Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi everyone,

My 29 year old son Matt was diagnosed in November. He complained of constipation and difficulty urinating, and was treated for prostatitis and a UTI by a urologist. His primary Dr. ordered a chest X-ray as part of a routine work-up, and at the same time Matt started experiencing excrutiating sciatic pain. After an MRI he was admitted to the hospital in South Florida for a biopsy of his left flank, and it took a pathologist at Emory to come up with the diagnosis of ASPS.

We are pretty sure his primary site is his lower back, around the sacrum. He has numerous mets to his lungs and also in the buttocks. I guess you could say we're still reeling, and our knowledge of this horrific malignancy is still very basic. I read all of your posts and it amazes me that you all are so knowledgeable.

Matt has been accepted into the Phase II cederinib trials at NIH. We are very thankful for this opportunity but these government "wheels" turn slowly, and we're so anxious to start some sort of treatment. Everyday we wait it seems is another day that his cancer spreads. His oncologist here in Tampa has been helpful in pain management, and he has done all the research and legwork to get Matt into the trial, but I just feel as if we're still in the dark. No one has talked to us about surgical options at all, except to say his primary tumor is "not resectable"

My question for all of you is should we also be seeking the advice of a sarcoma specialist??? If so then who is the best??? Who has had experience in successfully treating advanced cases like his? We are willing to travel wherever necessary, but do you think it better if we wait until after the trial?

I have faith that you all can steer us in the right direction...

Thanks,

Wendy

Dear Wendy,
I am so deeply sorry for your dear son's recent ASPS diagnosis, but I am grateful that you found your way to this Web site and that you have reached out for advice and support from our ASPS Community. I am grateful too that you and the doctors have moved forward so quickly in pusuing treatment, and in getting Matt accepted into the promising Phase 2 Cedirnaib Clinical Trial at NIH. Although I am not a doctor, based on my eight+ year experience with this very challenging disease, since Matt's primary tumor has been determined to be unresectable, and he has other disseminated mets, it seems that a systemic treatment like Cediranib is the best approach to try to stabilize the progression of his disease and shrink/destroy his multiple tumors. However, I do think that he should be seen by a sarcoma oncologist, and a sarcoma surgeon who could possibly offer a second opinion on the resectability of his primary tumor. We were initially told that our daughter Brittany's large spinal met could not be resected without intrumentation which would have made it impossible for her to turn her neck in any direction, so we sought a consultation with another neurological surgeon who was able to successfully remove the tumor without the placement of rods and pins. Also, we were initially told that her parietal brain tumor was unresectable without causing blindness, but we found a neurological surgeon who was able to successfully remove it with only the loss of her peripheral vision. With this very rare and poorly understood disease, it is extremely important that you have an oncologist who is experienced and knowledgeable in treating sarcoma, and preferably ASPS. Dana Farber in Boston, MSK in New York, and MD Anderson in Houston are three of the major sarcoma centers in the United States, although others on this Board may be able to provide you with information on sarcoma centers closer to where you are in Florida. Our daughter Brittany is currently participating in a Cediranib Clinical Trial in Edmonton, Canada, so if you have any questions that I might be able to try to answer regarding her Cediranib treatment experience, or anything else, please feel free to write me on this Board, at my personal e-mail address which is BonniHess@aol.com, or phone me at my Home phone number which is 425-392-7743. In the meantime, please take care dear Wendy, know that your son and you are not alone in this battle, and keep this Board updated as you are able.
With special caring thoughts and continued Hope,
Bonni Hess, mother of now 27 year old Brittany Hess diagnosed at age 19 in July 2001

Hi Wendy,

Welcome to the forum! It does seem that with widely disseminated ASPS, cediranib may be the best known medication. It is great that you are able to start it soon. The days do seem to tick by slowly before entering a trial. They need all their tests, and one has to be off medication etc.

In some cases if the disease can improve and stabilize, it may be possible that surgery could be performed in the future...especially if the tumor is pressing on important structures. If I remember correctly, Mania's sister was a Sutent before having her pelvic primary removed, and our daughter was on a course of Sutent before her pelvic primary was removed. There may be some benefit to being on these drugs before the tumor surgery - as it may reduce the vascularity - making it both easier to remove and perhaps lower the chances of metastases from the procedure.

At the NCI, you will be able to have access to a doctor with fairly wide experience with sarcoma...at least I'd think so. If the NCI is far from home, your oncologist can correspond with your local doctor when you return home. Dr. John Goldberg is at the University of Miami I think (used to be at Dana Farber) - but he had quite a lot of interest in ASPS - and he has always been kind to correspond with us by email. He is technically a pediatric oncologist, but he may be a local resource for you / your docs in Florida.

Hello Wendy,
I know how anxious you are to get started on treatment. It seems Ced trial is very promising.
My son who is 14 is currently on the ARQ 197. We were headed to Boston before being presently
surprised and excited to be able to receive treatment in Miami. As for the Tampa area, Are
you currently at Moffit? I believe Marilyn Bui is familiar with ASPS? Also, at Shands in Gainesville
Dr. Parker Gibbs is an oncology ortho surgeon who is also familiar with ASPS. If you need any
further help, feel free to message me. Best wishes for Matt and your family. This truly is a family
journey.
Peace,
Cindy

Dear Wendy,
I was so grateful to read in your response to Arch in the Cediranib section that Matt has now been approved for insurance coverage of Sutent. It is very encouraging that the insurance companies are now considering Sutent as a viable treatment option for ASPS. Do you have any indication of when Matt will be able to begin his Sutent medication? Also, was resection of his newly diagnosed abdominal subcutaneous met and/or his larger tumors in his sacrum and buttocks discussed or considered as something to be done prior to his beginning Sutent? I continue to think that reduction of tumor load as much as possible by resection, ablation, or radiosurgery is extremely important in enabling the body to better respond to systemic treatments such as Cediranib and Sutent, although this is of course only my personal opinion based on extensive observations of anecdotal patient experiences. Has Matt had any scans since being taken off of the Cediranib Trial to determine if he has experienced any rebound with increased tumor growth which is always a concern when anti-angiogenic TKI medications are discontinued?
My very best wishes and greatest Hope are with Matt for a very successful response to the Sutent and I will be anxiously awaiting your next update. Once Matt begins the Sutent it would be wonderful if you would post the updates about his Sutent experience and results under the Sutent topic so that everyone can follow it there. In the meantime, please know that Matt, you, and your family are in my continued most caring thoughts, give yourself and Matt a special hug from me, take care, have a most beautiful and blessed Holiday season, and keep in touch with the Board as you are able.
With special caring thoughts, healing wishes for Matt, and continued Hope,
Bonni

Dear Wendy,

Its been some time since we heard from you. Hope Matt is doing fine on Sutent.
What dosage is he on? Take care of yourself and please convey our regards and best wishes to Matt.

Arch

Hey everyone,

Yes, it's been awhile since I posted, but I will update you all as best I can. Matt started treatment with Sutent around the first week of December. This was after about a month of no treatment. We aren't sure how that affected him because he had had his last PET and CT at NIH around November 1st. He did have an MRI of the brain in Dec which, thank goodness, was clear. He also had a small surgical procedure to excise the new sub-q tumor under his ribcage. That went well and made us feel better that it could be easily removed. Sigh, I wish it were as easy with his other tumors.

Anyhow, after a short healing interval, his oncologist in Ft. Lauderdale, Dr. Gutierrez (formerly worked at NIH on the cediranib trial as Dr. Kummar's partner) started Matt on 37.5 mg of Sutent. DR. G. felt it better to be on 6 week cycles with 2 weeks off. We're not so sure about that proticol as all the trials were done with no break, but I guess we can re-evaluate that after Matt's PET scan. That won't be until he completes 2 cycles (mid-March).

Of course, Sutent is not without side-effects. Matt has similar GI issues as with cediranib, but has now added nausea and vomiting to the mix. He has a decline of appetite again, and is losing the weight he regained while off-treatment. He is also anemic and neutropenic (low white count) and is exhausted much of the time. He is forcing himself to work about 24 hours a week and I think that helps his sanity, but all he does is work and sleep. His pain is perhaps a small bit less, and that is encouraging.

So we really don't know much of anything yet, which is why I haven't posted. But I haven't forgotten all of you, and you are ALL in my prayers daily...

Hopefully the next scan will bring us some good news and a reason to celebrate. We get the results on Matt's 31st birthday.

Love to all, Wendy

Wendy, thank you for your update, and best of luck!

Dear Wendy,
It was so good to hear from you and I am so very appreciative of your thoughtful update on Matt. I am sorry that he is experiencing some negative side effects from his Sutent treatment, but am grateful that he seems to be having less pain. I am very grateful too that his brain MRI was clear, and that he was able to have his sub-q ribcage met successfully removed. My continued most caring thoughts are with Matt and your family, and my very best wishes will be with all of you for very good news results from Matt's mid March PET scan. Please tell Matt "HELLO", give both of yourselves special hugs from me, and keep the Board updated as you are able.
With deepest caring, special wishes for treatment success and healing for Matt, and continued Hope,
Bonni

Hi everyone,

We just returned from Ft. Lauderdale where we received the results of Matt's PET. After 2 cycles of Sutent it appears that his 2 major Tumors (lung and sacrum) have both grown. The radiologists could not do a proper comparison because they could not open the disc from NIH (how ridiculous is that?) but Dr. Gutierrez WAS able to open it and do his own comparison. He feels that the lung tumor has increased about 2 cm and the sacral tumor about 1 cm. Now of course we brought up the fact that Matt spent the entire month of November off treatment, and has not had a PET since Nov. 1st so perhaps some of this growth could be attributed to cediranib rebound? We also have to account for different PET scanners, different radiologists reading the scans, inflammation, etc. Dr. Gutierrez doesn't buy any of those theories, but he was willing to allow for one more cycle on the Sutent while he (and we) explore other options. Like there ARE any....

Dr. G. has been in touch with Yvonne and Dr. Kumar at NIH to check on the ARQ/Sorafanib study. He is also arranging a consult for us at MGH (Dana Farber)to speak with the Sarcoma docs there. We still have NEVER spoken with a sarcoma specialist yet. I think that's important. If anyone out there knows some names at Mass Gen'l I'd appreciate it. While up there perhaps we can also speak to them about a biopsy, as Matt also has another sub-q growth that can be removed. I know how the researchers need fresh tissue. Maybe that can do some good.

Wish I had better news about the Sutent to share. The irony of this whole experience is that while on Cediranib, Matt's primary tumors were completely stable. IT WAS WORKING.

Wendy, I am sorry to hear that. The name of the main sarcoma doc at the Dana Farber is Demetri. He is the head of sarcoma unit there:
http://www.dana-farber.org/pat/adult/sarcoma/
But why are you going that far for the second opinion? There is MDACC much closer there?
Can you verify something in your post - are you talking about "lung tumor has increased about 2 cm and the sacral tumor about 1 cm" - are these increases in cm or in mm? What are the actual sizes of these tumors now?

Hi Olga,

It's not really so much a second opinion as it is a consultation with a specialist. Dr. Gutierrez has colleagues there, and we'd like to know about the research and clinical trials there. The newest PET does measure in mm. The largest mass is right mid-lung, measuring 105x141x91mm. The left buttock mass is 126x64x74 mm. The sacral mass is 105x103x110 mm. When Dr. Gutierrez made the comparison, he reported the growth in cm.

Dear Wendy,
I am so very sorry about the heartbreaking results of Matt's most recent Sutent status scans and deeply share your pain and concern. Were only PET scans done, or were CT scans done also? If only PET scans were done, they can be somewhat unreliable, and one mm and two mm of growth is fairly minimal. I continue to be concerned and perplexed about Matt's primary tumor having not yet been resected because as I have told you several times before, having a large primary increases the body's tumor burden and I think that it then makes it very difficult for any systemic treatment to be successful because the treatment is having to fight too much disease. I encourage you to discuss possible resection of the primary with a sarcoma specialist prior to beginning another systemic treatment. Dr. James Butrynski at Dana Farber is an excellent sarcoma specialist who was previously Brittany's sarcoma oncologist at Seattle Cancer Care Alliance in Seattle before he moved to Boston. ASPS patients Paula from Poland and Jessie from Phoenix are currently patients of Dr. Butrynski's at Dana Farber, and they are also both currently participating in the NIH Cediranib Trial. There are also three other ASPS patients who are having encouraging responses to three promising anti-angiogenic treatments including LCMA who is being treated with Sorafenib (Nexavar) in New York, Jemisha who is on a Dasatanib Clinical Trial in California, and Clare who is on an Axitinib Trial in England. Please know that my continued most caring thoughts and very best wishes are with Matt and your family, and know that I am here to try to help in any way that I can with shared information and support. Take care dear Wendy, and keep the Board updated as you are able.

Dear Wendy,
When you said that Matt's most recent PET scan measured the amount of growth in mm. rather than cm. I mistakenly misunderstood/misread that the amount of growth was actually two cm. in the lung and one cm. in the sacrum rather than only two mm. and one mm. This amount of growth is certainly much more significant, and although PET scans can be unreliable, there is unfortunately apparently increased substantial growth that can't be attributed to scan error and needs to be addressed. I am so very sorry that the Sutent was unsuccessful in stabilizing the growth of Matt's large tumors and shrinking them, but am Hopeful that the large tumor load can somehow be reduced as soon as possible with resection/ablation/and/or radiosurgery, and then a new systemic treatment can be tried which will be more effective than the Cediranib or Sutent has been. I would also strongly encourage Matt to have a chest/abdominal/pelvic CT rather than PET scan, a full body bone scan, and a brain MRI to more accurately assess the status of his disease and identify any possible new mets so that the best treatment plan and priorities can be determined by a sarcoma specialist.
My heart, my deepest caring, my most special thoughts, my healing wishes, and my continued Hope are with Matt and you.
Bonni

Hi Bonni,

Yes, the new PET was measured in mm's, BUT the doctor did a conversion for us, and told us the results in cm. So, YES, his tumors have grown 2 cm. and 1 cm. He had the CT scans of the abd, pelvis and brain in December, and Dr.Gutierrez also used those as a tool for comparison. I wrote you a pm about our issues with surgery, I'm wondering if you received it. I am hoping to have an appointment set up with Dr. Butrynski in the next couple of weeks. Perhaps he can shed some light on this .

Wendy