15 year old ASPS patient from Denver CO - Dx 2013

[Deborah]

Debbie,
He is tired, but compared to how he was a month ago, he is great.

The biopsy of the tumor in the sinuses was very difficult and took a lot out of him. They ended up having to do an Angiogram to embolize some of the blood vessels in order to control the bleeding as the tumor is feeding off of the carotid artery. So he ended up intubated and in ICU as a precaution.

Once the radiation started, his pain level was so high-we had a hard time controlling it without having him completely zoned out. In the long run we found the right combination of Decadron and Oxycontin.
Since then, it has been a bit of a rollercoaster ride with the radiation and steroids. He finished radiation on 8/17 two day after school started. We weaned him off the steroid just after he finished, but the swelling got worse, so he went back on. This week is supposed to be the peak week for swelling and it seems to be taking a lot out of him, but he has not missed a day of school!

[D.ap]

Deborah

I am so sorry to hear of your sons biopsies complication as a result of having this damn ASPS..You are both so brave.
I sure hope the Doctors are working day and night to help your son with his swelling and size of the tumor so as to create relief from the pain

Thank you for response back as I can only imagine how hard it is for you all.
Please feel free to write ANYTIME day or night as we are all here for either a listening ear or to give an opinion.

all our love
The Josh P family

[Amanda]

"Oxycontin"I hate! an they gave me that with delated in the hospital an i was seeing things! I was so frightened

Do you have a safe pillow and blanket for him to take to the hospital? I would suggest you get them it helps me an i am not a tulle child i am a mother and a baby hehehe


after my jaw surgery i have a stuffed kitten i named Ginger she is a orange tabby. let him pick things that make a comfy signal inside him or the dryer sheets from home that have a home smell i would also suggest! BUT if he is having a lung surgery no smelly things at all... or is he has any reactions to things again no smell good stuff... maybe the perfume mom used when he was a baby? let him smell it an see what it reminds him of
I pray it is being a baby warm and safe with mommy

Ok hugs an hope an lots of love to you all! <3

[Deborah]

Update on the now 16 year old!

The radiation on the tumor in the sinuses appeared to have worked. Last couple of scans show the tumor in his sinuses shrinking and they are pretty sure it is dead. There has been no change on the spots in his lungs and no new "hot spots". He is also still taking 25 mg of Sutent. The 6 weeks of radiation was very exhausting and it took at least six months or so for him to regain his strength but he is stronger now and feeling pretty good. We are hoping that he will actual get to enjoy his summer this year!

[D.ap]

Deborah,

; >3
Such great news!!
Your son has been on sutent for almost a year is that correct?
How often are you having scans?

My hope is too for you all to have a great summer.
My heart and prayers are with you.

Love
Debra , Deb

[Deborah]

Deb,

He was on the Sutent two years ago and after his leg surgery, we made the mistake of taking him off so his leg could heal.(Drs said we were taking a risk) He was off for three months. Just as we were going to put him back on, we found the tumor in his sinuses. He has been on it for about a year now since then.
He was being scanned every 3 months. After this latest tumor they changed it to every 2 months. His last couple scans have looked really good so we are back on a 3 month schedule.

Deb

[D.ap]

Hello Deb

We ourselves are on a 6 week schedule with Joshuas brain scan what a nice opportunity for a guarded sigh of relief.
Really good news!
How's your sons pain doing ?Do the doctors see a time in the near future to remove the sinus tumor ? I realize it is being fed by an artery.
Have you been able to get off steroids ?
Also Deb was your son taking Sutent during the radiation , SRT ?treatments of ? dosage?
There are phase II trials testing the two at the same time
Unfortunately not a lot to report at this time
http://www.bioportfolio.com/resources/t ... ancer.html

You all have a joyous weekend and update when able

Love
Debbie and family

[D.ap]

Hi again Deb

You may of already read these aricles but I found them interesting and possibly helpful
"What’s new in nasal cavity and paranasal sinus cancer research and treatment?"
ACS-American Cancer Society

http://www.cureasps.org/forum/viewtopic.php?f=3&t=1092


"Response to sunitinib in combination with proton beam radiation in a patient with chondrosarcoma: a case report"

"Conclusions.
Our study is the first to show that sunitinib has beneficial activity in chondrosarcoma and may be safely combined with proton beam radiation therapy. Randomized phase II or III clinical trials are needed to assess the role of sunitinib in this disease and as a radiosensitizer."
NCBI and NIH case study of a normally radio resistant sarcoma like ASPS
http://www.cureasps.org/forum/viewtopic.php?f=2&t=1093

[Deborah]

Deb,

In response to some of your questions...His pain is better. We got him off the steroids in November and off the pain meds by Christmas.

He did take the Sutent during the radiation . He started with 37 mg. At some point, we dropped it down the 25 mg. As far as the dosage of the radiation, I can't remember and I will get a copy of the records. I just remember that we pushed the dosage and the amount of treatments to the acceptable limits.

The risk is too great to remove the tumor, however, the radiation and Sutent have reduced the size of the tumor significantly and it gets smaller with each scan.

Deb

PS-During the radiation treatment, the tumor did get larger and they had to adjust the area they were treating.

[Olga]

Deborah - thank you for an update, it is interesting. ASPS tumors usually do not respond well to a radiation treatment (unless it is a radiosurgery that basically burns everything in its focus). In your son's case the sinus tumor grew much faster than usual and this is the reason it responded so well to radiation, faster growing tumors respond well to radiation and traditional chemotherapy. It is now well documented that the tumors make up in the same person might be different - the mets differ from the primary tumor and between themselves depending on the location - the micro-environment of the host organ influences it a lot. We ourselves observed that Ivan's mets in the lungs usually grow slower than the brain met and the adrenal mets but also in the lungs there are faster growing and slower growing mets.
What type of the radiation treatment did you son get?

[D.ap]

Deb
I am so glad to hear of pain levels down and as well as off steroids for your son . You guys are tuly brave in the endurance AND perseverance of your sons primary to sinus treatment

What kind of reduction from base line scans has been noted and what kind of radiation was administrated ?
This seems to be a much needed information for your sons age group as well as us all. The size as well as treatment at 16 so gives us a time line if you will of progression plus treatment and the sucess of the 2!

When is his next scan?
Be assured that we are with you heart and soul
Love
Debbie


..

[Deborah]

Type of radiation- conventionally-fractionated IMRT - Dosage was a total of 6o grays spread over 30 days or 2 grays per day.

[D.ap]

Thanks Deb and family for the info

We had limited radiation to Joshuas brain tumors over a period of 3 treatments of 30 gray , as we scheduled systemic brain treatment approx 45,days after specific DNa damaging /interrupting chemo of temador as an adjunctive effort to combat micromets
I , we ,so hope that the radiation is currently helping alieviate the pressure and reducing the tumor size without issues

Much love
Deb

[Deborah]

Hello,

Latest update: The tumor in the sinus is dead and continues to shrink thanks to the radiation.-Lungs are stable. Unfortunately, another tumor popped up on the latest scan on his lower spine L5. We decided to keep him on the sutent while attacking the with the radiation. For us, the radiation combined with the Sutent has been the only success we have had with killing the "inoperable" progression.
We now need to decide what systematic approach to try next but until we figure that out, we are staying on the Sutent- as the last time we went off the Sutent the disease accelerated. Currently, the dosage is 25 mg and he has been able to tolerate that dosage with very little side effects.

The next scans are scheduled in mid December and we hope to change the medication then. The Oncologist was talking about trying Bevacizumab or Pazopanib. Taking the next step is scary as he has been able to live a relatively "normal" life so far and we don't want sacrifice his quality of life with a systematic approach that will only work for six months. I see the posts about Cediranib or MEDI4736 and don't know if we should be pursuing those drugs or if they are even an option for him. We are confused about the next step...

[Bonni Hess]

Dear Deborah,
Thank you for your thoughtful update with the VERY GOOD!! news that the combination of the IMRT radiation and Sutent appears to have been successful in destroying your son's sinus tumor and that his lung mets have remained stable . However, in the midst of this great victory, I am so sorry and saddened that a new met has now been diagnosed on his lower spine. As those of us in the ASPS Community know far too well, the journey with this unpredictable disease is a very difficult roller coaster of ups and downs, and we need to celebrate and savor the victories to strengthen and sustain us through new challenges. One of the very frustrating and challenging things about this insidious disease is that just as all patients may not respond to the same drug or treatment, not all of the tumors may respond to the same treatment as appears to have happened with your son's Sutent treatment since the Sutent thankfully seems to have been successful in stabilizing the growth of his lung mets and possibly contributing to the shrinkage and death of his sinus tumor, but yet was unfortunately unsuccessful in preventing the growth of his spinal met. Our daughter Brittany's Clinical Trial oncologist has appropriately described this as " the tumor not being a team player". What is the size of the new lumbar tumor, and has it been determined if the tumor is adjacent to his L5 vertebra or has it invaded the bone? Hopefully the combination of the IMRT and the Sutent will again be successful in shrinking and destroying this new met just as it appears to have been with the sinus met. I agree that he should remain on the Sutent while he is undergoing the spinal radiation treatment to prevent rebound and disease progression. Since he has shown a successful response to Sutent which is a targeted TKI, it might be advisable for him to try another TKI such as Cediranib or Pazopanib, although based on the anecdotal results posted by patients on this Board, Pazopanib unfortunately does not appear to be very successful in providing significant tumor shrinkage or sustained disease stability for those ASPS patients who have been treated with it thus far. Once again, each patient may respond differently to the same drug so it is a difficult decision. It might be worth having a molecular tissue test done on your son's resected primary tumor tissue to try to determine the molecular makeup of the tumor and what targeted treatment might be the most effective. I am personally not very familiar with the promising new PDL-1 immunotherapy drugs such as MEDI4736, but they seem to be showing success for some ASPS patients as per a post by Rachel in the PDL-1 topic on this Discussion Board so this treatment is certainly worth exploring and discussing with your son's doctors. My very best wishes are with your son, you, and your family for very good and encouraging results from your son's next scans in mid December that will Hopefully show a very successful response to the IMRT treatment for the newly diagnosed lumbar met with significant shrinkage and disappearance of the tumor, and continued stability of the lung mets with no new tumors elsewhere in his body. In the meantime, I Hope that you will be able to enjoy a beautiful Thanksgiving shared together with family and friends, and one which is filled with the blessing of continued Hope. With special caring thoughts, healing wishes for your dear son, warm friendship, and continued Hope,
Bonni