Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello, all. My name is Brian and I am a 36 year old husband and father of three from Virginia (just outside Washington, DC). I was diagnosed with ASPS in June 2012. I have never posted anything on an internet board before, so please forgive me if this is not in the right format, or if the details are a bit off. Anyway, here’s my story:

In April 2012, I started feeling considerable pain in my hip/back. After a trip to my general practitioner, several visits to spine doctors and a month of physical therapy proved to no avail, I was sent for an MRI. When I went in for the MRI, I thought I would be dealing with a torn labrum or muscle. Thus, the call from the radiologist to meet with a surgical oncologist the next day was quite a shock. The MRI had revealed two large “growths” -- one in my gluteus medius (roughly 12 cm), and one in the same muscle but attaching to the pelvic bone (roughly 7 cm). After two biopsies, the tumors were diagnosed as ASPS.

I met with a local orthopedic oncologist who suggested radiation and then resection. However, I was encouraged by a surgical oncologist friend to seek a second opinion at MD Anderson in Houston, TX, given the rare nature of ASPS. MD Anderson suggested doing Sutent prior to radiation and surgery. They believed that given the size and location of my tumors, trying to shrink the tumors through pre-surgery Sutent, would improve my surgical odds. I was on Sutent for 5 months, with MRIs and CT scans scheduled every two months. While on Sutent (37.5 mg taken daily) I experienced some side-effects (diarrhea, weariness and heartburn), but was more than willing to live with these discomforts, if the medicine was working. Unfortunately, while there was some initial shrinkage and optimism, my scan at four months revealed a “new spot” closer to my hip joint and little to no shrinkage of the primary tumors. Radiation and surgery were then prescribed.

I am currently undergoing radiation treatments on my hip. And, I am supposed to have surgery for the two primary tumors in the March timeframe. As of right now, the plan is not to surgically remove the “new spot,” given its small size (13 mm) and the difficulty in removing it while preserving mobility. Rather, the doctor wants to take a wait and see approach to this tumor.

As far as my lungs: my baseline scan revealed no lung mets; my two month scan revealed a number of indeterminate “dots” on my lungs (biggest was 5 mm, and most were 1 to 2 mm); and, my four month scan revealed no growth of the previously found dots. Further, at the four month scan the radiologist stated that it was not clear that the dots found at the two month scan were actually new, given the poor image clarity of my first scan. As such, the medical oncologist does not believe these dots are mets; he believes them to probably be pre-existing junk in my lungs (that is not the official medical term he used, but it is more or less what he said) and not related to the cancer. Reading this website gives me reason to question that diagnosis, but, as for now, I am choosing to believe his expert opinion. Regardless, the lungs are being monitored regularly.

I am currently considering getting second opinions on surgery and treatment options. And, that is why I started reading this website. Thank you for posting. Thank you for sharing your treatment plans. Thank you for talking about what you have been through. I can’t imagine it is very fun to post about your cancer, or that of your loved ones. But, it helps. It sucks to be part of this community, but I am thankful that it exists.

All the best,
Brian

Brian, welcome to this forum. I completely agree that is totally sucks to be a member of this community but I am very proud that you appreciate our efforts of trying to be united against this damn ASPS to increase the odds of everyone of us to deal with it in a most efficient way.
I will later move your excellent introductory post to a Personal Updates so you'd continue post what ever comes next.
As of now, I am not at all surprised to hear that Sutent was not effective for the primary tumor, we have seen that already. I am not sure on the radiation treatment plan before of the resection? May be they want the walls of capsule became more dense to increase the chances of the clean surgery but on the other hand it usually stays the same size and the radiation may cause the damages to that area so I would go with the simple good quality surgery for now.
Regarding the suspicious lung nodules. For your own benefit it is better if they stay undetermined until the primary tumor resected and that additional smaller one is ablated - may be it can be cryo or RFA ablated or radiosurgery zipped, you need to consult the respective specialists. Admitting that these nodules are mets and therefore the sarcoma is in its metastatic state may swing their mood from the aggressive surgery approach to a "there is nothing we can do look into the clinical trial etc" which is may be true for other sarcomas but not for ASPS which is usually provides some window of opportunity to deal with the primary, mets etc step by step.

Dear Brian,
I am so sorry for your ASPS diagnosis, but I am grateful that you found your way to this Board. I apologize for being so slow to respond but I somenow missed your post as I was away from the computer for a week with the birth of our new grandson.
I, as Olga did, question why radiation is being done prior to resection of your two large gluteus tumors since ASPS tumors are unfortunately notoriously resistant to radiation, but I Hope that your radiation treatment will be successful and provide some shrinkage. Where are you receiving your primary oncology care, and are your oncologist and orthopedic surgeon sarcoma specialists? If you do not have an oncologist and surgeon who are sarcoma specialists, I would strongly encourage you to find ones. If you are interested, I can provide you with the name and information for an excellent, highly regarded, and very experienced sarcoma surgeon in California. Regarding the new difficult to resect 13 mm. tumor near your hip joint, I agree with Olga that it might be advisable to explore Cryoablation or Radiofrequency Ablation (RFA) to try to destroy the tumor. I personally disagree with your oncologist's "wait and see approach" to this tumor since given the nature of ASPS, and without some type of intervention, it will unfortunately most likey continue to grow, and if it gets too large, it cannot be successfully ablated.
Regarding the suspected lung mets, they could possibly be scar tissue from previous lung infections or some other benign lesions, but again unfortunately, given the nature of this disease to metastasize to the lungs, they may very likely be ASPS mets which will need to be monitored with regular CT scans every 3-6 months. Because the "indeterminate dots" on your lungs are relatively small and didn't appear to increase in size between your two and four month scans, I think that your greatest and most urgent priority at this time is immediate resection of the two large gluteal tumors, and ablation of the smaller hip tumor if it is possible. Also, if it has not already been done, you need to have a brain MRI and full body bone scan. Vigilant monitoring is critically important in fighting this disease in order to find and treat mets at their smallest possible size.
I know that you and your family must be overwhelmed with everything right now dear Brian, and I know too how very difficult and confusing the treatment decisions are, but please know that you are not alone in your battle, and that those of us on this Board are here to try to help you with shared information and strengthening support and encouragement. Take care, stay strong, let Hope lead you through each day, and keep in touch with this Board as your time and the situation allow.
Reaching out to share special caring thoughts, healing wishes, and continued Hope,
Bonni Hess, mother of 30 year old Brittany diagnosed at age 19 in July 2001

Welcome to the forum, Brian!

Those spots in your lungs are probably mets, sorry to say. I concur with Bonni that it's not something you have to worry about right now, but just keep a close eye on them.

First order of business is, of course, getting rid of that primary. Definitely get a few opinions on that surgery.

Brian:
I am no doctor, but I was diagnosed with ASPS last Sept. and have read what I can understand of a lot of medical papers available online about it, as well as soliciting for info here on the forums. I know there is an old paper published by Memorial Sloan Kettering I have read stating that prognosis is best if the primary tumor can be removed while still small; the size they settled on statistically was <5cm. Given, this paper was very old and the data was gathered over the course of 50+ years, but I would still recommend getting those things out asap. I'm not really sure if you are seeking advice for other surgeons, but the gentleman who operated on my thigh did an incredible job. He is a sarcoma surgeon specifically, so he sees a lot. His name is Dr. Howard Rosenthal and he operates out of Kansas City, KS. The doctors who I have seen since then always marvel at how straight of an incisional scar he left me. One of the docs here in Germany asked if he used a ruler lol. But it may be worth seeing if he thinks he can safely remove those tumors. He and his staff are very kind and caring and really helped soften the blow when I was first diagnosed.
Good luck, and ask lots of questions.
Josh

Hello Brian,
I am sorry you have ASPS... I suggest you get that main tumor out ASAP! You dont have time to fool around with the "lets try this" stuff!" the Oncs do unless there is a REAL reason... I in the first 8 months went thru hell of two surgeries but i have been blessed so far and now at almost 5 years i am going to try and remove the few things in my lungs now! Please, take that thing out its like a mother ship sending out little fighters

Also, a few of the things in my lungs were not ASPS but others were... We had all hoped they were just what was found in my first lung surgery inflammation things. My point is that sadly i am almost positive that some are ASPS that you have and will need to be removed at one point an the main tumor is sending out mets to your body and lungs...

I see a Dr here in Los Angeles an his name is Earl Brian an he did the surgery on my leg... this is his specialty an if you want an opinion he is wonderful. A few have gome to him and loved him!

There were times i wanted to do the "lets try stuff" then i listened here and i got it out and i am glad i did!
It was so overwhelming at times i wanted to hid and not act!

Tell them to take that out so you can heal an get the things out of your lungs...

Hello Brian

My name is Debbie and I am Joshuas mom. I am curious how things are going for you and yours? I don't know if you know this but Josh is also a dad of a 4 1/2 year old daughter. Next to his wife ,she is the light of his life. His primary tumor was removed in September of last year 2012 just 3 months after your DX. His scans revealed tumors in his lungs..both of them. As I understand in the past doctors could remove a lung to perserve maligniant margins if and only if the cancer was in one lung. abviously not the case with Josh. So he has been to Germany to have one of his lungs alleviated of 77 tumors. His next course of treatment has yet to be realized as his latest treatment was just alittle over 4 weeks ago. We, as you, have been dealt a bad hand in life. All of us on this board look forward to reaching out to each other and helping when we can, listening when we can and crying when we can. Please be assured that we are here when you need us.

With sincerity and good thoughts

Debbie

Thank you for your kind words and advice; they have been both helpful and encouraging.

I am sorry not to have responded sooner; the last month has been a whirlwind of radiation sessions and meetings with orthopedic oncologist all over the eastern U.S. I visited with a number of surgeons and received a variety of opinions, but finally chose Dr. John Healey of Memorial Sloan Kettering Cancer Center (MSK) in NYC to perform the surgery, as he seems to have both an aggressive and learned approach.

I am scheduled to have surgery on March 12th to remove the primary and locally metastasized cites in my hip/butt. The surgery will remove two muscles from my hip/butt region, a large portion of my pelvic bone and require a total hip replacement. I will walk with a limp, need at least a month (probably more) off work and face a long rehab.... but that all sounds pretty acceptable in light of the fact that it may stop the cancer.

Anyway, I will likely go silent again for a while. I would appreciate prayers for the surgery.

Brian,
thank you for sharing your progress in planning for the next step.
I want to compliment you on your proactive approach and considerable efforts you put in to investigate a variety of opinions re. surgery. Your choose one of the places that sees most of the sarcoma patients in US and they have some of the best skilled surgeons. It is a very significant information for our board that the surgeon from the leading sarcoma expert centers considers the surgery to remove the primary tumor worth the risks it has. We had many cases when people were turned away when requesting the surgery for their primary tumors at their local treatment facilities less familiar with the slow growth nature of the ASPS. All the decisions at the MSK are usually done at the sarcoma board where the cases are reviewed by all docs and as understand they all concur with this recommendation, and less traumatic options are evaluated and ruled out such as local ablation of the tumor in the pelvic bone. I hope that other people with the complex locations of their ASPS tumors take notice of the institution if they need a second opinion.

You are facing a very hard surgery and a very long recovery but I would do the same and we all are rooting for you. You need to remove the primary tumor, we had no cases when people fared well with the primary tumors intact and your quality of life would not be good with this primary tumor's location anyways. Ivan also had two muscles removed from his right arm when his primary was resected. He consulted the doctor what specific exercises or moves he had to train in order to gain the movements back and he was very vigilant in it. As a result, he has no limitations in that arm use what so ever. Furthermore, the remaining muscles adapted and filled the space left after the resection and it is not even visible at all. I realize that everyone's case is different but this is just an example that you have to pay very close attention to the post-op rehab and find a good post-surgery physiotherapy doc to consult, it will pay out in the long run.

Brian

Yes the surgical choice of rending you body of this burdening disease seems to be the first step to fighting it. We ,as a family , have called it the search and destroy. Sloan Kettering is one of our choices too. Josh and his wife NIH and Sloan Kettering before their visit to Germany. They had friends they stayed with to offset the cost of the east coast expenses. You are in good hands. Please tell you family that they can participate on this forum as well. There are a lot of great people who would love to talk to them and answer any questions they may have. If they don't know the answer they will tell you and refer you to the appropriate person , if they can.

Prayers are always being said, but a special prayer will be with you guys March 12.

Thanks for the update

Sincerely

Debbie

P.S

Another link that we found helpful

Soft Tissue Cancer Information, Masonic Cancer Center, University of Minnesota

Dear Brian,
I am so happy that you are going to get that main tumor removed. I also walk a little at times with a limp but more from the 33 radiation treatments i had. Its worth it though! I know it sucks but i am so thankful you are going to get this over with... You have a surgery date an you are in my thoughts and prayers as everyone here is!

The impending surgery is frightening and frustrating an i looked at the date after on the calendar more then the surgery date it was more important to me because it was the day that it would be gone an i would rather celebrate an event then dread the surgery. I also rented things for recovery and looked at cool foods to order in and planed recovery with great things. I was so busy doing that time that was dragging pre surgery went by quickly. As they were putting me under i pictured all i had at home ready that was comfy and loving an it helped me a lot!

May your surgery be fast and thorough and may you be home with your loving family healing soon!

Brian, may be you already thought about it but just in case you haven't - may be you should start training the other leg and arms pre-surgery so it would be easier for you to walk on crutches and to use you arms to lift yourself during recovery time, to be able to do more with less suffering.

Brian,
Just wanted to say my son also had a month of radiation to his leg prior to tumor removal but the main thing we kept hearing was to get the primary tumor removed ASAP.
He did recieve physcial therapy and we continued with an excercise regimen to help him cope with the loss of muscle in his left thigh. I will keep you and your family in my prayers and you are are in good hands in NY.
cindy

Dear Brian,
Thank you for your thoughtful update. It was so very good to hear from you again. I applaud your pro-active search for a surgeon who is willing to do the surgery and capable of successfully removing your primary tumor and others in your gluteal, hip, and pelvic area. I am so sorry for the difficult surgery and post-op recovery that you are facing, but I admire your great personal strength, your willingness to do what needs to be done to reduce your body's tumor burden to better fight your ASPS, and the very positive attitude with which you are doing it. My most positive thoughts and very best wishes will be with you on March 12th for a very successful outcome to your surgery and in the following days for a speedy and full recovery. Stay strong, know that you are not alone and that the caring thoughts of those of us on this Board are with you, and update when you are able.
With special hugs, deepest caring, healing wishes, and continued Hope,
Bonni

Prayers for successful surgery on March 12 with optimal results.