Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Dear ASPS Community Friends,
I received the following personal e-mail from Deborah who is the mother of a 15 year old boy diagnosed with ASPS in June 2013. As per Deborah's request I am posting a copy of her e-mail on this Forum. I have responded to Deborah and encouraged her to participate on the Discussion Board when she is able. I am sure that any shared information, input, and support would be deeply appreciated by Deborah.
With special caring thoughts and continued Hope,
Bonni

My name is Deborah and I have been visiting this site since last June when our son who is now 15 years old was diagnosed with metastatic ASPS. The primary tumor site was in his leg. Our treatment started with Sunitinib and 5 weeks of radiation. Then they removed the tumor along with his Fibula last fall. They also discovered multiple nodules in his lungs which we have been treating with Sunitinib. At the time, they scaned the rest of his body along with the brain and did not spot anything else. He resumed the Sunitinib a few months after the surgery, but we found that his leg was not healing so he took a break. Just when he was to resume the Sunitinib,he started experiencing headaches and we discovered he has a golf ball size tumor in his sinuses. Unfortunatley, while we were waiting for the biopsy site to heal, he lost feeling on the left side of his face and then the vision in his left eye. The tumor is pressing on the optive nerve which is now swollen.

He is back on the Sunitinib and is completeing 6 weeks of radiation. We hope that both will reduce the size of the tumor and take pressure off the optic nerve. They can't remove the tumor, but we are having discussions about debulking it in the near future.

We are so thankful for all that post and share their stories on the site. We are not used to posting publicly, but felt it was important to be counted. Please post this where you think it should go.

Thanks for all you do,

Deborah

PS we are located in the Denver area and he is being treated at the Children's Hospital.

Awww geez ((
Hugs you!
I am so thankful you are posting here an so thankful they found this an that a plan is starting to help your son!
What state are you in an do you have a PPO insurance?

When i had my jaw tumor removed an it was a huge head an neck surgery my vision got better.
seems that the sarcoma was causing so much inflamation it was creating an eyeritus condition. that makes the retnas almost detatch
Also huge dental problems Please, make sure they look at scans of whole head including ears an jaw behind ears an full nech an clavical bones...

If they an you decide a surgery is recomened please let me know in a pvt msg an i will send you my phone number so we can talk an so if he needs me i will be there via phone so i hope he will not be as frightened <3

Hi Deborah and family
I am so sorry to hear of your sons diagnosis and double sorry to hear of the tumor causing the headaches and vision problems.
Have you all been able to find a sarcoma doctor who is familiar or is willing to go the extra mile in making sure your son is getting the most updated treatment and information. ?
As you have read time and time again , alveolar soft part sarcoma is not like anything they have seen before. And probably will see in there doctoring life time. So with that being said , the doctor really has to doctor by extensive research and consulting with teams of doctors.
My son Josh is 34 and was diagnosed in late August of 2012. He and his wife have traveled to the national cancer institute , Sloan Kettering and the institute of health on the east coast. They have consulted with and been treated by Dr Rolle in Germany and also have consulted with Dr Littrup in Michigan who performs cyroblation on tumors
They have also contacted people at MD Anderson and talked with those folks "to get their opinion " on this incredibly rare sarcoma .
What I am saying is as you know it is and will be an incredibly difficult road but don't give up .
Ask lots and lots of questions and keep the faith. !
Write when you can and nice to meet you

Our son Joshua's story

http://www.cureasps.org/forum/viewtopic.php?f=4&t=750

Love
Debbie
Ps

Bonni thank you for posting

Deborah
Was your sons leg amputated ?
I am so sorry to ask that of you .

Much love
Debbie

Thank you for your kind words and responses.

In response to some of your questions...
He is being treated at the Children's Hospital in Denver CO. From day one, he was treated by a team of Doctors and they reached out to colleagues throughout the country to discuss treatment options. We also consulted the Children's Hospital in Boston and they confirmed that the approach we were taking was appropriate.
When the tumor was discovered, they scanned his whole body including his head. We did discover nodules in his lungs, but nothing else at the time.
The treatments started with Sunitinib and radiation to the leg. Then he went off the meds and had his fibula resected. His leg was not amputated. About a month or two after the surgery he went back on the Sunitinib and the lungs were responding with some nodules disappearing and many getting smaller. We stopped the meds for a few months because his leg was not healing. We were just about to go back on when he started getting headaches and we discovered the tumor in his sinuses.

Since the last post, it was decided that debulking the tumor in the sinuses would be too risky, so we are relying on the radiation and Sunitinib. He will have a MRI in mid Sept to see if there is any response to the current treatment. If there is no response, our Doctor has suggested trying Pazopanib and/or Cabozantinib. I am also curious about the Cediranib trials and if he would qualify. He will be 16 in January.

Finally, does anyone know an ASPS patient that has participated in any of the immunotherapy drug trials?

Hi Deborah and family,

Thank you for the kind response
I am so glad the leg was able to be saved
How's the healing going today ?
It sounds as though your team is a good one to have.
The important thing for you to do is be as incredibly informed as you possibly can about each and every incident that you have with this disease. I am not trying to scare you or your team but there are windows of opportunities at each incident to make choices for treatments
Discuss the pros and cons so as to be informed , not intimidated.
The biopsy came back positive for ASPS, huh.

I am so sorry. Please talk to Amanda. She has been through a lot with her maxillary issues and has a wealth of information AND love to share.
Much love
Debbie

Dear Deborah,
Thank you so much for writing to the Forum and sharing additional and updated information about your dear son's ASPS battle. Olga or Ivan will probably move your topic to the Personal Updates section so that ASPS Community members can better follow your posts there.
I am holding very tight to Hope that the Sunitinib (Sutent) and radiation will be successful in stabilizing the progression of your son's disease and shrinking his lung and sinus mets. My most positive thoughts and very best wishes will be with him and your family for good news results from his mid September scans which will Hopefully show shrinkage of his mets and stabilization of his disease with no new mets. Since ASPS is unfortunately very resistant to standard radiation, has more focussed radiation like Gamma Knife or Cyberknife been considered to try to shrink/destroy his sinus mets? Regarding your question about the protocol and age requirements for the Cediranib Trial, the contact information for the Phase 2 Cediranib Clinical Trial currrently being conducted through NIH is available on the Home Page of this Web site and I would strongly encourage you to explore it now in order to have any information that you may need in the event that your son's September scans unfortunately show an unsuccessful response to the Sutent. It is always wise to be prepared with another possible treatment option in fighting this very challenging and unpredictable disease. Regarding your question about ASPS patient participation in the Immunotherapy Drug Trials, our daughter Brittany and several other ASPS patients participated in the Dana Farber GVAX Immunotherapy Vaccine Clinical Trial in 2005. Heartbreakingly, it was unsuccessful for Brittany and all of the other ASPS patients who I personally knew who were participating in it at that time, and four of the patients who I knew who had received the Immunotherapy Vaccine have tragically since then lost their courageous battles. However, the concept of an Immunotherapy treatment seems to be a very logical approach to fighting cancer and is being increasingly explored as a possible treatment option for various types of cancer in various Clinical Trials. I am closely following the journey of 15 year old ASPS patient Nathalie who along with her family has been very pro-actively seeking a compassionate use age exception to be allowed to participate in a promisng anti PDL1 Immunotherapy Clinical Trial which she was ineligible for since she is under 18. Thankfully, an incredible amount of mass media attention has just recently successfully gained her the compassionate use exception from the pharmaceutical company and things are moving foward for her to begin the Trial Hopefully sometime soon. If you are interested in more information about Nathalie you can access it at her personal blog which is available at http://www.4nathalie.com/ or you can follow her journey through her Facebook site which is https://www.facebook.com/4Nathalie I Hope that this information is helpful to you. Please keep in touch with this Forum as you are able dear Deborah and know that your son and your family are held very close in my heart and my most caring thoughts.
Heart to heart with deepest caring, healing wishes for your dear son, warm friendship, and continued Hope,
Bonni

Deborah

The home page link to cediranib trial.


http://www.cureasps.org/important-annou ... t-sarcoma/

Deborah
I hope tonight finds your son with less pressure and headaches.
When you get a moment could you let us know how big the tumor was in his leg and what kind of whole body scan was performed? That was a little over a year ago , right?
The pathology report was looked at and doubly verified as ASPS?
Give hugs to both yourself and your son from us all

Night
Love
Debbie given name Debra

Bonnie and Olga,
what about Cryo on the sinus met?

If it were me that's what i would look into thru UCLA...

When they did the inital biopsy they took about a week to give us the diagnosis because they wanted to send it out for a second opinion, which was confirmed ASPS. They then did a full body CT/Pet scan, bone scan and MRI. Becuase it was ASPS, they paid particular attention to brain and lungs. As a mentioned before, we found multiple lung nodules, but brain was clean. The original scans were done last july.

The original tumor was about the size of a baseball. I dont have the exact measurements. ( We did donate some of the tumor to ASPS)

They also did a biopsy of the sinuses and confirmed it to be ASPS.

Hi Deborah

How's your sons headaches doing ?

I know the size of a baseball as I am a sports person Played softball well into my late 40's.
That was quite large for a young person, huh?
Our son Josh thought that it was a pulled muscle then it didn't go away. Thank goodness his case was referred by GP to an oncologist for removal
Is the predominant sinus tumor on your sons same side as the leg tumor?
Amanda had a great idea of using ablation by cyroablation
Some areas are easier to perform than others.
Again maybe Olga knows or can refer you to the proper doctors or specialists in your area

Hang in there mom
Love
From
Moms to mom

Deborah, thank you so much for sharing your son story, this is an extremely rare location for the ASPS metastasis and people should be aware of it. I am hoping that Sunitinib is going to be a good sensitizer for the radiation treatment. Do you know what was the result after the sunitinib+radiation to primary leg tumor - did it manage to kill it, it should be said in the pathology report after the surgery.
Amanda and Deb, the child is currently under the treatment already and it is a sound plan, needs to be completed to see if it works, there is nothing to be done as of yet. Cryo is probably not an option due to the size and location - only Dr.Littrup could say for sure, and again - as mom said the radiation therapy is being done now already. I am moving this topic to the Personal Updates.

Tumor was in right leg and left sphenoid sinus.

As to radiation, the doctors told us from the start that this type of cancer was resistant to traditional radiation but they felt that it MAY reduce some of the bulk. We were told that the radiation did kill some of the tumor in the leg. However, we did not see a pathology report.

What I didn’t mention, is we have been monitoring our son’s legs since the age of six when he was diagnosed with Osteofibrous dysplasia of the tibia, a rare, benign, fibro-osseous lesion that typically is seen within the cortex of the tibia in children. So essentially, he has seen an orthopedic specialist every year since the age of six. We started with specialists on the East Coast and when we moved to Denver, they recommended the Doctors we are working with now.

We stumbled on to the ASPS a month before his scheduled annual visit when his leg collapsed on him. We took him to urgent care and they picked up a tumor like condition in his fibula. Initially, they thought it was Ewing’s Sarcoma, but pathology landed us on ASPS. They believe it started in the soft tissue and invaded the bone.

Anyway, this is all very overwhelming. This “SLOW” progressing cancer is moving faster than we anticipated. We trust our Doctors as they know more than we do, but we do feel like we need to be more informed. Do we need a copy of the pathology report? Is there a list of things we should be getting copies of?

I am so impressed with all of you! To have the strength and compassion to try to help the rest of us who are feeling lost and helpless is very impressive. Thank you all.

Hi Deborah
Just checking in..
How's your son feeling ?

You should whenever possible and always get copies
You have to ask for them and sign for them
Which I am sure you've been having to do for over 9 years



https://answers.yahoo.com/question/inde ... 421AAQ7VbC

CDs of scans , reports ie pathology and doctors who've seen and treated your son.
A notebook and or data file helps when you need to reference .

Since it's been determined that your son has ASPS unfortunately it will be a lifetime of scans and treatments until we find a cure
The folks before me, 2012 ,Olga , Bonni , Amanda and so many more have created this website and ALOT of research that we wouldn't have to day. Some have died and some are living with great pain and disseminate disease from this disease. In spite of their pain and suffering they are preserving and sharing so others can learn by there mistakes and more importantly their success.

Thanks so much for sharing the location of the tumors and your sons history
One more piece of the puzzel.
With us all pitching in maybe ASPS will become history

Much love
Debbie