Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi everyone
I am Sandeep 36yrs, live in Southampton, UK. I was diagnosed with ASPS in Dec 2008. It was discovered after I had chest pain on flight to SF, California..... rushed to ER and to my shock I was told I had multiple lung mets... it was as if I was role playing in the TV drama ER !
Anyway, now I have right thigh primary tumour, multiple lung mets and single brain met (seems typical from reading all other members' experiences).
I am under the care of Prof Judson/ Dr Jones at Royal Marsden, London. Been on Sunitinib (Sutent) since January 09. On and off I have essentially been on 3 months of 25mgs daily. Sore feet and sore throat seem to my biggest problems.
Repeat scans in May showed the various lesions to be stable. However, I feel the lump in my thigh is softer and my chest pain has almost settled.
I am going to continue with Sutent 25 for the foreseeable future. Any advice/ experiences regarding surgery to the various lesions would be very much appreciated. I am waiting to hear from Dr Rolle in Germany regarding laser resectability of my lung lesions (thought to be too many to operate on).
Marsden is a great place as they have quite a few trials.
I am a practising haematologist myself looking after patients with blood cancers. Now to go through what I am going through has opened my eyes in several ways. I just hope to be useful to others whatever way I can.

Sandeep, hi, welcome to the board. Thank you for joining us although I am sure very sorry to hear that one more person is affected by ASPS.
I moved your post from the Sunitinib topic here so people could ask all the questions or comments regardless if it is about the Sunitinib, I hope you don't mind, as an admin I have to keep this all organized. I also ask you to open a new topic there in the antiangiogenic treatments with the "Sandeep experience with the Sunitinib" or something like that name and post all the information that you will be comfortable to share about your treatment as it seems that Sunitinib is getting increasingly used in ASPS and we have to figure out if it really beneficial.

Welcome Sandeep
I am sorry to hear you also have ASPS! But, you are in a great place to talk about this disease. I have gain a lot of help from posting here
I do not have much help to give per medical advice yet. I was diagnosed in Jan 09 But, i have a good ear if you ever just need to vent or talk about what you are going thru i am here or you can e mail me any time!

In healing hopes for us all!
Amanda R

Hi Sandeep,
I'm sorry to hear about your diagnosis. My husband who was also dx in Dec 08, is about your age and he too has multiple lung mets. You can follow his story in personal updates under "Sree's recent diagnosis".
Its so important to share our experiences because ASPS is rare, published cases could be some years old, newer medicines/procedures are tried on ASPS now and a forum like this has current information about what different patients are doing for ASPS around the world and how it has helped or not helped them. Welcome to the board and best wishes.

Arch

Thanks a million Olga, Amanda and Sree...
Olga you do a great job keeping this site so organised. This site is a source of great info...so keep it up !

Hi Sandeep,

How are you? How is your sutent treatment going ? Did you hear from Dr.Rolle regarding resectability of your lung mets ?
Since you are under the care of Prof Judson you might also have a chance to get on the cediranib trail which seems to have some effect on atleast some of the ASPS patients.

Take care and all the best.

Arch

Hi everyone

Greetings.
Its been a while since I have posted any comments. So apologies.
Just thought it will be useful to add my progress and provide information on how the experts in Marsden, London are managing my condition.
Sunitinib has not been very promising so far. Lots of side effects and my lung mets have grown by 20%. There is the ARQ197 trial but reading the reports this does not seem to be holding much promise either. Cediranib data seems to be encouraging so I will endeavour to start this.
In the meantime, surgery for the various tumours has been considered disadvantageous or not feasible. Prof Judson's experience is that if the primary is removed then the mets can get bigger because of 'angiogenesis switch'. Radiosurgery to my brain lesion is considered unncecessary because I'm asymptomatic from it.
I heard from a friend about Rudolfsteiner treatment (alternative medicine). He has failed all conventional treatments for his cancer and after a 2 week course in Rudolfsteiner Health centre in Ann Arbor his hopes have changed completely. His tumours have shrunk significantly. Anyone else with any experience ?
I will write again later. Thanks everyone for all the useful info you all provide on this site....its the single most useful information place for any ASPS sufferer.
Best wishes
Sandeep

Hi Sandeep,

I would have said hello before, but I must have missed your post. Cediranib would look pretty good as an option for you I would think because it seems to have been the best for brain metastases. As you may have read, a number of the members of this forum have also had success with gamma knife for the brain met.

Our daughter also found her primary tumor got soft on Sutent, and the primary did seem to stop growing over the 9 months she was on it, but only 5% was necrotic after it was removed. There is a possible danger with removal of the primary (rebound growth of mets), but the primary is also thought to release metastases. Perhaps if you could go on cediranib and induce shrinkage and stability, then you could be a candidate for surgical removal of the primary after. I also saw an impressive powerpoint presentation on sequential targeted agents (TKIs) and the data are fairly impressive that switching to different agents (it didn't seem to match which or even the order) improved outcome over the long run in general - but perhaps you know this too as you are an oncologist. I have also seen this from another group but don't have a link immediately handy to give you.

Also, I will add somewhere in the forum, but I just learned Pazopanib is FDA-approved (are you in the US?) and there is at least one ASPS case my onc heard of who responded to it.

If you could get Cediranib, though that could be best.

Best wishes, 'F'

Hi everyone,
Its been a long while since I last wrote. I have to confess I rarely visit this site, mainly because I fear any unpalatable news that I might come across. May be I should try and atleast contribute my personal experience from time to time. So apologies.

I was on Sutent for 14 months, with largely stable disease. But towards the end when my scans showed 20-25% progression in my lung lesions, I decided to enter the cediranib trial in NIH, Maryland which you all are familiar with. I will be there on 13 Sep, 9.30 AM OP 13. Would be delighted to meet with any of you.

Anyway, I have been on 30mgs of this poison for about 4 months now. I've had 2 scans so far (getting fed up of these scans- have had 15 in total !!). My first scan showed 20% improvement in my lung nodule. Before the next scan, I was asked to drop the dose to 20mgs because of side effects.

My 2nd CT scan showed the nodules had grown again and I had more lesions in my liver and spleen. However, the PET acan showed the lung nodules to be stable. My thigh lesion has also grown slightly on the CT. My brain lesions are the same. The liver and spleen lesions remain a mystery because they are not lighting up on the PET. MRI seems to be a reasonable way ahead to get to the bottom of this, as many of you suggest. It is important to be sure whether the liver/ spleen lesions are part of mets or they are simply benign changes...

My chest symptoms had returned when I was on 20mgs. I am now back on 30mgs and my symptoms are much better again. But no gain without pain- diarrhoea, hand/ foot problems etc. But reasonably manageable. The hardest thing is the travel every month- from London, UK to DC. I wish they funded some of the travel for international patients too. But anyway I must'nt complain. I am lucky that I got into this trial. Dr Kummar/ Yvonne/ Agnes everyone is so kind and helpful !!

Today, I did find strength in the fact that so many of you are staying strong fighting this dreadful nightmare!! Best wishes.

Hi San
I am so glad you posted i was wondering what happened!

Start a thread in the Ced area so many are there and they are sharing all this info
From what i am told there are about twenty other ASPS paitents that are also on this trial yet are not posting
I wish they would even if they post under the name Fred or Anonymouse!

Wow will the airlines help in any way if they have proof that this is for a medical reason?

Dear Sandeep,
I am so very sorry that I somehow missed your initial postings beginning in June 2009, and that I have thus never personally responded to you. I am usually very vigilant in checking this Discussion Board each day, so I don't know how I missed your entries, but we were probably immersed in all of the travel, time, and appointments required for Brittany's Edmonton, Alberta Cediranib Clinical Trial participation. I am so grateful that you found the emotional strength to face returning to our Board today to update your information. Your reason for being reluctant to visit the Board very often because of your fear of reading some discouraging or negative news, is one that I have heard expressed by several other patients also, and I very much understand that it is extremely important for you to try to maintain as positive an attitude and as much Hope as possible in fighting this insidious disease. However, I also know that it is the invaluable researched information and anecdotal treatment experiences of other ASPS patients which is shared on this Board which provides all of us with one of our greatest weapons for our challenging battles with this extremely rare and poorly understood cancer. Knowledge is power, and to fight this disease we all need to be as knowledgeable as possible. Additonally, our ASPS Community is a source of immense strengthening support and encouragement, and we all have a unique understanding of what you are going though and feeling so I Hope that you will continue to participate on the Board and to reach out for shared information and support.
I am so sorry that after fourteen months on your Sutent treatment you developed resistance to the medication and had disease progression. Unfortunately, developed resistance and rebound seems to be a well known risk with tyrosine kinase inhibitors (TKI) like Sutent and Cediranib, but there is some anecdotal evidence and data which has shown that changing from one TKI to another can cause the disease to stabilize again.
Hopefully this will happen now that you have switched from Sutent to Cediranib. I am very sorry that despite the intial shrinkage of your lung mets, your second Cediranib status scan showed increased growth in both the lung mets and your primary tumor in your thigh. Based on our nine years of ASPS experience and our extensive research, I am perplexed as to your doctors' decision to not resect your primary tumor in your thigh and to not resect or treat your brain mets. Our research and shared anecdotal experiences of other ASPS patients has indicated that if your primary tumor is resectable, it is best to remove it to prevent the primary from releasing new tumor cells into the blood stream, and to help reduce the body's tumor burden and therefore strengthen the immune system to better fight the cancer. Also, although I am very familiar with, and I highly respect Professor Judson and his work, I absolutely don't understand, and strongly disagree with his attitude regarding not having you undergo radiotherapy treatment for your brain mets based on the fact that they are currently asymptomatic. You DO NOT want to wait for them to become symptomatic, because once they are, they will probably be too large to successfully respond to radiosurgery like Gamma Knife since the best chance of a successful outcome for Gamma Knife treatment for ASPS brain mets is if the brain mets are under 4 mm. It is critically important to treat the brain mets at the smallest possible size. Granted, Cediranib can cross the blood barriar to Hopefully prevent new brain mets, but if you have existing brain mets while undergoing Cediranib treatment there is a risk of cerebral hemorrhage which can be fatal. Also, it was my understanding that having existing untreated brain mets was a preclusion to qualifying for participation in the Cediranib Clinical Trial based on the risk of cerebral hemorrhage, at least this was the case for Brittany's Cediranib Clinical Trial in Edmonton. I am not a doctor Sandeep, although sometimes I feel like one after all of our experience and research, but I would strongly encourage you to discuss the above issues and concerns about your primary tumor and the brain mets with your doctors. Regarding the new lesions in your liver and spleen, you may have read on the Cediranib thread that in a recent abdominal CT Brittany also had a suspected liver lesion show up adjacent to the portal vein. Thankfully, a subsequent specialized MRI showed that the suspected lesion was a BENIGN "focal area of fat deposition" (also called focal fat sparing) which is apparently somewhat common in the liver and especially with patients undergoing chemo treatment such as Cediranib. Hopefully this will prove to be the same situation for you, although I think that the specialized MRI should be done to make a definitive diagnosis. I have requested that Olga post your entry also on the Cediranib thread so that others who are following this treatment can access it there. In the meantime Sandeep, please take care, travel safe on your long transcontinental commute to DC for your Cediranib Trial, know how deeply appreciative I am of your having reached out to update your situation and to share your treatment information, know too that I am here to help in any way that I can with shared information and support, stay strong, and keep in touch as you are able.
With special caring thoughts, healing wishes, and continued Hope,
Bonni Hess, mother of 28 year old Brittany diagnosed at age 19 in July 2001

Thank you so much Amanda and Bonni for your kind comments!!

I will keep you all updated, no worries. I dont know what to say about your advice about surgery to the primary and radiotherapy to the brain lesion. Part of me wants to follow Prof Judson's advice, as its an easy thing to do. Not having and invasive procedure means I can continue to work without jeopardising my job...Despite such extensive spread, my quality of life is pretty good and I could potentially risk this if I had any procedural complication eg. rebound spread of mets as suggested by Prof Judson or impairment of my vision as a result of radiotherapy to the brain lesion. My brain lesions are 1cm, 2mm and in the visual cortex. I could not function in my job if my visual field was compromised.
The trouble with ASPS is that there is not enough evidence base. Any opinion including that of experts like Prof Judson's remains an opinion or an educated guess at best!!
I wish I could just make a straight decision like so many ASPS patients have made and not worry too much about the consequences.
You all do a great job keeping this site going!!
Sandeep

Dear Sandeep,
Thank you for your very prompt response. Your treatment decisions certainly need to be based on your own personal situation and needs, and I understand your reluctance to compromise your quality of Life with invasive procedures or to jeopordize your job, but I also know and understand too well the harsh realities of this insidious disease, and the nature of it to continue to progress and grow which will certainly ultimately very negatively impact not only your quality of Life, but the length of your Life, and consequently your job. Hopefully the Cediranib alone will prevent further disease progression, new tumors, and tumor growth, and will shrink your existing tumors, but from the results of your second scans this unfortunately does not seem to be the case thus far. I am concerned that the amount of tumor burden that you have with the primary tumor still in your body may make it difficult/impossible for the Cediranib to work as effectively as seems to have been the case with other ASPS patients undergoing Cediranib treatment with an existing primary tumor, and that your existing brain mets may put you a risk for cerebral hemorrhage, but as I have said, I am not a doctor and this is just my opinion based on nine years of ASPS experience and relentless research. I continue to encourage you to at least discuss these issues/concerns with both Dr. Kummar and Professor Judson, and to possibly seek a third opinion from a sarcoma specialist. As you have noted, treatment decisions are extremely difficult and with this extremely rare and poorly understood disease, no one, not even the doctors, know defintively what the right/best treatment option is, so the burden remains on patients and their families to make the best decision based on not only the medical advice of several different highly qualified sarcoma oncologists, but also researched data, anecdotal information, and individual situations and needs, so that the treatment decision is based on being as knowledgeable and open minded as possible. My best wishes for a very successful outcome to your treatment are with you Sandeep, and I will continue to hold you very close in my heart and my special thoughts. Please take care and keep in touch as you are able.
With special caring thoughts, healing wishes, and continued Hope,
Bonni
With

HI Sandhar
I hope that you are feeling well and that you have come to a decision or at least come close to making one in reference to the tumor.
My brain is so far clear and i compleetly agree about the brain that would worrie me also are they stable? Bonnie knows alot about the brain things i dont so what i am talking about isnt the brain...
I understand about your promary and it was also hard for me to make decisions in my treatment and i went with my heart thats is really all we have after we read and talk to everyone.
You said "straight decision" I will be honest and i am sure others have also felt this way.. OMG am i doing the right thing ? this was as i was being wheeled to the OR..I swear before my rib SX they were wheeling me and i know he saw the look on my face and i was out i didnt have a hope of escaping, lol I have a tricky doctor.. For myself i did what i was told and I am glad i did. Though i have some problems from the surgerys it is all worth it! We are all different and for me it was quantity over quality I am responcible for a lil boy so it wasnt about me it was about him he comes first thats what pushed me and drove my heart.
Ok, i would have also done it for my cats sake also lol
It is a heavy thing to deal with and if you want to talk it thru here i know that everyone would be there for you .. you are not alone remember that!

I hope you post back soon ...

Sandeep had his scans today.There has been increase in the liver and spleen mets.We are very disappointed as he has been taken off cedaranib trial as it was our only hope.He has already been on sutent in the past which did not work.It does not look like there is anything we can try.If anyone else is aware of any new trials or medications that has helped ,please let me know.I would appreciate your suggestions in this matter