Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello everyone! Thank you for all your posts - it has been very helpful to read about everyone's advice and experiences.

I just wanted to share my story and status of my health and treatment. I'm open to any comments and advice that may help in my fight against ASPS. Here's a brief intro:

I had a lump in my left thigh ever since i was 17 or so but never got it checked out until December 2008. (I'm currently 29 yrs. old.) I was diagnosed with ASPS in January 2009 by my orthopedic surgeon at USC, who removed the ~2 inch tumor from my thigh. He at first thought it was some kind of benign tumor after biopsy-ing during the surgery, but a week later he found out it was actually ASPS. After receiving the bad news, I then received localized radiation to my thigh for a month. I also had a brain MRI and chest CT scan performed soon after my surgery. The brain MRI showed no mets, but my chest CT showed mets (innumerable in numbers >100) in both lungs. The 3 largest nodules ranged from 10-14 mm in size.

In mid 2009, I started seeing a UCLA oncologist to receive treatment for the metastases in my lungs. I was on Nexavar for about 3 months, but the results showed growth after my 3 month follow up chest CT scan. In 2011, I was on a clinical trial for a year where I received weekly infusions of Torisel and a study drug (I forgot the name). While on the study, I was fairly stable for about 6 months, but had slight growth for the remainder of the 6 months. Since my CT scans showed growth for 2 consecutive scans during the 2nd 6 month period, I was unable to continue the clinical trial due to the trial's restrictions.

In April 2012, I received radiation to 2 of my 3 largest nodules (about 17mm in size) in my right lung using Intensity Modulated Radiation Therapy (IMRT) by a radiation oncologist at UCLA. IMRT is a cutting edge technology and a highly precise external beam radiation therapy. Follow up chest CT scan have been performed every 6 months since the therapy and have shown that the radiation therapy was a success and the radiated nodules have shrunk significantly. Unfortunately, now I have pneumonitis as a result of the lung radiation. I have been coughing for over a year now, have trouble breathing deeply, and have constant shortage of breath. I've been taking an oral inhaler for the past month, but it doesn't help much.

Currently, I am taking Pazopanib, which has been showing good to fairly stable results. I have been on Pazopanib since July 2012. For the first 6 months, my chest CTs showed a slight decrease in the number of nodules in my lungs. My most recent CT scan in April 2013 showed slight increase to stable results. I will get my next 3 mo follow up chest CT scan in July 2013. Side effects on this drug are unpleasant and have made me pretty weak, but quality of life is still pretty good for the most part. My boyfriend has been extremely supportive and helps me stay uplifted and happy.

After reading many ASPS member postings, I am a little worried about not having annual brain MRIs or bone scans performed. I haven't had a brain MRI since early 2009. I emailed my oncologist today to request a brain MRI. How important is it to get a bone scan performed? Also, I read that several people have been getting surgeries from Dr. Rolle in Germany. Is there no other surgeon like him that can do what he does in the United States? According to my CT results, I probably have around 100 nodules in my lungs, so I'm guessing that's too many to get operated on...

I appreciate any thoughts or advice. Thank you so much for reading!

My thoughts and prayers go out to all of you fighting this horrible disease.

-Jen

Dear Jen,
As with everyone on this Board, I am so very sorry for your reason for being here, but I am grateful that you found your way to the CureASPS site, graciously shared your information, and reached out for information and input from our participating ASPS Community members. It sounds like you have been very proactive in your approach to fighting your disease which is so critically important. I am holding very tight to Hope that Pazopanib will be very successful in stabilizing the progression of your disease and shrinking/destroying your multiple lung mets. There are several other patients on this Board who are also being treated with Pazopanib, but unfortunately they don't update their treatment experience and results very often so it is difficult to know the status of their situation and treatment results thus far. There is a specific Pazopanib topic on this Discussion Board where you can post your updates so those who are following this promising new treatment can more easily access the information. It is very important that you have both a brain MRI and a full body bone scan since these areas apparently aren't being followed by your oncologist. Unfortunately, ASPS mets can, and sometimes do, show up anywhere in the body since ASPS is a metastatic disease, and even though the areas being scanned and followed may appear relatively stable. It is difficult for me to understand why most oncologists are not more vigilant in their scanning and monitoring of this metastatic disease which is known to metastasize to the brain and bones since it is so important to find the mets at their smallest possible size to provide the best chance of a successful treatment response and outcome.
Olga and Ivan can probably better answer your questions about Dr. Rolle and his lung met laser resection procedure, but as far as I know, he is able to successfully treat and remove large number of lung mets such as you have.
I am so grateful that you have a very supportive boyfriend as it is so important to have the strengthening love and support of family and friends in this challenging battle.
My very best wishes are with you for very good three month scan results in July, and I will be anxiously awaiting your update. Hopefully the scans will be chest/abdominal/pelvic CT scans and not just chest CT scans as you mentioned because, as I have said, it is extremely important to scan all areas of the body and not just those areas with known mets.
Please take care Jen and know that I and others on this Board are here to try to answer any questions which you may have and to provide strengthening support and encouragement on this difficult journey which we all share.
With special caring thoughts, healing wishes, and continued Hope,
Bonni Hess, mother of 31 year old Brittany diagnosed at age 19 in July 2001

Hi Jen, welcome to our board. I am sorry to hear that you was one of unfortunate 10-15 % of the people that got radiation pneumonitis as a complication after the IMRT treatment - this is why we are determined to avoid any radiation treatments to the Ivan's lungs as long as it is possible and use a cryoablation instead (in some cases it is not possible though by technical reasons).
To answer your question about laser assisted surgery by Dr.Rolle - it is not avail. in US and is a fairly rare technology overall and even if it avail. elsewhere in Europe, the experience with the very multiple mets cases Dr.Rolle has is unique (incl. his specific ASPS experience). But there are the technical limits to his technology as well and I am afraid that to successfully remove hundreds of lung mets they have to be fairly small and not seen on the CT scan - i.e. he has more chances to remove all mets when there are 20-30 are visible on the scan and 70-80 discovered on the surgery versus 100 being see on the scan just because when they are not seen, it means they are smaller than 0.5-1 mm so can be burnt on the spot with very little loss of the lung tissue. My other concern would the overall lungs condition - it is a fairly enduring surgery and to be able to recover after the extensive damage the surgery would do, the lungs have to be healthy. But you can ask Dr.Rolle directly, he is easy to ask for an opinion/pre-evaluation.
What oral inhaler do you use? I've read that sometimes the higher steroids doze is needed and under-treatment can cause the fibrosis to develop, and also that there is inhaled angiotensin-converting-enzyme-inhibitor (captopril?) that can be used, and also that radiation pneumonitis can lead to the development of bacterial infections that need to be considered if the patient keeps coughing. You probably need to investigate it more.

Hi Olga,

Thank you for your advice. I am using Advair as my oral inhaler. It seemed to help for a couple of weeks but my cough came back and is the same as it used to be before I started the inhaler. Is there anything else you'd recommend?

Regarding Dr. Rolle, I agree that since I have around 100 nodules in my lungs that are showing up on my chest CT scan, that probably means I actually have double the amount, which makes it too numerous to be operated on...

Thank you!

Hello Jen
My name is Debbie and I am Josh's mom
Could it be that there were over 100 counted in both lungs combined?
Dr Rolle can look at the report or scan and tell you whether you are eligible or not
Doesn't hurt to ask!

Sincerely

Debbie

I would recommend you to get a referral to a pulmonologist or someone who specifically treats or has more experience with the radiation pneumonitis to change the treatment plan because the anti-asthmatic inhaler might be not the right treatment for you? One part of the combo Advair is the anti-inflammatory steroid but I do not know about the other part or about the dose? And I agree with Deb, you can ask Dr.Rolle.

Hi Jen
I am also in Ca an have sarcoma..
I wanted to post that you can send the scan to Dr Rolle an that he may help you ...
I dont know if you have more then 100 mets in your lungs... I have had 13 for about five years now slowly growing an no new mets yet! I would stay open to the fact that there maybe more mets BUT i would first till you see more go with the i have 100 mets til i was told there is more! <3
I have two surgeries coming up at Saint Johns close by UCLA.. it is where i had my first thoracotomy an will have two more done in August of this year...
Are all your Oncs at UCLA? I have been told its a great place i am at Angeles Clinic an Cedars...

I read what Olga said about the inhaler an the medication an she is right .. please seek out that Dr to get on a possibly better med...

Sorry you also have sarcoma.. But, welcome the forums

Thanks Debbie, Olga and Amanda.

The oral inhaler (Advair) was prescribed by my pulmonologist. I am scheduled to see her again at UCLA in a couple weeks so I'll see what she has to say.

Amanda - All my doctors are at UCLA Santa Monica. I consulted with Dr. Forscher at Cedars Sinai a long time ago, but decided to go with my current UCLA doc (Dr. Chmielowski). Also, how have you recovered from the thoracotomy? Was it hard to recover from? I'm not familiar with the procedure...

Debbie - I think I have over 100 mets in the lungs combined...

Hello again Jen,
Did you receive a response from your oncologist yet regarding scheduling a brain MRI and full body bone scan? If you need information and data to support your request and the importance of regular brain MRI's for ASPS patients, Olga and Amanda have posted some excellent resource information in the May 20th and 21st "scanning to watch out for the brain mets" subtopic under the main "Brain Metastases" topic on this Discussion Board.
With special caring thoughts, healing wishes, and continued Hope,
Bonni

Hey Jen,

Welcome to the ASPS forums, even under these kinds of circumstances, it's good to know you're not alone. My husband was diagnosed last year with ASPS, primary in his chest wall, then two lung surgeries with Dr. Rolle (left and right, with over 130+ mets resected/burned per lung), and very recently, a new tumor in the sacrum.

I would say, based on our personal experience, get a bone and pelvic scan. Last year, when Kevin was first diagnosed, I asked the doctors about getting a pelvis scan and the general feeling was we didn't need it, we had a bone scan in Oct 2012 and that would be good enough. About two weeks ago, after months of complaining of pain and numbness in his left leg, a 5 cm tumor was found growing into his sacrum and surrounding tissue. Not going to lie, really wish I had insisted on the pelvic scan last year. Now it's too big to deal with easily and if surgery is possible, I understand it will involve very real risk to Kevin's quality of life.

So yes, get that pelvic scan. Better to be safe than sorry. I would also recommend contacting Dr. Rolle with your scans, he'll let you know if he thinks you're a candidate or not for his surgery. I'm really glad to hear you've had positive results on Pazopanib. My husband started it two weeks ago, and other than some fatigue seems to be doing okay so far, but I guess we'll see.

Glad you found your way to these boards, lots of love and hope to you and your boyfriend.

-Connie

Jen, your account seems to have disappeared. I'm not sure why, maybe it was some kind of a bug. We didn't delete your account. Please re-register!

Hi Jen
I am recovered as much h as possible an am almost 100% its been a few years though an it was not the lung surgery that was so hard to recover from. I had part of my chest wall removed *fun huh?* lol an also four inches of my 8th rib on my back removed... this is why the long recovery.

I will be having two more lung surgeries soonish an other then the right side being a re do an more scar tissue i believe that i will push thru this a lot faster then the last because its only the lung

Have you been to the Angeles Clinic yet it is in Santa Monica an its great my chest surgen is there Leland Foshag an he is a positive doctor no drama and also a great surgen. I would also consult with him if i were you.

I still see Dr Forscher every six months an he an Susan are doing well.. I have been told by many they love the doctors at UCLA so i am happy you are with them! They all talk btw because they are sarcoma specialists an so close i think they all do lunch because when i say i am talking to one they say to tell the other hello or ya he is a good doctor, lol

How is your breathing now an did you get on a new med?

I saw Ivan said that your account was gone I hope you are still here it is nice to talk to someone that is close by me

Jen, I suspect that the fracture to that rib might be a result of the damage caused by the radiation to your right lung, it is a well known complication of the radiation, but we also have a member here - Amanda - who had the met to the rib so I would mention that to the oncologist and ask for a review of the scan image from this point of view - to rule out the possible rib met. If the oncologist needs the confirmation that the rib met might occur in a setting of the mostly stable and slowly growing lung mets, I am sure Amanda would be willing to share with you the name and the contact info and your oncologist probably knows him personally - easy to ask.

Thanks Olga, I'll follow up with my onco about it and ask him about it.