Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Aloha everybody, My name is Keola and I am 19 years old from Hawaii. I am new to this website and was nervous at first. I had recently been dx with ASPS by my doctors here and it is definitely a different journey for everyone, so heres mine:
in 2002, i was 9 y/o at the time, i went in to see my pediatrician and showed her a really small lump on my left thigh. It didnt hurt and i could move it, so my doctor just let it go and payed no attention to it. At the time, she told us it was some type of clot or build up.
I lived with this lump from elementary, through middle school and high school, even now to my 3rd year in college.
In May 2012, i was playing football on the street when i had hit my leg really hard on a fire extinguisher, causing the lump to become purple and swollen.
I thought that this pain was nothing more than a bruise, but the weeks went by and the bruise had gone away. However, there was now a pinching pain shooting through my leg every now and then. I went in to my pediatrician thinking this would be the last thing i do with her before switching to an internal medicine doctor. She scheduled me for a CT scan on my leg and it showed a mass(like i didnt already know that), then an MRI on the same leg(which also showed a mass).
I was then sent to a surgeon who told me that the lump was some type of cancer(hit me by suprise), but he could remove the tumor if it had not metastasized and spread anywhere else. I took a CT scan of my chest/lungs, which showed that the cancer had spread(9 spots) to both lungs the largest being 7mm at this moment. 2 CT scans, 1 MRI, and a lung biopsy later, my oncologist broke the news that i had ASPS and there would be no treatment options for me here in Hawaii. My family was devastated because i had always been the health freak of the family, eating fruits and vegis, constantly working out and surfing, they didnt understand how it could happen...
Big news, i was scheduled to go to Bethesda, Washington to participate in the Cederanib clinical trial and had appointment dates and everything. Sadly, the FDA had called and told my coordinator in Washington that i am not eligible because they (the FDA) need to see my cancer is progressing. I am the first person in my family for the past 3-5 generations up. We are new to this but i want to put my story out incase someone could help me with figuring out a way to stabilize or defeat this demon of a cancer.
Mahalo me ke aloha,
Keola.

Dear Keola,
Your story is so sad, and so scary, because it seems many times ASPS goes undected for too long, or is misdiagnosed. Doctors unfortunately just do not seem to grasp what they are dealing with early on. It's such a rare disease. I certainly hope and pray there is a good treatment option for you so that your case of ASPS can be defeated or at least stabilized, as you said. It's a good thing that you've taken good care of yourself thus far with a healthy lifestyle. Surely that fact will be on your side.
My name is Trixi and I live in Houston, TX. I'm the mother of Patti who, unfortunately, lost her battle in August just a few weeks after her diagnosis. But do not let this discourage you. Patti was 44 years old and probably had ASPS for a long time before it was found. None in our family had ever even heard of ASPS before June of this year!
As you can see from the entries here, there IS hope, and there IS help. Many people here in this forum have extensive experience in combatting ASPS and can -- and will -- give you excellent advice.
Hugs,
Trixi

Dear Keola,
I am so very sorry for your ASPS diagnosis which brought you to this Board, but I am grateful that you found your way here and that you have reached out for support and information. Your misdiagnosis for so many years is very frustrating, but sadly all too common since this extremely rare disease is so little known by most regular doctors and even some Sarcoma specialists. There certainly needs to be more education and awareness raised about ASPS for primary physicians so that these kind of mistakes are not made and continually repeated.
It is my understanding from what you wrote that your primary tumor in your thigh has not yet been removed. I don't understand your surgeon's refusal to resect it just because it had already metastasized to your lungs. Based on my eleven years of ASPS experience, observations, networking, and extensive research, surgical removal of the primary tumor if possible is very important to prevent it from continuing to grow to an unresectable size, and to reduce the amount of tumor burden on the body which will help to strengthen the immune system to better fight this challenging disease. Typically the primary ASPS tumor is not encapsulated and it has fingers which can continue to feed tumor cells into the bloodstream so good margins are required to ensure that all of the tumor tissue has been removed.
Additionally, if they have not already been done, you need to have a brain MRI (NOT just a brain CT), abdominal and pelvic CT's, and a full body bone scan to ensure that there are no mets in those areas. Since there is heartbreakingly no current permanent systemic treatment and cure for ASPS, vigilant scanning to enable early diagnosis and removal/treatment of mets at the smallest possible size is currrently the best way to manage this challenging disease. Systemic treatment with Cediranib or other drugs has unfortunately thus far not been proven to be a permanent cure, and often resistance to the drug and disease progression ultimately occur. Systemic treatment is usually reserved for rapidly progressing disease or widely disseminated tumors which can't be resected/ablated/or treated with radiosurgery which was the devastating situation for our daughter Brittany who has now thankfully had 40 months of disease stability and disappearance of her multiple and unresectable tumors on her Cediranib Clinical Trial.
I Hope that your oncologist can help you find a Sarcoma specialist in Hawaii as it is very important that you be treated by someone who has knowledge of, and experience with this very rare disease if possible. It is also critically important that you be very pro-active in your care, and as well informed as possible through research and networking/communication with other ASPS patients and their families. This Board is an invaluable source of researched and shared anecdotal treatment information as well as strengthening support and encouragement from others in the ASPS Community. Please know that you are not alone in your battle and on your journey dear Keola. I and the others on this Board are here to help you in any way that we can with shared information, insights, and input. Let Hope lead you through each day, and know that I am holding you very close in my heart and most caring thoughts. Take care and keep in touch with the Board as you are able.
Reaching out across the miles with gentle hugs, deepest caring, healing wishes, and continued Hope,
Bonni Hess, mother of 30 year old Brittany diagnosed with ASPS at age 19 in July 2001

Aloha pumehana everybody,
Thank you to trixxi and Bonnie for your love and support. Im glad to hear that many people are on the same type of journey that i am. I forgot to mention that i did do an MRI of the brain which came out negative, a CT of the pelvic and stomach area with contrast which all showed up negative. As far as i know, there are 9 mets in both lungs (5left, 4right) and the largest of those mets is 7mm and the primary in my thigh(7cmx10cm). I understand that it is really small, but they do not belong in my lungs or my body at all. I had a few questions though.. How could the tumor in my thigh have stayed for 10 years without treatment, and would it give hint to it being a VERY slow grower? I am still currently having no breathing problems, no eating disorders, or anything physically out of the ordinary. I am still working out daily and surfing weekly, living life as i should at 19. I actually never would have known it was cancer unless i had hit my leg. also, Would removing the primary in the thigh be the best thing to do first? I think, i say think because im not very sure, the plan my oncologist had was to see if the Cediranib trial would shrink some or most of the tumors and THEN remove. but I have thought also of removing the tumor in the thigh first. It would definitely lessen the burden on my immune system and i feel like it can only do good. My family however is scared thinking if i remove the one in my leg, that it would trigger the tumors in the lungs to explode and grow out of control. That image in the back of my mind scares me in more ways than i can explain. has removal of the primary shown any negative side effects? as far as tumors in the leg is concerned. like i said, i am the first in my family for many years to have gotten ANY type of cancer so to be hit with one soooo rare is making it even more difficult.
Mahalo no,
Keola

Hello again Keola and thank you for your very prompt response and additional helpful information. I am so grateful that your brain MRI and abdominal and pelvic CT's were clear. It sounds like your doctor has been very thorough with his diagnostic scans, although if it hasn't already been done, I would encourage you to also have a complete bone scan as ASPS can unfortunately also metastasize to the bones. You sound like a very active and physically fit young woman with a zest for Life and a positive attitude which is very important in fighting this disease. Regarding your question about your primary tumor having been in your thigh for ten years without treatment, ASPS is typically an indolent slow growing disease, but once it metastaizes it can become more aggressive. I am not a doctor, although sometimes I feel that I know more than some doctors do about ASPS after eleven years of experience with the disease and relentless and extensive research, personal observations, and communication with other ASPS patients and their families. Although your lung mets are relatively few and small, your primary tumor is fairly large and, in my personal opinion, if it is in a resectable area, it should be surgically removed as soon as possible without first undergoing a systemic treatment or radiation to try to shrink it. ASPS is notoriously radiation resistant, and subjecting you to a systemic treatment with Cediranib or another drug could weaken your immune system, cause you debilitating negative side effects, and may pose the risk of you developing resistance to the drug followed by aggressive disease progression. In my experience and based on my research, removal of the primary tumor with good margins often results in a period of stabilization of the disease, rather than triggering an explosion of the tumors in your lungs as you and your family fear. Once your primary tumor is removed, you could pursue treatment of your lung mets to shrink/destroy/remove them with ablation, laser resection, or surgery. Of course each patient is different and each person may respond differently to different treatments, so all that you can do is make the most informed decision possible based on your personal research, your doctor's recommendations, and the anecdotal experience data that is available on this Board. I know that you and your family must be very frightened and overwhelmed with everything right now dear Keola, and I know that you are facing some difficult decisions as to which is the best treatment approach, but as I said in my previous message, you are not alone on this journey and those of us on this Board are here for you. Stay strong, hold tight to Hope, and let me know if there are any questions that I can try to answer for you.
With more hugs, special caring thoughts, healing wishes, and continued Hope,
Bonni

Aloha again,
My doctors and team here in Hawaii are supposed to be the best cancer doctors here in the state, having helped hundreds of people. I know that this cancer is extremely rare however, and have been doing research myself as well. I will push my doctors to to a bone scan as well and as for my gender.. im actually a male. haha.. its no biggie, maybe i should put some manly looking faces on here.. joking aside, i would really like to remove the primary tumor but there are no sarcoma specialists here in Hawaii as far as i know about. I sometimes feel like i might be the first patient in Hawaii diagnosed with this disease, but im sure there have been some before me. I have researched and found some doctors close by in California, but am still uncertain of who would be the best and have the most experience. Would anyone recommend any doctors who have experience in removing ASPS tumors? Also I have also read and researched on this Dr. Rolle in Germany, but i feel like it would be a really expensive procedure. I currently have another CT scan scheduled for my chest/lungs again for the FDA and the NCI to see if the disease has progressed in some way. if it shows no progression, i will not be eligible for the Cediranib clinical trial at Bethesda. Because i cannot take part in the trial just yet, I am currently taking plant enzyme supplements(break down tumors protective walls), Cell Power(increase oxygenation in cells), fish oils(joint/bone), B12, Vitamin C/D tablets, and natural Hawaiian teas/juices to fight off and protect organs that havent been affected yet. Based off what ive noticed and seen, Chemo/Radiation treatments do nothing but sicken the person and suppress the immune system which is the biggest factor in fighting all types of cancer. I do not want to go to a doctor who doesnt know what their doing and give me some random Chemical pill that only weakens and makes me sick.

Dear Keola,
Deepest apologies for my mistake of your gender which reflects my lack of knowledge of Hawaiian names! Nonetheless, you sound like a very strong and physically fit young man, as well as being very pro-active in your search for knowledge and information about ASPS which is very admirable. If you are considering surgical removal of your primary tumor in California, Dr. Fritz Eilber and Dr. Shlomo Raz at UCLA are highly regarded and very experienced sarcoma surgeons. Also, if you would like to obtain a second opinion regarding recommendations for treating your disease, the Sarcoma Alliance will provide funding for your travel expenses. You can access information about second opinion funding on the sarcomaalliance.org website, and you can find information about Dr. Eiber and Dr. Raz by doing an internet search of their names.
I Hope that the supplements that you are taking are beneficial to you. However, there is mixed data and opinions regarding the effectiveness of vitamins and nutritional supplements in helping to fight cancer, and there is some data which indicates that vitamins and supplements may actually have an adverse affect by not only strengthening the immune system, but also the cancer cells which enables the cancer to progress more rapidly. There is information available on this controversial issue in the Diet and Lifestyle topic on this Board. Whatever your decision regarding taking vitamins and supplements, it is important that you tell your doctor about any vitamins and supplements that you are taking as some may negatively affect the effectiveness of some treatments such as Cediranib with which eating/drinking grapefruit and some other anti-oxidant foods are prohibited.
My very best wishes will be with you for good news from your scheduled chest CT scans which will Hopefully show no new mets or increased growth of your exisiting mets, and I will be anxiously awaiting your next update. Take care Keola.
With special caring thoughts, healing wishes, and continued Hope,
Bonni

Hello again Keola,
If you have not already found it, the following is the link to access information about Dr. Fritz Eiber who I had referred you to as a highly respected and experienced Sarcoma surgeon in California. I Hope that this information is helpful to you. I will be looking forward to your next update. The link to information about Dr. Eiber is:
http://www.cancer.ucla.edu/Index.aspx?page=383
Also, Dr. Shlomo Raz, whose name I erroneously gave you, is a urology surgeon, so he would not be an appropriate choice for resection of your thigh tumor, but had assisted Dr. Eiber in the difficult resection of the pelvic area tumor of a young ASPS patient previously on this Board.
With special caring thoughts, healing wishes, and continued Hope,
Bonni

Dear Bonnie,
thank you sooo much for helping me find a sarcoma specialist close by. I was nervous at first, but because ASPS is resistant to traditional chemo and radiation i hope to have this tumor in my thigh removed as soon as possible. Ill try and get in contact with Dr. Fritz Eiber and hopefully get some good results. My family is still really pushing me to do the Cediranib trial first before going into surgery. But like you said earlier, Cediranib can basically only help me for a little while (if it does at all). So im trying to explain to them how removing the big tumor first might help me a little, but their too scared and still believe that by taking out the "mama" tumor(primary), the "baby" tumors in my lungs will begin to spread rapidly. I too am a bit scared at the removal of my primary, but something in my mind tells me that i really need to remove it BEFORE starting any treatments. Because we do not have sarcoma specialists here in Hawaii, I have been trusting the expert team i have here and they have not been keeping me informed on their plans. Today i have been scheduled for another CT scan with contrast done on my chest/lungs to see if it had progressed. If it has, i will be participating in the Cediranib clinical trial at Bethesda. In my mind, i plan on taking the trial for a month, and if the cons weigh out the pros i will seek other treatment options. As a native Hawaiian resident, I am very scared of these chemicals and medicines that the world has created, but i do have faith in the compassion and understanding of the doctors that have dedicated their lives to helping cure ASPS. Please if anyone could give me some advice on this it would be greatly appreciated. I will keep the board updated on my results and whether or not I remove the tumor first or take the Trial.
Mahalo me ke aloha pauole,
Keola

Keola, I am Olga - Ivan's mom who is fighting ASPS since 2003. I would suggest you to get familiar with the clinical studies that were written about ASPS by the leading ASPS researchers, the links are located on the main page in the library area. The research strongly supports the resection of the primary tumor as the primary tumor when left in place keeps spreading the new mets elsewhere, but on the other hand I somehow understand the concern about the removal of the primary tumor disturbing the balance and the other mets starting to grow - it is not the "baby" tumors begin to spread rapidly but rather the dormant sarcoma cells that have already spread from the primary and are sitting elsewhere are starting to grow and became visible on the scans. There is a hint that the primary tumor sometimes acts as a suppressor of the distant mets growth but on the other hand it is always only temporary for a time they do not get enough of the blood supply, when their blood supply is fully develops they are going to start to grow anyways. The logic behind of the primary cancer removal is simple - this is the only chance for some of the patients to try to beat this cancer, the road starts from this risky but necessary point.
May be you want to get the second expert opinion on it - I believe that the local cancer doc that you are seeing now is able to obtain this second opinion for you based on a fact that it is a very rare cancer - at the specialized sarcoma centers, long distance - they send all the scans and the tumor board at the sarcoma center reviews it, the ones that see most of the patients with the rare sarcomas referred by their local docs are MDACC in Texas and MSK in NY.
I would not go for the cediranib in the situation like yours - when the primary is potentially resectable and there are only few mets in the lungs, because cediranib is the temporary solution when the resection of the primary plus ablation/resection of the lung mets might be your chance for a durable tumor control? The clinical trial's acceptance conditions of having the unresectable situation and progressing tumors are there for a reason - the trial is an unknown territory, it may help, may do nothing but may actually shorten survival and it should be only used when there are no other options.

Hello again Keola,
I agree with Olga's very knowledgeable and highly respected advice that Cediranib is probably not the best treatment approach for you at this time since you currently have other options including resection of your primary, and you have a relatively small number of CT visible lung mets which can most likely be successfully treated/destroyed/removed/ with Cryoablation, laser surgery, or surgical removal. As I have previously told you, Cediranib is unfortunately not a permanent cure, and as echoed by Olga, it should be reserved for aggressive or widely disseminated disease for which there are no other treatment options which was the case with our daughter Brittany. If your today's CT scans heartbreakingly show increased growth of your lung mets and you decide to enroll in the NIH Cediranib Trial and then as you said "plan on taking the trial for a month, and if the cons weigh out the pros seek other treatment options", based on previous ASPS patient's experiences, you could be faced with rebound and aggressive disease progression if/once you discontinue taking the Cediranib, and then devastatingly possibly have no other treatment options.
I know and understand that you and your family desperately want an immediately effective treatment and permanent cure for your ASPS, as all of us in the ASPS Community do, but sadly, to my knowledge, there are currently none available and all that you can do at this point is try to manage and control this very challenging disease until a permanent cure can be found. Published studies and the most currently available data that I have read continue to indicate that at this time resection or shrinkage/destruction of ASPS tumors at their smallest most treatable size with ablation or radiosurgery is the most effective way to try to control the disease.
Because ASPS is typically a slow growing disease, you should have time to seek a second opinion, and as I have told you, The Sarcoma Alliance has a program which provides financial help for expenses involved in traveling to seek a second opinion for Sarcoma if you decide to do that. It is extremely important to be as knowledgeable and well informed as possible before you make any treatment decisions, and since you and your family are really struggling with which is the best treatment approach, and there are no Sarcoma specialists in Hawaii, I would strongly encourage you to seek a second opinion from a Sarcoma Center on the mainland.
I Hope that all goes well with your scans today, and I will be anxiously awaiting your next update.
With special caring thoughts, healing wishes, and continued Hope,
Bonni

Aloha everybody,
Thank you Bonnie and Olga for all your help and detailed knowledge. I really do appreciate everything that your telling me and take it into account. I have always wanted to remove the primary first, but had no idea how or who. I had just had a CT scan done and sent to the NCI in MD and they called me today saying that my first CT was miscounted. I had 7 mets to begin with and now it has progressed to 10, which is funny because i was told from the beginning that i had 10 mets anyway. I am now eligible for the trial, but now my plan is to go there and be able to question these specialist about what they think is the best option and if they are able to refer me to the best specialists. I understand that they are really going to want me to take the medicine first, but I have a barrage of questions ready and I really expect some answers. Because i have had 3 new mets in 6 weeks, who knows how much more my primary will "feed" cancerous cells into my blood/body?.. I plan on taking this step by step and learning as I go, while looking at others and their treatment to figure out what would be best for me. As of now, I will fly up to DC to the NCI and spend a week there looking around and getting my questions answered BEFORE i take any medication. Like you all have said, there is a chance of an aggressive rebound so i will definitely take that into account. Thank you all so much for your help, love, and support. Me and my family here really appreciate it and i will keep the board updated on my decision within the next week.
Mahalo me ke aloha pauole,
Keola

Keola,
first of all you have to make sure that you really have had 3 new mets in 6 weeks. It is very easy to find out as regardless that you have no sarcoma specialist there, lung mets are very common for some very common cancers like breast cancer and others so any experienced radiologist can do a reading again for the first scan and for the second scan and to compare them side by side, you can request it based on a discrepancies between what you hear about the numbers and the decision you will have to make based on this situation (Ivan does it himself to ensure the most accurate reading as the mistakes are very common when they are read since the mets are very small). His first lung surgery was done in more than a year since his first lung mets were found.
They actually have some very good lung surgeon there at the NCI David S. Schrump
http://ccr.cancer.gov/staff/staff.asp?profileid=5765

as I remember that has done quite a few asps lung surgeries, he was willing to operate on Ivan 8 years ago when we were looking for the options, they also have the very good orthopedic surgeon for the primary. There are very big limitations as what can be done when you are already on the trial as these tumors are being measured so no surgery is usually allowed. I would advocate doing surgery for the primary before you go on a trial, but you can try to meet the surgeons there as ask them. The principal investigator is an oncologist and I would really try to meet the surgeons.

Dear Keola,
Thank you for your thoughtful update. I am so sorry for the results of your yesterday's chest CT scans which appear to show three new lung mets since your last chest CT was done showing seven mets six weeks ago. I am perplexed as to why you were orginally told that there were ten mets if there were really only seven then, and I, like Olga, feel that you need to confirm that there actually are three new mets. Radiologists and doctors can and do make mistakes! Knowing the correct number of mets and if there are actually three new lung mets in six weeks is critically important to your treatment decision. Did you look at and review the original scans and the new scans with your oncologist and count the number of mets with him? It is very important to always visually review the scans with the oncologist and to also request a copy of the radiologist report and a CD copy of the scans to have for your own records. Did yesterday's scans show any increase in the size of the mets in addition to three new ones?
Based on my personal experience, research, and knowledge, I continue to have concerns about you beginning Cediranib at this early point in your disease, but I know that you have to make the decision that you, your family, and your doctors feel will best meet your treatment needs and offer you the best chance to stabilize progression of your ASPS and shrink your tumors. Unfortunately, there are no definitive answers, and each case may be different. Something that it will be important to keep in mind in your decision making process is that if you do decide to go forward with the Cediranib Clinical Trial and it helps to shrink your primary tumor, at some point you will probably still need to have the primary tumor resected because it is unlikely that the Cediranib will be able to completely shrink a seven by ten centimeter tumor. The issue that you may then face is that in order to have the surgery to remove the tumor you will need to discontinue use of the Cediranib for about two weeks prior and two weeks following the surgery due to the increased risk of hemorrhage caused by the Cediranib. Discontinuing the Cediranib for the surgery may unfortunately then cause rebound and disease progression, which if you had the primary removed prior to beginning the Cediranib would not be a risk. Also, having a large tumor like your primary may interfere with the effectiveness of the Cediranib since a large tumor burden makes it harder for the drug to successfully fight the disease. In Brittany's situation, she had a small superficial abdominal met which was not responding to the Cediranib and was continuing to grow, so Dr. Sawyer suggested that she have it surgically removed because as he put it, "it was not being a team player." Once she had the small tumor removed which reduced her tumor burden, she thankfully had a much more significant response to the Cediranib with dramatic shrinkage and disappearance of her multiple and widely disseminated mets. Additionally, it is important that you know and understand that Cediranib has some very debilitating side effects which can greatly affect the quality of your currently very active Lifestyle. I have detailed the side effects that Brittany has experienced in her information in the Cediranib topic on the Board. If you do decide to participate in the NIH Trial you will need to commute to Bethesda intially once a month, and then every two months for Clinical Trial scans and clinic appointments. These are important issues and questions which you will need to address and discuss with the NIH Cediranib Clinical Trial team.
Whatever your decision, please know that my most positive thoughts and very best wishes are with you for a very successful outcome to whatever treatment you undergo, and that I am here to try to answer any questions that you may have.
Take care, travel safe, and keep in touch as you are able.
With special caring thoughts, healing wishes, and continued Hope,
Bonni

I wanted to comment on Dr.Sawyer suggesting to remove the soft tissue met that was not responding while on a trial - this is not the typical situation, they usually very much opposed to it and the only reason that it was suggested in Brittany case is that it was a Phase 1 trial, when the efficacy is not investigated, its goal is to find the maximum or optimal tolerated dose and to look for the hints as how to take the particular drug - before or after meal, how many times a day etc. and what are the side effects. In the Phase 2 and 3 clinical trials we have seen the cases when there was a progression in some mets or in the primary and the response elsewhere, the patient was taken off the trial. You have to understand clearly that a clinical trial is not the treatment that is expected to bring a benefit, it is still unknown and is the subject of their work so they can not keep the person on a trial based on their own perception that it might be of benefit - there are strict criteria as how the progression or regression is calculated and the surgery is usually not used the midways to fix the progression in some of the mets. I do not think that we know of a case when the primary tumor was responding to cediranib at least for awhile (as well as the soft tissue mets) - you can add this question on a list for them - what is the reason for you to keep the primary and not to resect it before going into the trial, do they have a knowledge of the cases to support this strategy?
If the primary tumor is painful, your point might be tat it affects your quality of life and reduces your mobility.