Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi everyone... my name's Will Nocon. I ilve in San Diego, CA and I've been battling ASPS since 2002. My original tumor was in my left thigh and they found some mets in my lungs (like 4 or so). I had surgery to remove the tumor from my thigh (lost 70% of my quads), had a rod and screw inserted into my femur and went through chemo (iphosphomide and one more I can't remember the name of) and radiation therapy. For two years we monitored the mets in my lungs. In 2004, I had a partial lobectomy of my left lung, which removed three of the mets. Unfortunately, more started popping up in both lungs around 2007. We waited three years just monitoring them. Finally in 2010 I went through the CyberKnife procedure to radiate two of the new ones (the others are still too small to try and radiate. I thought things were under control and we just had to monitor these mets in my lungs. Unfortunately, I found out this week that the cancer has now spread to my right scapula. I read on here how some people may have had it spread to their brain and maybe even the kidney. I'm hoping to get scans done for those because I've been experiencing pain in both of those areas as well. I'm hoping the cancer didn't spread, but we'll see.

I'm looking forward to hearing some of the advice/stories any of you all may be able to share with me.

Dear Will,
I am so very sorry for your ASPS diagnosis and for your recent disease progression, but am grateful that you found your way to our Web site and that you have joined the Discussion Board and reached out for shared information and advice. The history of your disease sounds very typical of ASPS with the primary tumor location in your thigh and then the metastatic spread and indolent growth. Where are you receiving your oncological care and who is your primary oncologist? Hopefully you have been receiving regular chest/abdominal/pelvic CT scans at least every six months as well as annual brain MRI's and full body scans which are critically important in monitoring and managing this unpredictable disease. If not, it is very important that you schedule these scans as soon as possible especially since you seem to be experiencing symptomatic pain in your brain and kidney area. There are viable treatments available for brain and abdominal organ mets, but the mets need to be treated at the smallest possible size to provide the best chance of a successful outcome to the treatment. Radiofrequency ablation (RFA) may be a treatment option for your scapula met. It was successfully used to destroy a met in our daughter Brittany's tibia in 2005. Once you have had all of your scans, determined the extent of your disease progression, and obtained treatment for any new mets, you may want to consider/pursue a systemic treatment like Cediranib or Sutent to try to prevent the growth of new tumors, stabilize your disease progression, and shrink/destroy any untreatable mets. Please know that you are not alone in your battle. I and the others on this Board are here to help you and to share researched and anecdotal experience information, as well as to provide strengthening support and encouragement. Take care Will, know that my best wishes are with you, and keep the Board updated as you are able.
With special caring thoughts and continued Hope,
Bonni Hess, mother of 28 year old Brittany diagnosed in 2001 at age 19

I've been getting pretty good care at Scripps Healthcare here in San Diego. My primary oncologist is Dr. Joan Kroener. She's been having me get CT scans every 3 months or so to monitor my lungs and an abdominal CT scan every year as well. The pain in my kidney area is pretty recent, so I'll be asking for that, along with a brain scan. I've been pretty happy with the care so far, but this new met in my shoulder was a pretty big downer for me. I'm still hoping for the best.

Thank you very much for your kind words. I'll be looking into some of the other things you had suggested in your post. Thank you for that information as well and I'll be sure to keep this thread updated.

Hello Will
Welcome to the forums! Please, when you have time copy and paste this post you made to the Personal forums here is a link
http://www.cureasps.org/forum/viewforum.php?f=4
Wow! You sound like me and we are watching also what maybe in my lungs and i have been lucky so far and the few that are in my lungs are in the 2 mm and not moving stage. Like you i had this also in my back area in my 8th rib and 4 inches of the rib was removed along with 5 things in my right lung *if i remember 5 things is correct* and only two or three were ASPS.
You live close to me and if you need to talk MSG me and i will give you my cell number i am on La If you have a surgery to the bone i have some tips for you and dam good Onc to also see if you wish
There are a few of us in the La area you are not alone!

This cancer sucks but as i just posted to Jessie with staying on it with scans and treatments there are many long term survivors of stage 4. There is a post called The 15 year club.. it is in Personals. I suggest that everyone that reads here when they are feeling down to read it! It works for me and for a few others i know

Hello and welcome, Will

Have you been getting MRI's of the brain? How about CT scans of the abdomen? What is the current plan for dealing with the scapula met?

Have you discussed systemic treatment - cediranib - with your oncologist?

How big are you lung mets? I believe it's the first time I've heard about cyberknife being used on lungs. I think ablation (cryo) would probably be better.

Hi all

To answer a few questions, the mets in my lungs are only between 1cm and 2cm in greatest length. CyberKnife appears to have worked so far on the ones we targeted. Those mets have stabilized and begun to shrink. The others have not had any growth since the summer (nothing was done to them yet). Brain MRIs will start regularly for me now and we've been doing abdominal CT scans periodically. As for the scapula, I have my radiation simulation tomorrow. The current plan is to radiate for 10 sessions. Ortho surgeon felt radiation would be much better than removing my scapula, which I totally agree.

I have never heard of cediranib, I will look into it and talk about it with my oncologist next week.

Thank you all for all of the very helpful advice, my wife and I greatly appreciate it.

Will, I have a question re. the met in the scapula - what size is it? What do they say, is it the real bone met or the invasion from the close located lung met in the apex of the lung? I would say that the current plan is to radiate it for 10 sessions does not sound exactly right to me as generally ASPS mets and primary tumors are very resistant to the conventional irradiation (and since we are talking about 10 sessions it is not some type of the radiosurgery like Novalis, CyberKnife or Synchrony that have better chances to be successful but are not used for the bigger sizes of the mets or for some locations so that might be the reason it is not on the table now). It might be the reason they do not propose any type of the local ablation like cryo or RFA although I would definitely get the second opinion from the interventional radiologist Robert Suh (if he still works in the LA area) as he is one of the experts in the ablative technologies and if you do let him know that we have documented case of the succesful RFA ablation of the bone met in one of out patients Brittany Hess and he can contact her doc for the more info if he needs to, I am sure that Bonni would be able to provide the name and phone # in Seattle.

Dear Will,
Thank you for the additional information. I agree with Olga regarding her advice to pursue either ablation or radiosurgery for your scapula met as a better treatment option than the radiation which is being planned. As Olga said, ASPS is notoriously radiation resistant, as we heartbreakingly found when Brittany underwent unsuccessful 50 gray photon radiation to her residual spinal tumor. The radiation seemed to only stimulate the tumor's growth and only a short time into the radiation treatment the tumor devastatingly began to rapidly spread down Brittany's spinal cord necessitating an emergency very major spinal surgery. Depending on the size and location of your scapula met, I think that radiofrequency ablation (RFA) or Cryoablation would be the best treatment choice since it has proven success for ASPS mets including Brittany's tibia met, and the secondary choice would be one of the radiosurgery treatments that Olga has suggested. Regarding Cediranib, I encourage you to read the entries in the Cediranib topic on this Board. Cediranib is one of the most promising and effective systemic treatments that we have found during the past nine and half years of Brittany's ASPS battle. She is currently on a Cediranib Clinical Trial in Edmonton, Alberta where she has thakfully had 21+ months of disease stability and dramatic shrinkage/disappearance of her multiple mets, and several other ASPS patients are participating in the Cediranib Trial at NIH in Bethesda, Maryland where most have had disease stability and tumor shrinkage. I will be glad to provide you with any further information that you may want/need regarding Brittany's RFA treatment, her Cediranib treatment, or anything else that you might want to discuss, so please feel free to contact me by e-mail at BonniHess@aol.com and I will give you my phone number. I Hope that you will discuss the alternative treatments for your scapula with your oncologist before proceeding with tomorrow's radiation simulation. It is extremely important that you explore all possible treatment options and that you be very well informed an knowledgeable as possible before proceeding with any ASPS treatment because ASPS is so extremely rare that many (most) oncologists are unfamiliar with which treatments may work best for ASPS, and those which don't. My continued best wishes are with you Will. Take care, keep in touch, and keep the Board updated on the outcome of your discussion with your oncologist and your treatment decision.
With special caring thoughts and continued Hope,
Bonni

So I've been reading around the board and around the internet, in all of your opinions, are tumor resection and ablation the best methods of getting rid of this disease? I've read that while Cediranib can stunt the growth or even shrink them, resection still is recommended. I am going to ask my radiation oncologist tomorrow about the ablation and CyberKnife (since it appears that it worked for two of my mets in my lung). I know he's probably going to tell me the CyberKnife needs to be able to target big enough tumors. While the one on the spine of my scapula is certainly large enough, the other two are not. I got a little spooked reading about your experience with radiation. i did have radiation before but it was on the primary tumor's site after resection. The ortho surgeon, who's seen other ASPS patients and is well-respected in San Diego for his knowledge in bone tumors, felt radiation would be best suited.

Thanks again everyone. I'll keep you updated.

Will, may be the problem here is with the multiple mets in one scapula bone so if they do the ablation/radiosurgery it can break? Both of these procedures create the area of necrosis in size bigger then the original met by at least 10 mm as a safety margin in order not to leave any sarcoma cells in the area and the size of the scapula probably would not allow for a few necrotic zones to be created. Did I understand you correctly and you have few mets in one scapula? Are all of these mets in the bone or in the lung/pleura area under the bone?

Hello Will
I had a good size tumor on my 8th rib and we stayed away from radiation because of the lungs! Please, talk this over again with the doctors and look into what the others are posting. There advice has helped me alot! I had 33 radiation treatments to my right calf and they do not go away and they creat other problems in the long run at times! Again i am close by and if you or your wife ever need an ear or would like to also talk to others Oncs to get 2nd opinions let me know. May i ask who your Onc is on San Diego :/

Olga, that's what I was thinking. There are multiple mets in one bone. I was going to ask my doctor how fragile my scapula would be if we were to do ablation. The surgeon already told me if I lose the use of my scapula, I'd be very limited in the use of my right arm. Resection wouldn't work with there being multiple mets on a small bone. I would think freezing/heating a tumor would cause some fragility with whatever is left.

Amanda, my doctor's name is Joan Kroener. I do trust her. She's taken care of my godmother and has taken care of me as well. She does encourage me to seek second opinions and has always been open to me suggesting things. She does consult other oncologists as well as doctors within Scripps Healthcare. As for the radiation, the mets appear to be on the surface of the bone, and there are ribs between the scapula and the lung. So if I do have the radiation, I think the effect on my lung would be minimal. I will make sure to ask about that though in my appointment today. Thank you also for offering to speak to my wife and I. I will take you up on the offer some time soon.

Thanks again!

Will - when you see your radiation therapy doc, can you ask him what were the results of the radiation treatments in his previous experience that he's done to other ASPS patients he worked with before? Our experience with the ASPS bone mets is limited by what we hear from the people here on the board, may be he knows some better cases when it worked out OK?

So I had my appt and simulation today. I did ask my doctor about the effects of radiation and whether this will really work. Of course, I got the, "your cancer is rare" speech I've become so used to hearing. He said in most cancers, you still have to go case by case but use what's been done before as a jumping off point. Since the CyberKnife worked on my lung and prevented reoccurence on my leg, he's pretty confident it will work on my shoulder. I'll let everyone know how it goes.

Dear Will,
Thank you for your continued updates and shared information. Are you having Cyberknife for your scapula mets or photon radiation? Cyberknife, like Gamma Knife and Synergy radiotherapy, allows a significantly higher focussed dosage of radiaion to be directed at the tumor than photon radiation does, which is critical in treating radiation resistant ASPS mets. Because of the dangerous location of Brittany's spinal tumor adjacent to her spinal cord, Cyberknife could not be safely used without risking paralysis, and photon radiation was the only option. Unfortunately, as I have previously told you, the high dose photon radiation was totally unsuccessful in destroying the spinal tumor and preventing its spread. As you have found in your research, resection and ablation continue to be the best treatment options to remove/destroy ASPS tumors, but if resection/ablation is not possible because of tumor location, number, or size, then radiosurgery like Gamma Knife, Cyberknife, or Synergy seems to be the most effective treatment to shrink/kill the tumor(s) if the tumor(s) is/are small enough ( less than 2 Cm.) to respond to the treatment. However if tumors are located in an area of the body where they cannot be safely and successfully resected, ablated, or treated with radiosurgery as was the case with Brittany's met which was located in the head of her pancreas, then a systemic treatment such as Cediranib is needed to try to shrink and destroy them. Cediranib is unfortunately not a permanent cure (and tragically there is not yet any available permanent cure for ASPS) since resistance and disease progression eventually have occurred in patients being treated with it, but it has been one of the most successful and promising new treatments for ASPS thus far in providing stabilization of the disease and tumor shrinkage. Once your scapula mets have been successfully treated, I would encourage you to at least explore and discuss Cediranib or another systemic treatment with your oncologist to try to stabilize the progression of your disease.
I am grateful that you feel confident about Dr. Kroener's care of you and that you trust her as this is very important. Is she a Sarcoma oncologist and does she have any other experience treating ASPS patients? It is very important that you be treated by a Sarcoma specialist since ASPS is so extremely rare and most regular oncologists have very little knowledge about it.
My very best wishes are with you Will for a very successful outcome to your radiation treatment and good news resutls from your brain MRI. I will be anxiously awaiting your next update. In the meantime, please take care and keep in touch as you are able.
With special caring thoughts, healing wishes and continued Hope,
Bonni