Cure Alveolar Soft Part Sarcoma International (iCureASPS)

My friend Larry was diagnosed with ASPS 3 days ago and is meeting with radiologists and oncologists at Roswell Park Hospital in Buffalo this week. ASPS is a scary disease with no clear treatment answers, so I hope some of you might offer suggestions and insight.

Larry is 62 years old and physically fit. His primary tumor is in the front of his thigh and impacts his femur. He has 2 mets in his lung. I don’t know the size of any of the tumors. He has had a biopsy and a central body CAT scan, but not a brain scan or a bone scan.

I called NIH about the cediranib trial and was told another trial would be better for Larry (where the patient is put on either sunitinib or cediranib, and then switched when the first drug stops helping). NIH also told me about the financial benefit of participating in the trial. I read in this forum that a lot of you favor cediranib, but what do you think about this new trial where you get either sunitinib or cediranib, and then switch? What about going to NIH in Maryland for the trial?

What about a getting a second opinion? I know that many of you recommend it, but where would you suggest Larry go? Dana Farber? Sloan Kettering? Any particular doctors you would recommend?

What about chemo?

One last thing-–Larry has a lot to live for. He has a wonderful wife and family, and he plays an important role in his granddaughter’s life. Larry was a runner, golfer, and bowler until his thigh pain got to be too much. He still works full time and laughs easily. His friends and family want to keep him around as long as possible–so thank you for any help and insight you can give.

Dear Jenn,
I am so very sorry about your friend Larry's recent ASPS diagnosis, but am grateful that you found your way so quickly to our Web site Discussion Board and reached out for information and input from our ASPS Community members. Larry is fortunate to have your special friendship, caring, and support. It is unusual, but not completley unknown, for someone Larry's age to be diagnosed with ASPS since most ASPS patients are typically in their late teens or early 20's and 30's when diagnosed. Because ASPS is so extremely rare and little known by most general oncologists, it is extremely important for Larry to be treated by a sarcoma specialist. I am not sure if Roswell has a Sarcoma department or not, although one of our Board members, LCMA, was previously treated at Roswell before changing to Johns Hopkins during the past year. Both Memorial Sloan Kettering and Dana Farber have highly respected Sarcoma departments. Our daughter Brittany receives her primary oncology care at Seattle Cancer Care Alliance (SCCA) in Seattle, but she participated in a Clinical Trial at Dana Farber in 2006, and her excellent former SCCA Sarcoma oncologist,
Dr. James Butrynski is now at Dana Farber. Dr. Butrynski has worked with several ASPS patients at Dana Farber so he is experienced in treating this disease and I would highly recommend both him and Dana Farber for at least a second opinion. It is very important that Larry have a full body bone scan and a brain MRI ( a brain CT is not adequate because it can't detect very small brain mets). I am not a doctor, but based on my ten+ years of experience and personal observations during our daughter's challenging ASPS battle, I would strongly recommend that Larry have his primary tumor in his thigh surgically removed (resected) as soon as possible before proceeding with a systemic treatment in order to reduce his tumor burden as much as possible and better enable the systemic treatment to be more effective. Following resection of his primary tumor, I think that the Cediranib/Sunitinib (Sutent) Trial at NIH would be a good systemic option since it offers two of the currently most promising medications for ASPS. Since it is a Clinical Trial,the medication would be paid for by NIH, whereas Cediranib is not yet FDA approved and available by prescription and if Larry were to try Sutent prescribed by his oncologist, he and/or his insurance company would have to pay for it since Sutent is now FDA approved and very expensive. NIH also assists in transportation and housing expenses for Clinical Trial patients which is a huge benefit.
I know that Larry, his family, and you must be very overwhelmed with everything right now Jenn, and I know that I have given you a lot of information to try to digest, but please know that I am here to try to answer any questions and to help in any way that I can with shared information and experience, so please feel free to write me on this Discussion Board, or to contact me by e-mail at BonniHess@aol.com. In the meantime, please express my best wishes to Larry, know how very appreciative I am of your reaching out to help your friend, and keep the Board updated as you are able.
With special caring thoughts, healing wishes for Larry, and continued Hope,
Bonni Hess, mother of 29 year old Brittany diagnosed with ASPS at age 19 in July 2001

Jenn,
welcome to the board. I suggest you to have Larry involved here in this board discussion, from your description he looks like a very active person and it would have been beneficial for him to take the charge over the treatments and educate himself about the disease and this board and the info on the web-site's main page is a great source of the information. It will be hard for you to reiterate what we say.
Despite that you are right and there is very little info about ASPS treatments out there, the surgery was found to be the best way to prolong patient's life, with other treatments being only moderately active and mostly of temporary duration. There is a library link on the main page of this web-site, find an article from MSK - they advocate the surgery for the primary and the mets whenever it is possible, it is proven that people live longer when the primary is resected and the mets are resected or controlled by the local ablation. The systemic treatments like sunitinib or cediranib are reserved for the situation when the resection is not possible and such are the inclusions in the clinical trials incl. the ones at NCI - only people with unresectable disease have to go for it, as the surgery is his best chance to live longer.
My son Ivan lives with the metastatic ASPS for the last 7 years, he had multiple surgeries but his quality of life is good. If Larry's primary gets resected, his few lung mets may get resected as well or locally ablated and he may start to enjoy his previous favorite activities again. He has to consult with the multiple surgeons before he starts to think that his primary is really unresectable. I would consult at the MSK - they have few very good surgeons, than with Dr. Martin Malawer at Washington University at sarcoma.org , at the MDACC, may be people here can advise you on the closer located surgeons that he needs to see to get few opinions before to proceed. Once again, you have to understand that the clinical trials are not the treatments - they are just trying to find out if it is going to work and we know that it is usually doesn't when the primary is in place, while the surgery is the treatment.

Hello
I am sorry you also have ASPS.
I have this also and I am close to your age meaning I am not a teen. There are a few things at the stage and age you are that maybe different and though I love Bonnie with all my heart I strongly feel that chemo at this point would be the wrong choice. Rebound is just one reason that i would not with the small amount of tumors you have that can be removed *Hopfully* or watched. Chemo can create more problems in somone that is stable. There are others like myself that are doing surgeries and not chemo and doing fine. Some go as long as twenty years between surgeries. Chemo would weaken the immune system and I fear create more cancer activity.
My story is in the PERSONAL area of this forum under Amanda R . Or read IVAN he is also only surgeries...
Please ask any questions and i will post back to you.
Also please read in the lung area at others experiences.
My advice would be to see a sarcoma specialist and then have the tumor removed as fast as you can with a surgeon that has a lot of experience with ASPS / sarcoma.
A regular oncologist cannot deal with this tumor!
So,
Scan bones and MRI brain with contrast.
CT- pelvic, abdomen and lungs with contrast
Have tumor in thigh removed in the next few weeks! This is changing and must be removed as fast as possible!
See a thoracic surgeon in reference to the two possible tumors in the lungs and have then removed or wait to and see if they are stable. I would wait and see about stability three months. There are times that they stay stable. Mine are stable I have eleven things in my lungs still in the mm size no changes.
Get scanned every three months and watch for ANY new tumors.

This was my surgeon and a few others with sarcoma and he has done amazing things!
The area he has the main tumor is not done well will make it hard for him to be as active as he is now and that would not be good. Please, either see Dr Brian or find another doctor with his background to do this surgery!
Earl Warren Brien, MD 310-423-9887
http://cedars-sinai.edu/Bios---Physicia ... en-MD.aspx

Keep laughing it is great for the immune system..
Be strong and be a fighter…
Be your own advotae by reading and learning. You will end up teaching the doctors at times more then they know because here in this foum you have the experience of the paitents information.
Knowledge is power!

Hello
Olga.. I wanted to say I agree 100% with what you said
"The systemic treatments like sunitinib or cediranib are reserved for the situation when the resection is not possible and such are the inclusions in the clinical trials incl. the ones at NCI - only people with unresectable disease have to go for it, as the surgery is his best chance to live longer"

Doctors I have spoken with that have seen a good share of ASPS and others are also saying this about ASPS. Also this patient is age 62 this means that like me he may have had this for a very very long time and it is just very indolent an stays almost stable. So my point is age may play a role in ASPS and how I am not clear on.

Chemo will also NOT be tolerated as it would be in a younger person and this must be also considered! I hope that mine remains as it has for the last three years. But, I feel that age an hormones may play a larger part in ASPS then published or looked at and that chemo when started and these factors of age and stability low resectable tumor load are present can create more issues and new growth.
Cediranib though an amazing drug is one I would chose as you said if I had no other ways of dealing with ASPS or as last choice for myself. It is a tuff drug from what I have read to stay on for some and the rebound is very bad! For our patents that have been on this with amazing results… there are also reports of the ASPS going wild!

There are other drugs that are keeping are ASPS family stable. I am at this point also questioning if they are keeping it stable or was the disease already stable an the Oncs just went to chemo? Make sure you are NOT stable before a drug is used is my opinion. ASPS is slow growing in many and three months if the tumors are mm an very few is a much prudent an safer choice.. Again this is my opinion.
Chemo for some is a great option but make sure you are NOT stable when you start any drug because ASPS is in some very indolent an in some it just turns off an no one knows why. Many of these paitents are not on line posting so the numbers are unknown how many there are with ASPS that is just sitting there not growing.

Many Oncs go to drugs way to fast and if they read these forums and others stories an studied ASPs as we do I think they would also draw the same conclusions an back off drugs unless surgeries and whatever other situations leave no other option like a very large tumor load.

Try less toxic choices before going to cediranib and other great yet strong drug with many very life changing side effects… Be sure to have a plan for if this drug is not working or they remove you from taking the drug because of the side effects because the re bound can be very bad.
Chemo is saving lives in some of our ASPS family and for this I feel blessed for if my condition changes there are choices and hope! As Bonnie whom I consider to be the most loving person I have been blessed to meet these drugs are saving her beautiful daughter!

My feelings and choices are mine an have been developed for myself and the advice I would give others that are also in my situation with age and tumor load. I am not a doctor I am a ASPS patient.
I advise before you make any choices that they are ones that are well educated guesses. At this point even the oncologist’s are making educated guesses in reference to ASPS. An again, don’t be surprised when you question something and they actually look at you and say “Ya know what I agree with that”
<3

Thank you, thank you, thank you for your suggestions! It feels like Larry was tossed into a labyrinth. He is lucky to have such kind guides to help him decide which way to turn.

Larry is meeting with a surgeon today, Dr. McGrath, who is with the Roswell Park Cancer Institute sarcoma team and has experience with ASPS. Larry met with the radiologist yesterday. Surgery is planned for Larry’s primary site in eleven days–-that is the good news. The bad news is that the radiologist said Larry has more than two mets in his lung. Larry does not know how many lung mets he has or the sizes.

Larry is waiting to hear whether his insurance will cover a second opinion, but he may not get one anyway because he feels comfortable with the Roswell doctors. Larry is going to call NCI to get more information about the cediranib/sunitinib clinical trial. Larry said that Roswell plans to give him a drug–-he thinks it is cediranib.

I will share with Larry your opinions about whether or not to take drugs. And I will suggest that he get the other scans and MRI you suggested. I will also ask Larry to read your responses, so nothing gets missed or misinterpreted. I will ask if he wants to join your forum and/or read the posts. I am concerned that this is all overwhelming–which is why I am trying to help.

I’ll keep you posted.

Thank you again,
Jenn

Hello jenn,
It is very overwhelming! If he wants to talk i will give you my cell number please msg me in the forums i cant post my number here...
Tell him i am a mother an i know the fears and what he is feeling like with his children! he is NOT alone an just let me know if he wants to talk.
Some times paitents cant post or read about there diagnosis and they have friends or family that read and talk for them. Though this can work it will be alot of work an stress on your end an if you are willing to do this you are a love

It again IMHO depends on how many and size are in his lungs! Please, read Ivans personal postings..
I am now even more concerned thay they are just sending him to a drug Make them back the need with sugrens stating that the things in his lungs cant be removed! See the surgens yourselfs dont just go with they said this after looking stuff please. Then and only then would i move to a drug. When they went in my lungs 2 or 5 things *I think thats right number* Were not even cancer!

How was the tumor in his leg diagnosed? Was it open biopsy or ? If it was open i see a reason on waiting. If not then get it done before the eleven days if possible.

Dear Jenn,
I definitely agree with and support Olga and Amanda's respected advice regarding the critical importance of resecting the primary tumor and addressing treatment of the lung mets, and not pursuing a systemic treatment unless/until there are unresectable tumors or aggressive and widely disseminated progression of the disease. Surgical removal of tumors, laser resection, radiosurgery (Gamma Knife or Cyberknife), or ablation (Cryoablation or Radiofrequency Ablation) remain the documented best and most successful methods of controlling ASPS and prolonging ASPS patients' Lives since ASPS is notoriously radiation resistant and there is currently no available permanent systemic cure. However, if or when a systemic treatment is needed, I continue to personally feel that Cediranib or Sutent are two of the most promising currently available systemic treatments, although unfortunately neither of these two targeted treatments have proven successful for everyone nor have they provided a sustained long term response for most patients, and there is a risk of developed resistance to the medication and rapid rebound and aggressive disease progression once the medications are discontinued. For the first five years of our daughter Brittany's ASPS battle, we did not pursue a systemic treatment because traditional chemo had not proven to be successful. Instead, we continued to closely monitor her disease with regular scans every 3-6 months, depending on the development of new mets and met growth. This approach was successful until, despite deceiving relative stability of her lung mets, Brittany suddenly developed aggressive disease progression with widely disseminated mets in her liver, brain, tibia, breast, and spine over a period of three years. We treated all of these tumors with either surgery, Gamma Knife, Cryoablation, and Radiofrequency ablation, but in realization of the increasing disease progression and at the oncologist's urging, we sought systemic treatment. Brittany subsequently participated in two Clinical Trials ( GVAX Immunotherapy Vaccine and ARQ-197 C-Met Inhibitor) which were unfortunately unsuccessful. When Brittany then developed a couple of new brain mets and unresectable and untreatable mets in the head of her pancreas, spleen, and small bowel intususception, we had no choice but to try a new systemic treatment which could cross the blood brain barriar to prevent new brain mets, and which would Hopefully destroy her unsurgically removalble pancreatic met which ultimately would have devastatingly taken her precious young Life if it continued to grow, so we found a Clinical Trial with the promising new drug Cediranib in Edmonton, Alberta which has now thankfully shrunk/destroyed all of Brittany's widely disseminated mets and provided her with 29 months of disease stability.
The basic message which I, Olga, and Amanda have tried to share with you is the critical importance of being very pro-active and well researched and informed in fighting this very rare disease. I am grateful that there is a Sarcoma team at Roswell where Larry is being treated and that they have ASPS experience which is extremely important. I am also grateful that they are moving quickly forward with removing Larry's primary tumor. Once the primary tumor is removed, I think that treatment of his lung mets should be addressed, and depending on the number, size, and location of the mets, surgery or ablation may be the best treatment options. Also, depending on the results of his brain MRI and bone scan, I agree with Olga and Amanda that a systemic treatment with Cediranib or Sutent may not be necessary at this time, and vigilant scanning and monitoring may be the best approach. I Hope that all of this information is not too overwhelming or confusing for you, Larry, and his family, but it is very important that all of you be as well informed as possible before making critical treatment decisions. In the meantime, my very best wishes are with Larry for a very successful outcome to his upcoming surgery, and I will be holding him, his family, and you very close in my heart and most caring thoughts.
Reaching out to share deepest caring, healing wishes for Larry, and continued Hope,
Bonni

Hello Bonnie,
I love that we are all on the same page when it comes to treatments!
It means we are learning the best things to do when it comes to ASPS an that we are advancing in many ways from sharing and caring for each other! <3

Bonnie,

My heart goes out to you and your daughter. What a battle you have waged! I commend you for your willingness to help others fighting ASPS while you are fighting your own battle.

After I speak with Larry, I'll post about his treatment plans.

With healing thoughts and prayers,
Jenn

Dear Jenn,
Thank you for your very kind, caring, and gracious words. It has certainly been a very diffcult and challenging journey during the past ten+ years with Brittany's courageous ASPS battle, but we move forward with continued Hope in our hearts that an effective treatment and cure WILL be found sometime soon, and we have found invaluable shared information and strengthening support and encouragement through this wonderful Web site. It is critically important to be very proactive and as well informed as possible in fighting this insidious disease, and to be aggressive and relentless in researching and networking with others to find as much information as possible and any possible new treatments. Shared anecdotal information from ASPS patients and their families is truly one of our most powerful weapons since ASPS is so extremely rare and little known even by many oncologists. I truly consider all of the ASPS patients and their families to be a part of my family, and care very deeply about everyone. Although I am not a doctor, I have ten+ years of ASPS research and personal experience to share so that others can learn and benefit from what we have learned during our journey, and Hopefully avoid making any of the treatment mistakes that we or others may have made. It is an education which we never wanted, but one which we Hope will be able to provide information to help ASPS patients and their families make more knowledgeable treatment decisions. I'll be anxiously awaiting your next update on Larry's treatment plans, and will continue to hold him, his family, and you very close in my heart and most caring thoughts.
With special hugs, deepest caring, healing wishes for Larry, and continued Hope,
Bonni

Larry has multiple lung mets (but the actual number was not disclosed). I have read in this forum that Dr. Rolle lasers multiple mets at once. Is laser the only procedure available for multiple mets? Is cryoblation used for only one or two mets? What about RF ablation?

Is there anyone in the USA who performs YAG 1318 laser surgery in the lung like Dr. Rolle does?

Thanks

Any types of percutaneous ablation are only used for a single digit numbers of mets. There are no surgeons in US that use the YAG 1318 laser - it is still not approved by FDA, this is the reason few people from this board had to travel to Germany to get it done there, besides it is a very technologically demanding tool and you would not like to have it done by someone with the limited experience.

This is Larry from Buffalo and I want to thank all of you who responded to Jenn's posting. I would have gotten on sooner but it seems like the last two weeks all have been doing is going from one appointment to another, one test to another, etc. My surgery is scheduled for Tuesday - no question the tumor has to come out. I am, however, struggling with what to do afterwards. I have mulitple spots in my lungs, probably mets although they are doing a needle biopsy tomorrow to be sure. Nothing in the pelvis or brain, so some limited good news. I hate to rush right into sunitinib therapy - the side effects look hellacious and some articles talk about a rebound effect. Jenn found some articles on laser type procedures used on as many as 124 mets. Don't know if anyone has any experience or suggestions. Thank you again for all your responses and your encouragement.

Hello Larry!
Welcome and i am sorry that you have had to come here but glad you did it makes a difference in decisions an also friends to vent too. I gave my number to your great friend and if you or your wife ever want to talk i am here.
I will be honest i am concerned about needle drag an spread and I would wait and see if you are stable before i made any moves. I and my doctors think i have had ASPS for a very very long time and ASPS can go dorment an or you can be stable for years. The risk of needle drag to myself is not worth the possible problems and it should be just thought that the things in your lungs is ASPS. I dont know the sizes or numbers you are dealing with in yoru lungs so i hesitate on saying some thing wrong. Also when they removed five mm size things in my lungs almost half were not even cancer and what if they do this to you on the wrong one?
I would ask your doctor if it would also be a good idea to wait the three months and then see if you are stable ? You will need this time anyhow after the surgery they will not be able to start another treatment while you are healing anyhow...

I am very happy you will be going in to remove on tuesday an i hope and pray that you will heal fast and they will be able to do this clean and you will be back running around and being you fast!
Let us know how you are doing and if you want some tips pre surgery post or ask your wife to call me