Cure Alveolar Soft Part Sarcoma International (iCureASPS)

My name is Alexander and I am 26. I was diagnosed with ASPS in February 2014. Unfortunately, by the time it was diagnosed it had spread into various parts of body including soft part of right thigh, left scapula, left and right flank-bones, lungs (multiple mets), peкitoneum (multiple mets), vertebra Th4. I have taken Votrient (Pazopanib, 800 mg every day) and Zometa (Zoledronic acid, 4 mg once in 28 days) since February 2014. Most tumors are stable but the latest CT scan in July showed 1-2 mm increase in some of the lung metastases. I am very anxious about this.

In spring we consulted many top doctors in Russia to find out if surgical treatment is possible. All the doctors we spoke with took the attitude that if disease is widely spread it is useless to surgically remove the tumors.
In particular, we consulted CyberKnife doctors in Moscow regarding my vertebra Th4 met. They all told us that they could help us in case I had pain. In their opinion, there is a high risk of using CyberKnife now when I do not have any discomfort or pain. They are afraid to make the situation worse.

On the contrary, many highly respected and experienced participants of this forum support the idea that the best treatment option is surgery or radiosurgery.

So my problem is to find out the doctor in any country who will agree to do the resection/ablation/radiosurgery. If you know somebody who will probably do it please let me know.
I also wonder if you know the links to published medical studies which support surgical option in treatment ASPS.

Sincerely,
Alexander

Alexander, welcome to the forum.
I was the one of the members that you contacted before already, I recommended the surgery when possible and the radiosurgery for the T4 before it gets symptomatic. I have to say that when we communicated before, there was no known peritoneal spread and I was not aware of many other mets you had - I am not sure if they were Dx later or if it was simply not discussed at the moment. Looking at the situation as it is presented now, it is going to be very hard to find a surgeon that would support the aggressive surgery approach. The interventions in the late stage are usually only done for the pain control or where it is obvious that some metastases might limit your survival and quality of life earlier than other ones, like it might be the case with the T4 spinal met - if wait until it becomes symptomatic, it might be less treatable by the radiosurgery - versus being easy treatable when it is small. Soft tissue and visceral metastases usually have more space to grow without pain/functional damage versus bone metastases.
But if the spine metastasis is completely stable on Zometa, radiosurgery might not have a sense for now. As I understand, only lung mets grew, right? Radiation-induced spinal cord injury is a complication that might happen after the SRS for the spinal mets so the risk should to be only taken if there is a documented progression.
About the articles supporting aggressive surgical or ablative treatment in the metastatic setting:
There is an article publ. by Dr.Kayton from MSK in NY
we have it in the library http://www.cureasps.org/bibliography/
under this link
Case studies of Alveolar Soft Part Sarcoma in MSKCC leads to the conclusion that practicing appropriate diagnostic techniques, aggressive surgical control of the primary tumor and long-term surveillance for metastases may result in long term survival (Kayton et al. 2006)
They conclude that long-term survival is possible even with metastatic disease. Resection of metastases may play a role.

There is some article that seems to advocate the early SRS treatment of spinal mets:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3291699/

but again, in the event there are so many mets everywhere, systemic approach would be the more reasonable. You need to have some other systemic treatment options that could be used if resistance to Pazopanib develops, like Sunitinib or
a clinical trial for Cediranib versus sunitinib
http://www.clinicaltrials.gov/ct2/show/ ... pen&rank=4
or cabozantinib
http://www.clinicaltrials.gov/ct2/show/ ... ib&rank=49
I realize that travel to US for the clinical trial is very expensive and might be difficult if the disease progresses, my hope is that cediranib will be approved soon for ASPS to became avail. locally by the prescr. Cabozantinib is avail. by the prescr. but not for ASPS for now, but dr can prescr it on "off label" basis if there is an information about the cases when it was beneficial for ASPS patient. We have few ASPS patient on it now, to obtain the latest info you can try to contact them directly by clicking on their name and sending them a PM.

Dear Alexander,
Welcome to the CureASPS Discussion Board. As per our previous communications in which I encouraged you to post on this Board, I am very grateful that you have now reached out to share the information about your ASPS journey and to seek information about treatment resources. Olga has provided some excellent insights and advice regarding your current situation with your innumerous and widely disseminated mets, but I continue to personally feel that reducing tumor burden through resection, ablation, or radiosurgery of as many mets as possible enables a systemic treatment to be more effective as proved to be the case when Brittany had an unresponsive superficial abdominal met surgically removed a few months after she began her Cediranib treatment. Of course, as Olga has indicated, the problem remains with finding a doctor/surgeon who is willing to surgically remove/ablate/or treat with radiosurgery those mets which are resectable/ablatable since you have such widespread disease, and I am not personally aware of the names of any Russian/European doctors/surgeons. There is an excellent Sarcoma surgeon in the United States at UCLA in California whose name is Dr. Fritz Eiber who has successfully performed some difficult surgeries for a couple of our ASPS Community patients, and I encourage you to write to him to explain your situation and seek his opinion and advice. His contact information is available when you do an internet search for him, but if you can not locate it I will be glad to send you the link. I agree with Olga that whether or not you have resection/ablation/radiosurgery of some of your most concerning or symptomatic mets, you definitely also need a systemic treatment to try to stabilize the progression of your disease and shrink/destroy your many mets. It appears that thus far the Pazopanib has thankfully prevented the growth of most of your mets with only a slight growth in some of your lung mets, but in the event of developed resistance to the Pazopanib and disease progression it will be very important to have alternative systemic treatment options to move forward with as quickly as possible such as Cediranib or Cabozantanib (Cometriq) so I encourage you to explore and pursue those treatments now through the informational links that Olga has provided. In the meantime, know that you are not alone in your courageous battle with this very challenging disease dear Alexander and that those of us on this Board are here to try to provide researched and anecdotal treatment information as well as strengthening support and encouragement on this journey which we all share. Take care and please keep in touch with the Board as your time and the situation allow.
With special caring thoughts, healing wishes, warm friendship, and continued Hope,
Bonni

Bonni - to clarify some of your comments for Alexander. When Brittany's mets were few and came with the interval in between, they were treated one by one, but when she developed disseminated mets in her peritoneal space- small bowel intussuception, duodenum, and pancreas, the only treatment available for her was a clinical trial with cediranib, i.e. a systemic treatment, and only after she obtained a response in all her mets except one subcutaneous soft tissue met that progressed while under the cediranib treatment, she had a surgery to remove that non-responsive met. If all the mets stayed stable - I am sure the advice would be the same as Alexander is getting now, to keep the traumatic interventions minimal as the healing process after any surgery elevates a VEGF and could be a growth promoter in the other mets. As for Dr. Fritz Eiber - he is an excellent abdominal sarcoma surgeon but the surgery cost starts from $120,000 as we were told by his assistant. The surgeons in Germany are much more reasonable and some of them are also very experienced, especially in the former East Germany lands.

Hi Olga and thank you for your additional comments which I concur with as long as Alexander's mets and disease remain relatively stable and do not become symptomatic or cause debilitating pain. However, he previously told me that he is unable to use his arm because of his arm tumor which would therefore seem to support the need for some type of surgery or treatment to remove the tumor or shrink it. Hopefully the Pazopanib will be able to shrink/destroy the tumors despite Alexander's large and widely disseminated tumor load which thankfully did occur for Brittany with her Cediranib treatment.
I understand that surgery with Dr. Eiber would unfortunately probably be cost prohibitive, but i thought that he might be able to offer his expertise and advice as well as some possible referrals for well qualified Russian and European Sarcoma surgeons.
Alexander, can you clarify for us if you have had your primary tumor in your right thigh resected as I can't remember what you told me in your initial e-mail? Based on my ASPS knowledge, research, observations, and experience, removal of the primary tumor is critically important in order to prevent the continued dissemination of tumor cells into the bloodstream from the vascular primary tumor, and also to reduce the body's tumor burden which helps to strengthen the immune system and disease fighting ability.
You are in my continued most caring thoughts and I will be anxiously awaiting your next update regarding your treatment decision.
With deepest caring, special thoughts, healing wishes, and continued Hope,
Bonni

Dear Olga and Bonni,
thank you very much for excellent insights, links and advice regarding my current situation. I will definitely use the information you provided to investigate further.

I did not want to go into details in my first comment so I did not describe how and when all my mets were found.
When I was diagnosed with ASPS in February 2014, only my left scapula, lungs and vertebra Th4 mets were found since they are visible on a CT scan. The treatment with Votrient and Zometa started. In spring I read this forum and followed the advice to do brain MRI, full body bone scan, abdomial CT and hip joint MRI. They all were made in April-May 2014. So some mets were found after the treatment had started. But there is good reason to think that they had been present earlier. Among them is a big tumor in soft part of right thigh. My doctor believes that this one is the primary tumor although it makes me much less discomfort than another tumor in left scapula.
I cannot lift my left arm but I use my left elbow joint normally so I can for example type on a keyboard or even eat something with my left hand. So no one of my tumors was removed and I do not know for sure which of them is primary.

Dear Alexander,
Thank you for the helpful detailed additional information. I agree with your doctor that the "big tumor in the soft part of your right thigh" is most likely the primary tumor since the primary ASPS tumor is usually located in the extremities and very often it is in the right or left thigh although it is sometimes located in the arm and even other parts of the body such as 2 cases that I know of where it was located in the gluteus maximus ( buttock).
I don't understand why more complete scans weren't done at the time of your diagnosis in February and prior to your beginning systemic treatment to try to determine if the scapula tumor was the primary tumor, or if there was a primary located elsewhere in your body, and if so, if it was resectable...
How large is the thigh tumor and is it visible as a lump on your leg or is it more deeply located? If the thigh tumor is the primary I would personally pursue resection of it since my understanding/experience is that the primary tumor is usually not encapsulated unlike the metastatic tumors which usually are encapsulated, and the unencapsulated primary tumor can therefore continue to feed tumor cells into the bloodstream. We always refer to Brittany's primary tumor as "the mother load' since it was from that tumor that the metastatic tumors originated and were disseminated into her bloodstream and throughout her body. Based on your widely disseminated mets the tumor cells are obviously already in the bloodstream and some doctors feel that "the cat is already out of the bag" and it is too late to prevent tumor spread by resecting the primary tumor, but our feeling is that as long as the primary remains in the body it continues to disseminate tumor cells and it should be removed if surgically possible. There is mixed data and opinions on this matter, but every patient who I have personally followed who has not had the primary tumor resected has unfortunately had limited/unsuccessful responses to systemic treatments with continued disease progression. I of course know and understand too well from Brittany's and many other patients' experiences that removal of the primary does unfortunately not prevent disease progression once the metastatic tumor cells are already in the blood stream, but it just makes sense that removing the primary will at least prevent a continued dissemination of tumor cells and reduce the body's tumor burden. Perhaps Olga and other Board members can offer their opinions and further information regarding this issue.
In the meantime, I Hope that you are able to utilize and pursue the links that Olga provided to explore possible other systemic treatment options in the event that the Pazopanib is unsuccessful in stabilizing the growth of your mets. My very best wishes are with you for good news results on your next scans which will Hopefully show stable disease and shrinkage/disappearance of your mets, and I will be anxiously awaiting your update.
With special caring thoughts, healing wishes, and continued Hope,
Bonni

Bonni Hess wrote:Dear Alexander,
Thank you for the helpful detailed additional information. I agree with your doctor that the "big tumor in the soft part of your right thigh" is most likely the primary tumor since the primary ASPS tumor is usually located in the extremities and very often it is in the right or left thigh although it is sometimes located in the arm and even other parts of the body such as 2 cases that I know of where it was located in the gluteus maximus ( buttock).
I don't understand why more complete scans weren't done at the time of your diagnosis in February and prior to your beginning systemic treatment to try to determine if the scapula tumor was the primary tumor, or if there was a primary located elsewhere in your body, and if so, if it was resectable...
How large is the thigh tumor and is it visible as a lump on your leg or is it more deeply located? If the thigh tumor is the primary I would personally pursue resection of it since my understanding/experience is that the primary tumor is usually not encapsulated unlike the metastatic tumors which usually are encapsulated, and the unencapsulated primary tumor can therefore continue to feed tumor cells into the bloodstream. We always refer to Brittany's primary tumor as "the mother load' since it was from that tumor that the metastatic tumors originated and were disseminated into her bloodstream and throughout her body. Based on your widely disseminated mets the tumor cells are obviously already in the bloodstream and some doctors feel that "the cat is already out of the bag" and it is too late to prevent tumor spread by resecting the primary tumor, but our feeling is that as long as the primary remains in the body it continues to disseminate tumor cells and it should be removed if surgically possible. There is mixed data and opinions on this matter, but every patient who I have personally followed who has not had the primary tumor resected has unfortunately had limited/unsuccessful responses to systemic treatments with continued disease progression. I of course know and understand too well from Brittany's and many other patients' experiences that removal of the primary does unfortunately not prevent disease progression once the metastatic tumor cells are already in the blood stream, but it just makes sense that removing the primary will at least prevent a continued dissemination of tumor cells and reduce the body's tumor burden. Perhaps Olga and other Board members can offer their opinions and further information regarding this issue.
In the meantime, I Hope that you are able to utilize and pursue the links that Olga provided to explore possible other systemic treatment options in the event that the Pazopanib is unsuccessful in stabilizing the growth of your mets. My very best wishes are with you for good news results on your next scans which will Hopefully show stable disease and shrinkage/disappearance of your mets, and I will be anxiously awaiting your update.
With special caring thoughts, healing wishes, and continued Hope,
Bonni

Hello Alexander

My name is Debbie and our son's name is Joshua. He was DX in 2012 and is currently 34 years old.
I am so sorry that you had to be part of our group as I am sure there are other things you'd like to be doing than discussing cancer
I suspect your scans were under the direction of a doctor who was just as confussed as the average doctor who sees this sarcoma, and who also had limited information to alveolar soft part sarcoma research.
Is your doctor a sarcoma /oncologists?

I agree with Bonni on persueing the removal of the thigh tumor and then addressing the scapula tumor. However I would be interested to the size of your arm tumor as well as Bonni's question on the size of the thigh tumor if you have access to those dimensions. They should be on your scan reports?

I know everyone has given you lots to think about however know we are here to listen as well
Sincerely
Debbie
Ps Joshua"s primary was in his left lower thigh and at 6cm felt like " a pulled muscle "

I have to say that there are many cases when sarcoma patients were not staged properly from the beginning, in Russia or elsewhere, not all the scans are done right away and only after people see what should be done and discuss it with their oncologists, they get done. The treatment would still most probably be the same and it is the same like our people get here now when diagnosed in the wide metastatic state - Pazopanib or Sunitinib or a clinical trial. The only really different thing re. overall management would be a treatment for the arm tumor - if you can not lift your arm then it affects a functionality and a quality of life, so it would have been treated here (I believe it would). Surgery or at least a radiation therapy. Is it in the bone or a soft tissue and what its extend?

Dear Debbie, Bonni, and Olga,
thank you very much for the helpful opinions.
I am sorry for the delay in reply, I am having CT and MRI scans this and next week. After I get latest results I am going to write to you details regarding my tumors and mets.

I agree with Olga on how it often happens in practice when patients are diagnosed with ASPS.

Olga wrote: I have to say that there are many cases when sarcoma patients were not staged properly from the beginning, in Russia or elsewhere, not all the scans are done right away and only after people see what should be done and discuss it with their oncologists, they get done. The treatment would still most probably be the same and it is the same like our people get here now when diagnosed in the wide metastatic state - Pazopanib or Sunitinib or a clinical trial.

Dear Alexander,
I too agree with Olga about ASPS unfortunately (too) often being inproperly/inadequately assessed and scanned following the initial diagnosis because of the extreme rarity of the disease and the limited knowledge many oncologists have about it. I still feel that diagnosis and resection of the primary should have been explored following your diagnosis despite the already widespread dissemination of your disease, but also know that many oncologists inexplicably do not share this opinion and practice once the disease has metastasized. However, in Brittany's case, despite the presence of innumerous (over 50) mets in her lungs at the time of her ASPS diagnosis, the doctors immediately moved forward with resecting her primary tumor in her left thigh. Thankfully following the resection of her primary her disease remained relatively stable for a couple of years which we think may be attributable to the reduction of the tumor load and the discontinuation of dissemination of new tumor cells into the bloodstream from her unencapsulated primary tumor.
My very best wishes and most positive thoughts will be with you during your upcoming scans, and I will be anxiously awaiting your update about the results and holding very tight to Hope for good news of stable disease with no new tumors and shrinkage of your primary tumor and numerous mets.
With deepest caring, healing wishes, warm friendship, and continued Hope,
Bonni

Alex, I just wanted you to see this post I made re. some very relevant article from Russia publ. on the Pubmed
http://www.cureasps.org/forum/viewtopic.php?f=3&t=961

Dear All,
I am sorry for being absent for so long because I wanted to spend some time without thinking about my disease.
I have rather good news. My thigh, scapula, vertebra Th4 and lung tumors and mets are stable with some insignificant improvement. I continue taking Votrient and Zometa.
Another good news is that I do not have peritoneum mets. It was a mistake of local polyclinic which reported about my mets in May 2014. The disc was revised in oncology center and it was found that the multiple mets are in the lower parts of lungs, not peritoneum. We made the revision of the disc by accident so the news was fully unexpectded. Abdomial ultrasonic scan in October confirmed absense of mets and ascites.

Some of you asked about the sizes. The current sizes of tumors: soft part of right thigh (primary) - 68x36x46 mm, left scapula - 63x37x56, right flank-bone - 40x27x38 mm, dozens of metastases in lungs - 2-14 mm each, Th4 - 25x15 mm. My tumors are encapsulated, they are not visible as a lump.

I also know that Cediranib trial lead by Prof. Judson is currently open (locations in the UK, Spain and Australia). But the patients who do not have medical insurance in these countries should pay for scans, clinic visits, and blood tests. So it is financially very challenging to participate in the trial for patients from other countries.

I am also investigating the possibility of surgery. I thank all of you for excellent insights and and links to useful materials. If you have further information to share I encourage you to do it.

Olga, thank you very much for your latest comment about the article from Russia. I will investigate this option.

Hi Alex, this is a great news - that there is no progression and that the abdominal mets were a mistake. This is why I advocate asking for a second review of the scans if the findings are unclear! However an ultrasound scan is not that good to rely on to rule out mets, I would try to get an abdominal MRI or at least an abdominal CT scan with the contrast.
Some comments:
- since you are def. a responder to Pazopanib, you most probably is going to be a responder to Sunitinib if the resistance to a first drug develops.
- The bone mets are large, they probably are very painful and you should keep trying to get a radiosurery to treat them. Scapula is thinner than the size you quote, what do they say - is it stable enough? It can break down.
- a cediranib clinical trial in US is free for the patients and would accept you but you would still have to pay fr the flights/living expenses. I expect that the drug might be approved soon and be avail after that locally.
- do not feel that we expect any of our questions answered. This is a low pressure board, we completely understand that there are times when you could be not in a best mood/health/nothing to say/not sure what to say/waiting to find our what to say/does not want to talk about this damn stuff etc. But do not hesitate to post as often as you want - when you want or need it.

Dear Olga,
I really appreciate your help in finding the best possible diagnostics and treatment.

I asked a month ago if people were being hired for Cediranib trial in the US and Canada. I was answered that there were no trials available for me: "Our study is closed to accrual for pts older than 16 years of age. Our other cediranib study is for patients who have not received prior pazopanib".

My tumors are large though they are encapsulated and not visible. So sometimes I could not believe they are as large as it is written in MRI and CT reports
Regarding my left scapula tumor my medical English is not good enough to describe all details. I will send you MRI report in Russian. If you find it informative I kindly ask to share this info here so that other participants understand the details. As I wrote earlier, I cannot lift my left arm but I got used to it.
Regarding my flank-bone tumor it makes me almost no discomfort right now. And it has greatly improved since spring. Probably it has happened thanks to yoga classes I have attended since April. My discomfort with scapula tumor has also declined since then. I should stress that the sizes of tumors have not changed since then.
I will try to ask my doctors about scapula break down. According to my experience they do not want to change anything if there is no significant deteriorating.

Did I understand you right that abdomial MRI is better than CT with contrast for ruling out mets?