we have at our fingertips .

Icureasps has been our resource from day 1. We have been thru the proverbial trial and error of what has worked and not worked for the ASPS patients.

It's an ongoing research project and with our input,a cure hopefully comes closer.
Your doctors are not the first to skirt surgery unfortunately . However , have they ever had an ASPS patient before ? Are they aware ASPS is typically radio resistant ? And last but not least , are they aware of the vascular nature of this disease.

These discussed ,above, are all true and doctors should be able to answer.

I understand 100 percent the need to remove the shoulder tumor, but in reality the want to remove the sarcoma should be performed by an experienced surgeon who understands ASPS , which I have no doubt you all have available .

Make sure that you have the absolute most credible surgeon to perform this procedure .

Sounds like your basis of expertise is a good group

Please make sure they reach out to experts as I am sure Olga can supply you with .
Any time touch base
Love
Debbie

[Olga]

Hi, I am sorry to hear that your daughter was diagnosed with ASPS. We live in Vancouver, my son Ivan was Dx in Nov.2003 and it was a rough road since then, nothing was the same after that. My son's primary was in his right shoulder. Chemotherapy or radiation was not recommended and the additional wider resection was done after the initial one when it was not expected to be sarcoma. He had no local recurrence, but multiple lung and other distant metastases. The local radiation does not prevent a possible distant mets appearance as ASPS spreads very early in course of the disease and these malignant cells stay dormant sleeping in different organs. They can die there but they can suddenly start to grow in there and start multiplying. Some people never get any distant mets, the reason is unknown.
Based on what we know about the radiation for the ASPS primaries done as an attempt to shrink the tumors, I am puzzled as why it is even recommended. It never worked for our patients and only caused the long term damage and fibrosis of the surrounding muscules, we had patients that were very sorry they had a radiation before removing the primary tumor. It is well known that ASPS is chemo and radioresistant, there are some single cases of the response to the chemotherapy of the small distant metastases but not the primary tumors. There are the well known physical reasons for that, based on the chaotic blood supply and non-perfused areas inside of the tumors and on the slow dividing ASPS cells. You have to ask them a very direct and simple question - please provide me with the published articles or studies supporting use of the radiation and chemotherapy in a primary tumor setting, with the results that show it worked as planned, reducing the tumor and allowing for the less damaging resection. They will most probably try to provide you something about use of it in other sarcomas, do not buy it please. Sarcomas are very different, like 60 types and most of them are faster growing, so what works for them does not apply to ASPS. The studies I read said that conventional radiation was not successful for the primary tumors management. After that conversation they might be willing to talk re. just surgery. Is there a concern re. resectability or why are they talking about the radiation first?

[arojussi]

I have little to ad here. I would have surgery as soon as possible. Radiation and chemo rarely work for asps and even if they work they will make healing from surgery more difficult. I believe treatment guidliness recommend radiation and or chemo before surgery, but these approaches dont work in asps. If you can find surgeon who believes he can achieve clean margins, then go ahead with surgery. I have found that if surgeon feels confident about operation it usually predicts good outcome. Bleeding during surgery can be dangerouns, because asps is so vascular. Every moment primary tumor isnt removed it can send more metastases to blood stream, that is main reason why I recomment surgery as soon as possible.

[gray12]

Hi again..thank you for your replys, genuinely appreciate them. Quick update... Orthopedic Oncologist was on holidays, so did not get a chance to discuss their proposed tx plan until today. However, a week ago, she did have a PET/CT Scan done that came back clear other than 2 nodules of suspicions on her lungs. They are less than 5mm, and could be scar tissue as she had whooping cough as a child. We are prepared for whatever they may be. She had a CT SCan without contrast today, no results yet. During our visit today with Orthopedic Oncologist, we told him that we did not want to proceed with the low dose doxorubicin/radiation combo as it was explained to us that neither the chemo OR the radiation is used to treat the SARCOMA, but chemo to make radiation work better and the radiation to sterilize the outer edge of the tumour itself. It does abut a nerve bundle in her shoulder which they may be concerned about (just my own after thought, that has not been said to us). Anyway, he then said if these spots on lungs are mets, this changes our treatment plan and the primary would no longer be the FOCUS and we would be referred to Medical Oncologist to be hooked up to systemic chemo to treat the mets. There was no talk of surgery for mets, clinical trials ( which we may not be there yet and I understand) I had EVERY FLAG AND RADAR GOING OFF in my head and heart to say the least!! This goes against all i have read on here! I asked him why, and he said that once mets show up, there is no reason to treat the primary as the first course of treatment because it has already spread! His thought is too take the primary out before it spreads, which in a perfect world we ALL want. After more discussion, at which time, he asked my daughter, who is considered a mature minor and ultimately decide how she wants to proceed, what she wants. She said she wanted it out as soon as possible. After backing him up against the wall, he agreed that he could just take it out, without chemo/radiation or radiation alone,within the next week. Any suggestions or recommendations out there?? I still have a uneasy feeling about proceeding with this specialist and wonder if anyone of you have been in this position as well. We are seeking second opinions from Alberta Childrens Hospital, Princess Margaret in Toronto. We just want an experienced doctor that respects what we decide and will still do an AMAZING surgery for her. Have any of you disagreed with the course of tx laid out by your doctors and still had positive outcomes.??

Thank you in advance~ grateful...

[arojussi]

I had bone tumor once, orthopedic surgeon, who operated me didnt have best bed side manner, but surgery was enormous success. So make sure bleeding risk is considered and surgery is most likely best way to go. Lung nodules can indeed be just scars, but if they multiply and grow treating them with laser assisted surgery and or ablation is still best Treatment. Systemic treatment is usually started whem tumor burden cant no longer be managed with local treatments. This approach only works in asps and there is real science behind it. So if doctors dont want to remove primary tumor or treat lung mets with surgery you can find several scientific articles proving that they are wrong. Your child sounds very smart.

[D.ap]

Hello
I agree with Jussi
Bed side manners shouldn't disqualify a surgeon.
However , how are his qualifications according to those who have had surgery by him?

The surgeon wants to change the game plan if it's determined the lung mets are metastatic tumors . I highly disagree as ASPS typically will not grow in leaps and bounds as most sarcomas will and do. Especially in the lungs.
With that being said , removing the primary and THEN having the time to treat the possible metastatic lung tumors is VERY doable .
The 6cm plus shoulder tumor needs to be removed .

ASPS is known as an indolent sarcoma .

We have been in situations where we have moved on from one primary oncologist to another as they have ignored our request . It's such an emotionally charged time for all .

[Olga]

ASPS is a very rare tumor and most surgeons have not seen one in their life time. But it does not disqualify them from performing a good surgery if their surgical experience in sarcomas in general is sufficient. I am a bit unclear here - did you meet an Orthopedic surgeon or an Oncologist? There are no Orthopedic Oncologists, it should be called somehow different. Was she seen by the pediatric oncologist? They mostly see osteosarcomas, rabdo sarcomas and Ewing sarcomas, all of them are aggressive and more radio and chemosensitive, that may affect his judgement but they have to base their decisions and advice on the published literature. Any time the treatment suggestion is done, ask for the supportive articles to read, say that you need to take it home and think about it. Never decide on spot, always sleep it over. Primary tumor has to go regardless if there are any distant mets, if it is possible without any significant loss of function. If they suggest that these 2 spots might be not the mets, so be it for now, do not allow them to biopsy the nodules, it is not in your daughter interests now. You need to meet the surgeon at the children hospital and to discuss what could the surgery be like - if it looks completely resectable.

[Bonni Hess]

Dear gray12, I just posted a message to you on the ASPS Facebook site, but didn't want to intrude on your privacy by using your name here. I am so sorry to be so slow responding to your posts but my summer was filled with travel, vacationing, and company which kept me mostly away from the computer until now when I am trying to catch up on my ASPS communications. As I told you on my FB message, I am so deeply sorry for your precious 17 year old daughter's July ASPS diagnosis, but am very grateful that you have admirably taken a very pro-active approach and reached out to both the FB site and this Discussion Board for shared information, input, and strengthening support. I am very grateful too that your pro-active research, networking, and perseverance has resulted in the scheduling of resection of your dear daughter's large primary shoulder tumor on Wednesday without her having to undergo the oncologist/radiologist recommended pre-op radiation and/or chemotherapy which would most likely have been unsuccessful and most probably would have caused unnecessary damage and debilitating side effects. Regarding the small lesions that were detected in your daughter's lungs, given the nature of this disease to metastasize to the lungs, the lesions are unfortunately probably ASPS mets rather than scar tissue from her previous Whooping Cough infection. As Olga has wisely advised you, don't allow your daughter to undergo a lung biopsy to try to determine the nature of the lesions as this is a completely unnecessary procedure which could possibly seed tumor cells along the biopsy needle track if the lesions are ASPS mets. If the lung lesions increase in size and number you will ultimately know that they are most probably ASPS mets which will eventually need to be addressed through a surgical thorocoscopy, Cryoablation, laser resection, and/or systemic treatment, and if they remain unchanged in size and number you will know that they are probably benign scar tissue for which nothing needs to be done. Regular and appropriate scans are essential in managing this very challenging disease so that any new mets and increased met growth can be found at the smallest most treatable size. If your daughter has not already received them, she needs to have complete chest/abdominal/pelvic scans, a brain MRI, and a full body bone scan with the chest/abdominal/pelvic scans being done every 3 to 6 months depending on the status of the disease, and the brain MRI and full body bone scan being done once a year unless brain or bone mets are found and more frequent brain or bone monitoring is then required. The removal of your daughter's primary tumor is a critically important step in fighting this metastatic disease because as long as the primary tumor remains in the body, it can continue to disseminate tumor cells into the bloodstream. It is incomprehensible and infuriating to me that doctors continue to recommend against and even resist prompt surgical removal of primary ASPS tumors if they can be successfully resected. I know how very nervous and busy you must be as you prepare for your daughter's Wednesday surgery, so I will close for now with my very best wishes for a very successful surgery with good margins, and a full and speedy recovery for your precious young daughter. My heart breaks for all that I know FAR too well that your dear daughter, you, and your family are going through right now. Take care, know that you are not alone on this difficult journey which those of us on this Board share, stay strong, let the bright light of Hope lead you through each day, and keep this Board updated as you are able. With special hugs for you and your daughter, caring thoughts, healing wishes, and continued Hope, Bonni

[gray12]

Thank you so much everyone! Surgery tomorrow to get this thing OUT~ !! Will post after surgery! I am so grateful for this board and the support that we have received so far!

[D.ap]

Prayers coming your way !
Love
The Pearson family

[gray12]

Good Evening All! Surgery went well! Surgeon says he got CLEAR margins, had to take part of her shoulder joint (which he repaired) and 25% of a nerve bundle, but did say that he was able to move her shoulder while she was still under. It was a pretty major surgery, she is recovering nicely at home, tolerating pain meds and getting lots of rest. She will be in a sling for two weeks and then we will discuss physio I am guessing! Next on the list is complete set of scans, which we will discuss at the follow up appointment next Monday. So VERY relieved to have this bridge crossed! Thank you to all of you for prayers, support and guidance!