Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi everyone, i'm Mint from Singapore. I was diagnosed with ASPS in Aug 2009.

It began with a mass growing in my left thigh but i didn't bother it at first as it was painless and wasn't affecting my daily activities at all. I started to get worried when the mass started to grow bigger so i went to the doctor's and did an MRI for left thigh as well as chest Xray (instructed by doctor). Results showed that the mass in my left thigh was a tumor and the images from the chest Xray of my lungs were not clear (with multiple metastatses). Thus the doctor concluded that my tumor was a cancerous one.

I was arranged for a surgery to remove the primary tumor so as to prevent it from further spreading. About 2 weeks after the surgery, my oncologist started me on my first chemo treatment with the drug Duxorubicin for 5 cycles as that was the limit my body could take. CT chest results showed some reduction in the size of the tumors in my lungs and i took break for close to a year as i thought my condition had stabilised (with regular CT scans in between). However i started to have very regular cough and that was when my oncologist suspected that the tumors in my lungs were growing bigger, that was in late 2010.

I was then on Sutent (37.5mg) with 4 weeks on 2 weeks off for 6 cycles, but at the same time, i also experienced bad swelling on my left knee. I then did an MRI scan on my left knee and mulitple lesions were found in the knee bone and shin bone. Sutent helped in ceasing my cough and easing my leg pain. CT scan also showed promising results in reduction of the tumors' size also with other tumors disappearing. It was good news to know that Sutent was working well for me but during the midst of the 5th cycle, i started having very bad headache, double vision, vomiting and even had one incident of seizure. MRI brain results showed multiple tiny tumors in my brain and i had to begin on whole brain radiation therapy due to the multiple tumors discovered. I was still continuing with Sutent and had to take Dexamethasone (to stop my brain swelling) and Dilantin (to control my seizsure and prevent it from occurrng again).

During the 6th cycle of Sutent, i did not experience the side effects i had from previous cycles and my persistent cough is back. After the cycle had completed, i did CT chest scan and MRI left knee scan to see the progress. Results this time round showed that the condition of my lungs had worsen and the tumors in my bone have a slightly increase in size. Sutent had lost its effect on me and i couldn't continue anymore.

Right now, after failing sutent, i am on another chemo drug called Gemzar+Everolimus (just started yesterday). Any promising results from this chemo drug? I am getting really pessimistic and starting to lose hope already as my condition keeps getting worse. I am also very worried about the condition of my left leg, i fear that if the condition keeps getting worse, my left leg may have to be amputated away, i really do not want that.

Sorry for the long post! Just sharing with you guys my personal story with ASPS.Take good care!

Regards
Mint

Dear Mint,
I am so deeply sorry for your ASPS diagnosis and all that you have been through with continuing and disseminated disease progression during the past two years, but I am grateful that you found your way to our Web site and that you have reached out to our Discussion Board. I would like to welcome you to this Board which is an invaluable source of shared researched and anecdotal experience information and strengthening support for ASPS patients and their families who are fighting this extremely rare, little known, and poorly understood disease. It sounds like your disease has taken a similar path of progression to that of our now 29 year old daughter Brittany who was diagnosed ten years ago at age 19 with a primary tumor in her left thigh and over 50 mets in each lung. We have tried to manage her disease during the past ten years through vigilant scanning, continued resections of any new resectable tumors, Gamma Knife radiosurgery for several unresectable brain mets, radio frequency ablation (RFA) for a met in her tibia, Cryoblation for several lung mets, and three Clinical Trials ( a GVAX Immunotherapy vaccine trial and an ARQ-197 trial were unfortunately unsuccessful). Brittany is currently on a Cediranib Clinical Trial which has thankfully been successful thus far for the past two years in stabilizing her rapidly progressing disease and shrinking/destroying her multiple widely disseminated mets. Cediranib is another anti-angiogenic tyrosine kinase inhibitor (TKI) similar to Sutent, but which has shown more dramatic tumor shrinkage and longer sustained disease stability than Sutent for some, but unfortunately not all, ASPS patients. However, like Sutent, it seems to eventually lose its effectiveness and rapid rebound can occur when resistance to the medication occurs and the medication is discontinued. Having said that, it is still the most promising and effective systemic treatment that we have found during the past ten years of Brittany's challenging battle, and since you had an initial successful response to Sutent, it might be worth trying Cediranib now because there is some data to support that switching to another TKI when the first one fails may provide renewed disease stability and tumor shrinkage. To my knowledge Cediranib is currently only available in Clinical Trial at the National Institute of Health (NIH) in Bethesda, Maryland USA, but we have had a couple of international ASPS patients participate in the Trial there so it might be worth at least looking into for you. Regarding your new treatment with Gemzar (gemcitabine) there is information available in the Systemic Treatment Forum under the Chemotherapy Topic on this Board about one ASPS patient who did have a successful response to Gemzar, so you may want to read that information if you have not already done so. Ther is also in the Systemic Treatment Forum information about Everolimus (Afinitor) having received FDA approval, but I am not personally aware of any ASPS patients who have been treated with this drug. Also, I was wondering if RFA or radiosurgery like Cyberknife has been considered for trying to destroy the bone mets in your leg, and if not, I would encourage you to discuss these options with your oncologist. Additionally, Cryoablation might be a possible treatment option for some of your most concerning unresectable lung mets to try to reduce tumor burden.
I know that this response is very long and you are probably overwhelmed with everything right now, so I will close, but please know that you are not alone in your battle dear Mint, and that those of us on this Board are here to try to help you with shared information and support. My very best wishes and most positive thoughts are with you for a very successful response to your new chemo treatment, and I will be anxiously awaiting your next update. In the meantime, please take care and keep in touch as you are able.
Reaching out to share special caring thoughts, healing wishes, and continued Hope,
Bonni Hess, mother of Brittany Hess diagnosed in July 2001

Dear Bonni,

First and foremost, i would like to thank you for taking time to reply to my post. I am so glad i found this forum. Similarly, i feel deeply sorry about your daughter, Brittany's, ASPS diagnosis. As an ASPS patient, i can totally understand what Brittany has gone through and is going through. It is devastating to know that despite seeking treatments already, the vicious cancer still continues to spread. Could diet be even part of the reason why the cancer spread?

With regards to Brittany, how is she right now? Everything going well? Condition stabilised? Coping well with the side effects?

About the drug Cediranib, my oncologist did mention it to me, along with Sorafenib and Ifosphamide. However, he said that this Cediranib belongs to the same "family" as Sutent, thus there might be a slight chance that the drug might fail me now as Sutent has lost its effect on me. Therefore he suggested to try me on the combination chemo of Everolimus+Gemzar for 2 cycles first (at the same time to see if my leg pain and persistent cough go away). In addition, i will definitely discuss with my oncologist the various options you provided. As i am currently on Everolimus+Gemzar already, i will constantly provide updates on my condition. Hopefully all goes well please.

The fact that my cancer has spread to many areas (despite treatments) has made me very upset. How has Brittany been reacting to her condition for the past years? Based on what you have mentioned, i really admire her courage to keep on fighting this battle. Not to forget, it must have been tough on you as well. I truly wish for the best for Brittany and all ASPS patients. Take good care. Hope to hear from you soon Bonni!

Regards
Mint

Mint,
One thing I can personally attest to in the case of Brittany, and my daughter Jordanne, is that they are fighters. Keep a positive and fighting spirit. Don't let the new mets get you down for more than a couple of days and keep looking for new treatments. This forum as well as the ASPS facebook page are invaluable tools to help you get good information to share with your doctors.
You may get more information than you need or more advice than you can handle so sort through it and choose what is best for you with the advice of your doctors and those who love you.
Surrond yourself with a good support group.
Thank you for sharing your infomation that may help someone else out there.
Blessings,
Dotty Wheatley
Mom of Jordanne Gerbing dx 2009 at age 22.

Hi Mint!
Welcome to the forums.. we all had long posts to srat with i think because we all have so many un answered questions and untill i came here i had not much information at all!
I am very sorry that you are also now dealing with ASPS but there is light there are many new treatments now and there is real hope.
Anyhow, just wanted to say hello and welcome

Hi DottyW,

Thank you for your reply! It's really comforting to see replies and words of encouragement from people i do not personally know, as well as knowing that i am not alone in this. All these have definitely helped in telling me to perservere on and not give in to this vicious disease. Really admire Jordanne and your courage in fighting this! I pray for the best for Jordanne and all ASPS patients.

Regards
Mint

Hi Amanda,

Thanks for your reply! Really nice to hear from you. Indeed i have many doubts and concerns regarding my condition, sometimes even the doctors are unable to give me any answers. I am so glad i found and joined this forum, and at the same time, share my personal story with others. Hoping for the best for all ASPS patients.

Regards
Mint

Hello Mint, and welcome to the forums! Great first post, with all the necessary information - well done.

Yes, cediranib is the same class as Sutent (sunitinib) but there is a good chance you will respond to it. It might give you another period of shrinking and stability. We've had some remarkable responses, like Brittany. I would focus on locating the drug and giving it a try.

Also, cediranib is unique in that it crosses the blood-brain barrier, so it may even help with the brain mets.

Hi Ivan,

Thank you for replying! Seems like Cediranib has indeed received many positive feedback and has helped many ASPS patients. I will eagerly talk to my doctor about this miracle drug. Thanks Ivan for the information! Hoping for the best for all ASPS patients.

Regards
Mint

Mint,
please also ask you Dr about trying http://www.cureasps.org/forum/viewforum.php?f=59 from what i have seen many are stable and the side effects are managable. Right now this is my first choice if i needed a drug for me...

Hi Amanda,

Thanks for letting me know another drug that could help in treating ASPS! This forum is really such a big happy family which i didn't regret joining at all. Hoping for the best for all ASPS patients.

Regards
Mint