15 year old ASPS patient from Denver CO - Dx 2013

[D.ap]

Debbie

Unfortunately your son has had a pathology report dx ASPS in his sinuses , however I hope there is further discussion and investigation to the "growing" sinus tumor. I agree with everthing but the biopsy as I know you do too, Deb.

Evaluation and diagnosis
Evaluation of patients with nasal symptoms is often performed by an otolaryngologist and involves taking a complete medical history, performing an examination of the head and neck region, and often nasal endoscopy of the nasal and sinus areas (looking inside the nose with a small scope). If there is any concern for a tumor (mass or growth), imaging studies are performed and may include CT scan, MRI scan and in certain situations PET-CT scan. If a tumor is suspected, a biopsy is typically performed to determine exactly what sort of growth is present. A biopsy involves taking a small sample of the tissue of the nose, and this can be done either in the clinic or the operating room depending on the circumstances. A biposy is important since there are a number of different types of sinus and nasal tumors, and many are treated differently.

http://care.american-rhinologic.org/sinus_tumors

[Bonni Hess]

Dear Deborah, It was so good to hear from you. I am very appreciative of your thoughtful update, but am so sorry and saddened that it appears that there is now renewed and increased growth in your son's treated sinus tumor which had previously appeared to be shrinking and dying. I am Hopeful that the Pazopanib will be effective in stabilizing his disease and shrinking his tumor, that the side effects will dissipate and become more tolerable for him, and I will be anxiously awaiting your update following his next scans in eight weeks. Is your need for a Compassionate Use Exception request for him to participate in a Cediranib Clinical Trial based on age restrictions of the Trial? Based on information from your previous posts, I think that your son recently turned 18? If he is not now 18 and he is too young for the Cediranib Trial based on the Trial protocol, I can provide you with information about one of our ASPS Community families who may be able to help you with your appeal since they succeeded in obtaining a Compassionate Use Exception for their daughter to receive a drug which had been denied to her because of her pediatric age. Please let me know if you are interested in their contact information and I will share it with you in a private message. In the meantime, please take care, know that I am here to try to help with any shared information that I may have, and keep in touch as you are able.
With deepest caring, healing wishes for your dear son, warm friendship, and continued Hope,
Bonni

[Deborah]

Bonni et al,
Thanks as always for your concern and sharing. He just turned 17 in January, so above the pediatric trial age and below the adult age limit. Also, by obtaining CU we are hoping that we can be treated locally.
Also, Cediranib was our Doctor’s first choice but my husband and I are more excited about trying one of the immunotherapy drugs or even a vaccine. We have shared that info with the Doctor and we will discuss with him over the next few weeks. (His tumor was negative for the expression of the PD-L1 but the doctor said there have still been positive results for patients with the negative expression.) See the link below for a discussion on this.
https://www.cancercommons.org/2015/09/0 ... -question/
We would appreciate you sharing whatever information you have on obtaining CU Exception.
Debbie to answer your questions, the pazo was started a week or two after the surgery and the hole in the tumor was from the radiation.

[D.ap]

Hi Debbie

I'm so glad to hear of your excitement for other treatment
Looking at your link.

I hope today brings more answers to your sons tumor growth .

Love
Deb

[Deborah]

Latest update: We tried Votrient and my son was stable for about 6 months. He went off it when there was renewed growth in the tumor in his sinuses/base of his skull. As always, we were discourage that the new growth was not “measurable” enough for him to qualify for any trials.
He is now almost 18 and our insurance approved the use of Keytruda and radiation even though his tumors tested negative for PD1. Apparently, combining radiation with Keytruda increases the response rate. So far he is tolerating both very well. He was scanned after three infusions (infusions every 3 weeks) and the tumors in the lungs are stable (some disappeared) and the tumor in the sinuses was slightly smaller. So we will continue with another 3 infusions and then scan again. I will post after the next set of scans.

[D.ap]

Deborah
Great news ! Lots of folks have had great success with keytruba.
So glad to hear.
When they measured the growth it was from your sons first scan, right?
I've heard that PET scans give a good read on chemo type response
However I imagine they are performing MRI'S?
Love
Debbie

[Olga]

Deborah, hi, it is a very significant development that the insurance agreed to pay for the Keytruda and I am really hoping for the excellent response that could allow for the resection or even regression of the sinus tumor (it can also (probably) be cryoablated if it is small, Dr.Littrup has done a jaw bone cryoablation with the success). Can you please share the name of the treating oncologist so our patient's oncologists (who are unable to get their Keytruda treatments approved) could contact doctor to doctor to build the case.

[Deborah]

Dr. Brian Greffe is his Oncologist (720) 777-6740.

His scans consist of full body PET, CT for lungs and MRI for the head/sinuses.

[ntran727]

That is great news. Keytruda seems to be promising. What kind of side effects is your son having?

[Deborah]

After the three treatments, the side effects have been minimal -mostly mild fatigue which could be attributed to the radiation. They do check his blood every week but so far everything is stable.

[Bonni Hess]

Dear Deborah,
It was so good to hear from you, and I am so appreciative of your thoughtful update. I am very grateful that your dear son received insurance approval to be treated with the very promising new Keytruda immunotherapy drug, and that after his first three Keytruda infusions he is thus far having a very positive and successful response to his Keytruda treatment with minimal side effects . What type of radiation is he receiving in combination with the Keytruda, and are only the tumors in his sinuses/base of his skull being treated with the radiation? When Olga is able, perhaps she will move your posts to a Keytruda topic where your son's Keytruda experience and results can be more easily followed. I will look forward to your next update following your son's next post infusion scans and am holding very tight to Hope for more good news of continued tumor shrinkage and disease stabilization. Take care dear Deborah and know that you, your son, and your family are being held very close in my heart and my most caring thoughts.
Sharing the encouraging good scan results with special hugs, healing wishes, love, and continued Hope,
Bonni

[Deborah]

Only the sinuses were treated with the radiation. Type of radiation- conventionally-fractionated IMRT - 6o grays spread over 10 treatments (2 weeks of 5 days a week). The radiation oncologist was excited as he has seen other cancers respond to the combination of Keytruda and radiation. He is hopeful that all the tumors will show response even though we were just treating the one area.
We just had his 4th infusion and still no significant side effects. Next scans will be late December or early January.

[Bonni Hess]

Hello again dear Deborah and thank you for the additional information. It is indeed very exciting and encouraging that the combination of radiation used in conjunction with Keytruda seems to provide a more successful response than radiation alone since ASPS is traditionally so notoriously radiation resistant. There was a presentation given yesterday by MD Anderson at the prestigious CTOS meeting in Lisbon, Portugal regarding the very encouraging success of Mogamulizumab+ PD-L1 Immunotherapy treatment for ASPS. Hopefully additional information regarding these successful study results will be made available soon. I Hope that your dear son continues to tolerate his Keytruda and radiation treatment well. and that his upcoming late December or early January scans will show continued stable disease and tumor shrinkage/disappearance. In the meantime, take care and have a wonderful coming Holiday season filled with special happiness and strengthened Hope from your son's thus far very positive and encouraging Keytruda and radiation response.
With special caring, positive thoughts, healing wishes, love, and continued Hope,
Bonni

[Deborah]

The side effects from Keytruda continue to be minimal. It has been 9 weeks from previous scan and the tumor at base of the scull is “more dead” and a little bit smaller. The lungs stable. He just turned 18 and is looking forward to college next year!

[Bonni Hess]

Dear Deborah, Thank you for sharing this very good and encouraging news . I am so grateful that your dear son is continuing to experience minimal side effects from the Keytruda, that there is necrosis and some shrinkage of his tumor at the base of his skull, lung met stability, and apparently no new tumors in other areas of his body. What is the status of his sinus tumor? Happy Healthy 18th Birthday to him and continued Keytruda success as he finishes his Senior year of High School and makes his college plans . Sharing your great joy with special hugs, caring thoughts, very best wishes, and continued Hope, Bonni