15 year old ASPS patient from Denver CO - Dx 2013

[Deborah]

Bonni,

As always, thank you for your support and concern!

The tumor is in the vertebra and biopsy confirmed asps. Unfortunately, we have tried genomic testing and our samples keep failing. We are hoping we can get a viable sample from this latest biopsy. We are frustrated but not sure it would help us anyway. We just keep praying for new advancements in cancer treatment and keeping him heathy until they come!

[Olga]

Deborah, hi,
why are you hoping to change the medication mid December? Is it because there are new bone mets on it? There are the multiple case we know when there is an overall response - all mets are stable or shrinking but there are new or non-responding single mets that can be treated locally - as you done with radiosurgery. It does not mean you need to change the current meds if the rest of the mets are still responding, as long as the overall situation is under control. Also I have a question - are all the new mets that appearing - located in the bones? May be you should discuss cabozantinib - a rare case of TKI drug that is able to reduce bone mets or adding some kind of biphosphonate - in attempt to prevent new bone mets.

[Deborah]

Olga,

Thank you for your post. I guess we were thinking that the disease progressed (new bone mets) so it made sense to look for something new. However, we were not sure of what to do and expressed our concern in switching to something that he may not tolerate as well as the sutent. The final consensus was to leave him on the sutent for now.

Thank you for pointing out that switching may not be necessary at this point. There has been no reoccurance in the areas that have been resected or radiated.

[D.ap]

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...

Hi Deb

What glorious news to hear !
The IMRT procedure of 30 days of 2 rads was in Aug or Sept of 2014 and the tumor is looking every bit of dead in November 2015?

Wonderful news

I'm sorry to hear of the spinal tumor

I agree with Olga in keeping with sutent for the time being and treating the spinal tumor.
How often are you able to have head MRI's?
I wouldn't cut corners on those scans at all.
Make sure your dentist is on board and does exams as well
Thanks for checking in

Love
Debbie

[Bonni Hess]

Hello again dear Deborah and thank you for your very prompt response and additional shared information, Since your son's tumor is in the vertebra and not just adjacent to the spinal cord, have the doctors discussed any concern about the risk of spinal decompression which was a very serious and dangerous issue for Brittany when the unresectable remains of her resected cervical thoracic spinal tumor aggressively and rapidly grew into her spinal canal during the time that she was receiving post spinal surgery radiation thus necessitating an emergency and paralysis/Life threatening major laminectomy. You can read the details of Brittany's spinal met surgery in the Spinal Muscle/Spinal Canal Metastases subtopic in the Other Metastases main topic.
Also, FYI, there are some of us on this Board who have concerns about biopsies of suspected ASPS mets due to the possible/potential risk of the biopsy seeding the tumor cells and spreading the cancer in the body. Because of this concern, my family's personal practice has always been to refuse biopsies of any of Brittany's suspected lesions as was the case when a lesion was seen on Brittany's breast on her chest CT scan. We refused the recommended breast biopsy and instead proceeded directly and immediately with resection of the lesion which, as we suspected, proved to be an ASPS met. A biopsy would have been a completely unnecessary procedure when we knew whatever the lesion was it did not belong in Brittany's breast and needed to be removed. I am perplexed as to why doctors continue to recommend and perform biopsies of suspected lesions in ASPS patients since ASPS is known and documented to metastasize almost anywhere in the body including the lungs, brain, liver, spine, bones,breast, abdominal organs, and several other areas as we have learned during the past fourteen and a half years of Brittany's very challenging ASPS battle. I am not telling you this to alarm you Deborah, but just so that you are aware of this issue and concern and can make an informed decision if the doctors recommend any further biopsies for your son.
I also agree with Olga and Debbie regarding their advice for your son to remain on the Sutent instead of switching to another systemic treatment at this time,and as long as it seems to be working to stabilize the growth of his lung mets. As I told you in my previous post, sometimes not all of the mets respond to the same treatment, and this may be the case with the spinal met which he developed.
All of us in the ASPS Community share your great Hope that advancements will be made in cancer research to help find an effective ASPS treatment and permanent cure sometime very soon. In the meantime, we must all continue to aggressively and relentlessly search and research, share invaluable anecdotal treatment information on this Board, and network with other ASPS patients and their families because knowledge is power in fighting this extremely rare disease.
Take care dear Deborah and keep the Board updated as you are able.
Heart to heart with deepest caring, special thoughts, healing wishes for your dear son, and continued Hope,
Bonni

[Deborah]

Ps. Primary was leg, then sinuses, now l5 spine in the bone. Will talk to the oncologist about cabozantinib. Thanks again.

[D.ap]

Deb
What is the size of the L5 tumor?

[Deborah]

Deb-when he had the sinus issue, we were scanning every 8 weeks. Since things have stabilized, we have gone back to every 3 months for MRI and pet. We also see the dentist every three months.

Bonni-I agree with you on the biopsy issue and will keep that in mind the next time around.
He does have some pain in the back and recent X-ray shows a little "collapse" of the vertebra. We have scheduled a kyphoplasty for after the scans in December if his pain does not go away. The ortho feels there is a chance that by building the vertebra up with this "cement" it may ease the pain. It is a simple procedure- one day in the hospital and then no restrictions afterwards.

Once again, thank you all for your posts. This forum helps immensely. I don't post a lot but I am always on here "researching" and keeping up to date on everyone and all the treatment options.

[D.ap]

Deb

Found this link
It talks of the cement procedure to alieveate pain
It appears to be a pain relief ablation rather than an elimination procedure

Radiofrequency Ablation of Vertebral Alveolar Soft Part Sarcoma Metastasis With the STAR Tumor Ablation System After Attempted Palliation With External Beam Radiation

Wed, 02/18/15 - 10:38 | 0 Comments | 2310 views


http://www.interventionaloncology360.co ... r-ablation

[Vega343]

Deborah,
So sorry to read about your son, this disease is so terrible. My daughter is 9 and battling asps in Lincoln, ne. It sounds like you have great doctors and are using all your resources, including this site. It takes bravery and strength to be this involved!
I am following your story, and just have an option if you and your professionals wanted to shift gears as you mentioned. Although I agree that your plan seems to be stabilizing the existing sites, so keeping with it seems reasonable.
We are hoping our daughter gains enough weight over the next few months to go on a phase 2 trial for cediranib, it is the only one in the us open to kids we have found so I thought I would save you some research if you are ever interested.
The trial isn't a placebo trial and they aim to help the kids, you do need to get them at least your most current scans and reports to start, but Melissa Amaya there was very helpful, and I know they still have 4 or 5 child places to fill. The max age is 16, and if he older than that there may be a whole world of trials open?
http://clinicalstudies.info.nih.gov/cgi ... -0192.html

Please take care, keep us posted, and continue your strength!

Mj

[Deborah]

MJ,

Thank you for sharing. I have been following your story as well.
We are not sure what we are going to do next and have considered a few of the treatments mentioned on this site including cediranib and immunotherapy treatments. We are proceeding slowly in fear that some of the side effects may cause more discomfort and wreck his quality of life. There is so much to digest and consider. In addition, he will be 17 in a few months and there are many other opportunities that are open to him now that he is 16.
If you would like to talk more, send a private message to me and I will share my phone number with you and we can discuss all of this further.

Deborah

[Deborah]

Kyphoplasty was completed without any major complications; however, after the 3rd day, he started experiencing spinal headaches. They sent him for an MRI as a precaution (next one was scheduled for March) and they noticed that the tumor in the sinuses was beginning to fill in and grow again. The radiation to that site was done about 1 ½ years ago and the tumor had a hole in the center of it and was shrinking consistently up until now. We are considering radiation again but to treat the same area again comes with increased risk. We have decided to try a new systematic approach and use the radiation as a last resort.
He is using Pazopanib to start while we attempt to get compassionate use of cediranib. He started Pazopanib 800 mg a few weeks ago and they just backed him down to 400 mg as he was experiencing severe fatigue. It has been a few days, the rash on his hands disappeared, but the fatigue remains. Hopefully, that will get better in time. We will scan again in 8 weeks and make our decisions then.

[D.ap]

Hello Debbie
I'm so sorry to hear of the headaches and the sinus tumor enlarging
I've been reading of CSF.
It can happen in the sinus cavity as well after spinal surgeries.
Could the enlargement be CSF?

https://www.nlm.nih.gov/medlineplus/enc ... 001068.htm

Hugs and love
Debbie

[Olga]

Debbie I am sure that a fluid and a tumor growth would look very differently on the MRI pictures, especially knowing that ASPS tumors are very vascular, so the tumor vascularity is going to be very clearly seen on the scans with the contrast.

[D.ap]

Morning Debbie and Olga

My thought was the tumor was shrinking from the radiation of your sons Sphenoid sinus cavity .
The spinal surgery AND the
Sphenoid sinus cavity surgery both have /had risks of CSF.
The sinus tumor has a hole where the artery had to be embolized?

I thought the MRI was performed at the ER but misread on your last post.

I suspect the MRI served 2 purposes. One to FIRST eliminate spinal fluid leakage and then 2, to look at the sinus cavity. It seems like with having a clear MRI 2 months or less ago , that the spine surgery would be the culprit of the headache?

Debbie, I assume the pazo was started after the spine procedure and before the MRI?

Love
Debbie