Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi everyone,

I am new to this forum and very grateful that I found it as there are not many cases of patients with ASPS and it is difficult to find information on this topic in general. I have read many stories in this forum and my heart goes out to all of you who are suffering from this disease. I am also happy to see the many success stories as well.

I will write my story here and list my questions below. I am currently a 30 year old female working as an Urgent Care Physician currently. I first noticed a right gluteal mass 3 years ago during my intern year of residency. I thought it was a knot in my muscle and didn't pay much attention to it. My boyfriend (who is now my husband) at the time was much more concerned than I. Of course, being in the medical field, I thought it was 99% likely to be a benign soft tissue tumor of some sort. I continued to ignore it as it did not grow much and was busy working over 80 hours a week. I completed residency (trained in Family Medicine) July 2015 and at that point had a lapse of insurance until I started by new job in late August. The mass had been growing steadily and becoming more pronounced. Still, I put it off as I did not want to have surgery yet. I was busy planning my wedding and got married 2 months ago. I finally had some time to myself and asked my colleague to order an ultrasound for me to investigate the mass. I went for the US on 5/10/16 which showed a heterogeneous and vascular mass. They recommended an MRI which I had done quickly on 5/12/16. The report on the MRI stated that again it was heterogenous, vascular, with central necrosis concerning for a sarcoma. The mass was approximately 5 x 5cm on the report. I was referred to Dr. Lee from Columbia (an orthopedic surgeon who specializes in soft tissue sarcomas) who quickly scheduled my biopsy on 6/1/16. The biopsy report confirmed the dx of ASPS. I had surgery on 6/13/16 with wide excision of the tumor which measured 5.5 x 5.7 x 5.0cm. He was able to achieve negative margins. I had a CT chest/abdomen/pelvis and bone scan prior to the surgery which were negative for mets. We did not do a brain MRI as I am not having any symptoms. I followed up with Dr. Schwartz (medical oncologist from Columbia) who did not recommend chemotherapy or radiation at the time. However, they recently discussed my case in their tumor board and decided that I may benefit from radiation.

After doing a lot of research, I don't see too much evidence out there that radiation will help. I also know that it seems some patients respond better than others and there is just no way of knowing. My dilemma is whether or not I should have the radiation because it is so close to the pelvic area. I am planning on having children as soon as possible and the possibility that radiation could cause permanent damage including infertility is not an option for me. I still have to meet with the radiation oncologist and fertility specialist for an initial consultation. Perhaps, it will help me make my final decision. I just wanted to know if there are others that had experienced anything similar and if radiation vs. no radiation had any impact on local recurrence. At first, I was going to opt out of radiation but my oncologist said that my risk for local recurrence is about 20% currently and will be reduced to 5% if I do radiation. He gave pretty specific numbers, so I guess I will have to consider it now. I also saw a few posts about pregnancy and how ASPS may be hormone sensitive. Of course, there were only a few cases but again nothing confirmed. I wish there weren't so many uncertainties and that I didn't have to make so many difficult decisions.

I am recovering well from surgery, pretty much full functional status minus the missing chunk in my buttocks, so I am very lucky. I am trying to keep a positive attitude, improving my diet, doing the things that make me happy, and living one day at a time. I will appreciate any comments/shared experiences and will keep you all updated with my story.

Hi Nhi, welcome here.
Re. radiation -
1. The radiation oncologist can not give you any specific numbers for ASPS resected with negative margins, only for sarcomas in general and even that - for the negative margins it should not matter that much. It causes a permanent damage to muscles and bones in the area (post irradiation fibrosis) we have cases when people suffer from that. We did not see any cases of the local recurrence if the primary ASPS was resected with the good negative margins. If it metastasized to pelvic trough the lymphatic system the local radiation would not fix that probably. For preserving fertility there is some way to move the ovaries from the way and beside you have it both sides or collect eggs. But overall I am negative for the post resection radiation when it was done with the good negative margins.
2. The most frequent place of metastasis is lungs/brain/abdomen organs/bones. We have seen few cases when with the location like yours it has metastasized to pelvic bone, but mostly the route of dissemination is via blood vessels.
Some people do not get distant mets at all so there is a hope for you, but first 6-12 month after the primary resection are important to watch, chest CT scans.

Hi Olga,

Thanks for the quick response. Yes I have read all of those things and not sure how he arrived at these numbers. I am thinking that I will get a second opinion from MSK depending on what the radiation oncologist at Columbia says. I know that there are many ways to preserve my fertility, but of course I would rather not have to do it if there is no clear benefit in radiation + the possibility of multiple negative side effects as you have mentioned. Also, that would delay the time it takes for me to get pregnant. I am getting a repeat MRI of the local site + CT chest in 3 months.

Hi Nhi, welcome to the forum and so sorry to hear about the asps. Glad to hear that you are recovering well from the surgery and have no mets -- hopefully it stays that way forever.

I personally had radiation done to my primary site (left thigh) even though chance of recurrence is unlikely.

If you choose not to get radiation done, will you be undergo any systemic treatment or participate in any clinical trials? I'm guessing the answer is no since you plan to get pregnant soon..?

~Jen

Hi Nhi

My name is Debbie and my(our) son Joshua was dx'd in August of 2012

After surgical removal of negative margins we opted for NO radiation as we were too given the possibility of a 20% reoccureance.
I agree with Olga that those numbers had to be gotten from more agressive sarcomas studies and really shouldn't be factored in with ASPS when negative margins have been acheived. Reoccurance happens and should be looked at when negative margines can not be acheived.
Looking at the cost benefit factor I wouldn't go that route.

I'm glad you have joined us, but without a doubt under sucky circumstances,( by the the of Alveolar Soft Part Sarcoma) and I am sure looking forward to you input on your treatment and progress!

Truly glad to hear of your successful surgery and recovery.

Sincerely
Debbie Pearson

Ps our link

http://cureasps.org/forum/viewtopic.php?f=4&t=750

Also Kathy and Tom are in your neck of the woods .
And Olga can correct me as Toms sarcoma began in the lower spinal area ?


They've been combating ASPS since 2002

http://cureasps.org/forum/viewtopic.php?f=4&t=270

Memeorial Sloan Kettering is where our primary doctor is but we live in Kansas
Looks like Dr Schwartz is a doctor in your area? Was he orginally at MSK cause we were consulting with him in 2013 if so

I would also recommend Dr William Tap at MSK. He was my original doctor at UCLA before he moved to MSK in NY.

https://www.mskcc.org/cancer-care/doctors/william-tap

Hey there Nhi,

very glad that you found this forum that has gathered a lot of experience and knowledge of the people that are fighting this disease with years.

you will see that most of them here can offer much more that biggest number of doctors that encounter ASPS for the first time.

Regarding your scans I would strongly recommend that you do a Brain MRI. Waiting for symptoms is the worst decision when dealing with ASPS, after that maybe even a Bone scan so that you have a clear starting point.

hope all goes well

Dear Nhi,
I am so sorry for your recent ASPS diagnosis, but am grateful that you found your way to this Web site and that you have reached out to the Discussion Board for information, advice, and support. I am grateful too that your primary gluteal tumor has been successfully resected with negative margins, and that your pre-op chest/abdominal/pelvic/and bone scans showed no mets thus far. Based on my 15 years of ASPS experience with our now 34 year old daughter Brittany's disease, and my extensive research and observations, I, like Martin, personally disagree with the decision for you to not also have a brain MRI just because you are asymptomatic. Although there is statistically very little likelihood of you having brain mets prior to the development of lung mets, it is important to develop baseline scans for future comparison. Vigilant, regular, and pro-active scanning of ALL areas of the body is critically important in fighting/managing this unpredictable and challenging disease, and it is extremely important/essential to not wait for symptoms to develop because mets need to be diagnosed and treated at the smallest most treatable size BEFORE they become symptomatic. I strongly encourage you to obtain a brain MRI, and NOT just a brain CT, which is inadequate to detect small brain mets. I agree with Olga's and Debbie's advice regarding post-op radiation most likely being unnecessary since the surgeon was thankfully able to obtain negative margins on your primary tumor resection. Our daughter Brittany did undergo six weeks of post-op radiation to her resected left thigh primary tumor site, and although she has thankfully not had any reoccurrence to the primary tumor site, the radiation unfortunately did not prevent metastasis of her disease and she has heartbreakingly had widespread metastases to her lungs, liver, brain, tibia, breast, spine, duodenum, small bowel intususception, pancreas, and a superficial abdominal met. After 23 surgeries, ablations, radiosurgeries, and 2 failed Clinical Trials, Brittany has very thankfully now had seven years of disease stability for the past 7 years on a Cediranib Clinical Trial. It is extremely important to be very pro-active and knowledgeable in fighting this insidious disease, which it seems that you are. Regarding pregnancy, this is a very difficult issue because there is some anecdotal evidence that the hormones of pregnancy can cause renewed/increased/ aggressive disease progression which has tragically resulted in the loss of at least two ASPS patients who I am personally aware of. However, I also know one young woman whose disease was amazingly stabilized with aggressive traditional chemo treatment about 12 years ago, and she has since had two children with no disease reoccurrence thus far. It is certainly an issue which needs to be addressed and discussed with your oncologists before you proceed with getting pregnant. I know that you must be very overwhelmed with everything right now, and I know too how very difficult all of these decisions are. Please know that those of us on this Board are here to try to help you in any way that we can with shared researched and anecdotal information and strengthening support and encouragement on your ASPS journey which we all share. I Hope that you will find this Board to be very helpful to you in your battle, and that you will actively participate on it because shared information is one of our strongest weapons in fighting this extremely rare, little known, and poorly understood disease. Take care, stay strong, let Hope lead you through each day, and keep in touch as you are able.
Reaching out to share special caring thoughts, healing wishes, warm friendship, and continued Hope,
Bonni Hess, mother of 34 year old Brittany diagnosed with ASPS at age 19 in July 2001

Hi everyone,

Thank you for all of your responses and advice. I have scheduled an appointment with the radiation oncologist just to hear what he has to say about the potential benefits of post-op radiation. I am also planning on getting a second opinion about it from MSK before making my final decision. I am leaning towards not getting it due to the high risk and little potential benefit. There also seems to be a consensus for not getting radiation based on your responses. I have also read that post op radiation does not help prevent metastasis or improve overall long term survival. I believe they are hoping to control local recurrence in order to avoid having to re-operate in the same area which would be the only benefit that comes with a high cost. For those of you that had post-op radiation, how long did you have it for and at what interval? Any permanent side effects/damage from the radiation?

As far as the topic of having children, I have read about the pregnancy stories that were posted in the diet section which made it very difficult for me. Before being diagnosed with ASPS, I have everything in life to be thankful for.. a good career and great husband. I feel like my life has just started after 11 years of being in school and being a broke medical student. I finally make money and can afford to go on vacations and enjoy myself a little. The only thing that is left and missing is having children. We were planning to start trying in October after our honeymoon (before my diagnosis). I know that my husband really wants children, but he told me that he would rather have me live than to potentially risk having disease progression through pregnancy. My oncologist, Dr. Schwartz, told me that I "shouldn't let the diagnosis of this disease influence when to have children" when I had asked him about it, so I'm not sure if he is aware of these cases. We only had 1 visit so far so we have been communicating through e-mail. My plan was to get the 3 month scans and if clear I will try and get pregnant. The only issue is that I won't be able to get any scans during pregnancy so I will have to go almost a year without knowing if there are new mets. At this point, I think that I may have to just take the risk because of how important it is for me and my quality of life and hope for the best. I doubt that there will be much new information out there in the next few years regarding pregnancy and ASPS since we are dealing with an already very rare disease.

I will talk with my oncologist about the brain MRI. He did say to me that getting the MRI as a baseline is not a bad idea when I had asked him about it. I do agree that by the time I have symptoms, it would be too late so it's better to stay proactive. Of course, it is very rare that the mets would appear in the brain before the lungs.

I will keep you updated with my decisions. In the mean time, I have completely changed my diet to a cancer fighting one high in fruits, vegetables, healthy oils, nuts. I have stopped drinking alcohol, eliminated all the "junk food," highly processed foods and switched to all organic foods. I bought the Berkey water system to eliminate the flouride and other carcinogens in the water. Next step is to change my shampoo/soaps, detergents, dishwashing soaps and cleaning products. Of course I plan to do more yoga, meditation, exercise and most of all work less!! (I was a workaholic before this). I may have been diagnosed with this cancer as an order from God to stop and smell the roses and to enjoy myself. I am a big believer in positivity and being happy/grateful with whatever life throws as you.

I have read all of your stories (Bonnie, Debbie, Olga, Kathy and Tom) and my heart goes out to you and the many years of fighting/helping your loved ones fight this horrible cancer. I will always be thinking of you during my journey and praying for all of us.

Dear Nhi,
Thank you for your thoughtful updated information and perspectives. You are to be commended for your knowledgeable and pro-active approach to fighting your disease and your very positive attitude, all of which are critically important in this challenging battle. When Brittany was first diagnosed in 2001 we flew to MD Anderson in Texas from Seattle to try to obtain a second opinion and advice from the reputed to be very knowledgeable and experienced Sarcoma experts there. Very disappointingly and infuriatingly the pompous uncaring oncologist who we met with was very negative and rude, and in an abrupt response to our precious young then 19 year old Brittany's optimistic statement that she thought that she would/could win this battle because she had a very positive attitude and spirit, he arrogantly and with absolutely no compassion told her that it didn't matter what her attitude was, that she WOULD die from this disease and to just go back to Seattle and basically get ready to die!! That was almost 15 years, 23 surgeries/ablations/radiosurgeries, and 3 Clinical Trial ago. I would like to walk in his office today with Brittany and ask him to tell us again that attitude doesn't make a difference!!... We of course know and understand FAR too well that there is a harsh reality to this insidious disease, and that tragically attitude does not always win the battle, but it certainly makes the battle easier for everyone involved, and we personally feel that a positive attitude helps to stimulate the endorphins to strengthen the immune system to better fight this insidious disease. I am glad that you are going to pursue having a brain MRI to establish a base line for further scans, and to obtain reassurance that you have no brain mets. We strongly feel that stress can contribute to and stimulate disease progression, and that it is very important to try to reduce the stress in your Life as much as possible, which having the scan confirmation that you have no brain mets would definitely help to do. Regarding your question about post-op radiation, Brittany underwent post-op radiation following the resection of her left thigh primary tumor for six weeks, five days per week for a total of 30 treatments. She experienced severe burning and discoloration of the skin surrounding the targeted radiation site on her upper left thigh, but the burning eventually healed, and the discoloration over several years eventually faded. Otherwise, she did not seem to experience any other significant side effects of the radiation. I know and understand FAR too well the deep heartbreak of this disease interrupting and interfering with Life's plans, Hopes, and dreams. Brittany wanted to be a nurse and most of all, has always wanted to be a mother. Being diagnosed at age 19 and having to courageously aggressively fight her disease for the majority of the past almost 15 years has devastatingly prevented both plans and dreams from coming true, but we continue to hold very tight to Hope that someday she will be able to fulfill them. Although she has very thankfully now had 7+ years of disease stability on her Cediranib Clinical Trial, her oncologist feels that she may be macroscopically tumor free, but heartbreakingly probably not microscopically so we cannot risk taking her off of the drug which prohibits pregnancy as long as she is taking it. Quality of Life and following your heart and dreams to have a child is of course very important, as long as you do it with an awareness of the known nature of this disease to metastasize and progress, and are as knowledgeable as possible about the long term implications of Living with this disease. If you do decide to proceed with a pregnancy, perhaps you could be followed with MRI's which would not pose the CT scan radiation risk to your unborn baby, because vigilant, regular, and appropriate scanning is essential in fighting ASPS. Whatever decisions you make regarding, post-op radiation and pregnancy, please know that my very best wishes, most positive thoughts, and greatest Hope are with you and your husband. Take care dear NHi and stay strong, positive, and pro-active.
With special hugs, caring thoughts, healing wishes, and continued Hope,
Bonni

Dear NHI,
I had post-op radiation on right thigh every week for about 2 months in 2014.
The burn scars were very bad, and it is still very noticeable until now and maybe for a lifetime.
After radiation program was finished (there were 3 small mets in my lungs at that time) I still have a normal sex life and my wife got conceived and our daughter is living as a normal 9 months old toddler now.
Still even until now I'm still questioning whether the radiation was necessary back then, I took it just to eliminate even a 1% local re-occurrence.

Hello Nhi

Thanks so much for prayers and heart felt thoughts..

Life will happen as will /does ASPS.
As Bonni and Daniel have so gracefully presented, don't let it make you loose focus of your dreams.

Our daughter, Joshuas's younger sister, used a glutent free diet to become PG.
Discuss low glycemicia and acidosis with a naturopath oncologist..lactose useage

It seemingly can cause and induce metatic disease.

http://www.news-medical.net/news/201411 ... rcoma.aspx

Hormones should be another conversation as well

Mri's are being used more and more to DX tumors and their progression if needed. ASPS progresses very slowly as a rule.

What stage are you being called ? 2 ?

Is your doctor Dr Carl Schwartz from MSK?

Much Love
Debbie Pearson--mom of Joshua Pearson

Thank you Bonnie, Daniel, and Debbie for your comments regarding radiation. My oncologist is Dr. Gary Schwartz, so he must be different than the Dr. Schwartz you saw Debbie. Anyways, I asked him how he derived at the percentages (he said I have up to 20% change of local recurrence and they can reduce this to less than 5% with radiation), and he gave me a very vague answer stating that it was based on lower extremity sarcomas. He did not say if it was specific to ASPS. I have asked him to schedule a brain MRI, so I will be getting that done soon. I have also scheduled an appointment with a radiation oncologist on 7/7/16 (at Columbia) to discuss the treatment regimen and potential side effects for the specific location. Hopefully I will get some insight as to how high the risks are because I would be getting radiation to the right buttocks (close to my pelvic organs). I went ahead and scheduled an appointment with MSK for a second opinion as well. I tried to get an appointment with Dr. William Tap as recommended, but he is booked out until August and told me to see one of his colleagues. They have a team of about 6 sarcoma specialists at MSK, which is great. My appointment there will be on 7/12/16. I will keep everyone updated. Have a wonderful July 4th and hope everyone enjoys time with family and friends

Hi Nhi,
Hope you had a nice 4th of July holiday. That's good you're getting a 2nd opinion. Dr Tap is really good, but I'm sure if you get another doc from the same team it's just as good.

Hi everyone,

Just posting a small update. Since the last post, I have had 3 appointments total. I went to see the radiation oncologist from Columbia who told me that I should seek a second opinion at MSK regarding RT. I first went to see Dr. Gounder from MSK (medical oncologist) who told me that I should see Dr. Alektiar (radiation oncologist at MSK). Basically, all of the doctors said the same thing, which is that the overall risk of local recurrence from all types of sarcomas is about 20%. With RT, they may be able to reduce that number to <5%. Dr. Alektiar said that my risk of local recurrence is likely closer to 10-15% due to the size of the tumor (5.7cm) which is very borderline to the 5cm cut off and because my surgeon was able to achieve negative margins. He told me all of the risks of radiation which includes short term side effects such as radiation dermatitis, fatigue, pain in the area along with potential long term side effects (scarring of the surrounding tissue, possible damage to the bones/nearby structures and damage to the ovaries, and secondary tumors). He said that the risks would be very low as they would do everything possible to focus the radiation on the area to avoid as much damage as possible. However, if I did want to get RT, he said it would be wise for me to see a fertility specialist and freeze my eggs before proceeding. In the end, both of the radiation oncologists told me that it is 50/50 and that the final decision is up to me because they do no strongly recommend for or against radiation.

After multiple appointments and debating what to do, I have decided not to pursue RT. I would rather just monitor it and have another surgery if it does come back. I think that is better than to do RT and risk having potential long term side effects (especially effects on my ovaries). I just have to be happy with my decision and understand the potential risks and benefits from both options.

I also had my brain MRI done w/ and w/o contrast and it was thankfully negative.

My next scans will be in September, so I will update at that time.