Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello Everyone!!!

The first thing I would like everyone to know is how hard, I have been debating whether to post or just stay quiet; to be honest with everyone, I have been reading this forum for more than six months now. Every time that I read a story, or a publication on ASPS, I kept saying to myself, you got to share your story too. So, finally here I am, please bear with me as this disease is not new to me, but the fact that I am opening up about it is.

My name is Mario. I am 21 years old. I was diagnosed on May of 2007; I had just turn 19 then and really did not even care about Cancer at all. I never in my life thought that Cancer would touch me or my family for that matter.

Since I was 16, I started feeling a slight bump in my left chest. I did not pay much attention to it then. I remember that once I tried to ask a doctor about it, and he told me to get insurance and then we would talk about, so I just really let it go.

When 2007 came around, I started having pain in my chest while I worked out. At first there really wasn't much, but with time it got worse. I finally decided to go to the doctor on April, and the walk in clinic doctor send me to a the sonogram technician. She then took a look at my "bump" in my chest. She call two more doctors so that they could take a look at the images and let me tell you there were so many blood vessels visible it scare me.

They all decided that the wisest thing to do would be a biopsy and it was done. Three weeks later, I was given my diagnosis of Alveolar Soft Part Sarcoma. It devastated my family and I was seriously scare. Once, we were able to go beyond the point of knowing that I had cancer; we started asking about treatments and such. We talked to a thoracic surgeon and he flat out told us, he did not had any experience whatsoever with this type of disease. He suggested we removed the primary which was 5.8 cm. At the same time, he also told us that he would try to call around and see if he could find me a experience oncologist.

A week after this, I answer the phone and I am told that I had been accepted at St. Jude Children Research hospital. We were so happy and bless to hear that. It was amazing, because of my age we had never thought of St. Jude, but they accepted me because of how rare the disease is. I was so bless that day. We drove to Memphis and met with my primary oncologist Dr. Sherri Spunt. She explain how the disease usually develop and how we could treat it. We decided to remove the Primary asap. Two weeks later, I had a Masectomy (I am a guy and a masectomy really sounds bad lol) in a very large scale; pretty much all of my left pectoral muscle was taken off. We then decided to do 7 rounds of Ifosfamide and Doxorubicin. At the same time we decided to do 36 rounds of radiation, total radiation given was 55.8 grays to the site of the Primary ( Reason was that, they save my ribs, instead of taking them out).

By the end of November, I was able to go ahead and go home. I was pretty much disease free as far as scans went. Now, I did had three spots in my lungs since the beginning in May. Dr. Spunt kept a close watch on them and told us that they were more than likely pneumonia or a infection or such. They never grew in 2007. I had a pretty good Christmas in 2007. I went back to St. Jude every 3 months for check up and did for 9 months until late 2008. We found that there were new spots in both my lung. they vary from 3 to 4mm. I was devastated again, even though I knew of the high likely hood of metastases to the lungs. We decided to watch them for a bit and we did for 6 months. Growth on the little nodules was substantially small the most we saw was 2mm in growth in a 6 month period. I think that it is pretty much safe to say that, I believe I had a positive reaction to the chemo because it did keep my nodules from growing or appearing. I did had 9 months of clean lungs. well by the start of 2009 we decided to start looking at ways to fight the nodules. We decided to do the ARQ197 trial. Dr. Spunt started the process to get me in the trial while, I decided to go ahead and do a thorocotomy on my right lung and leave the left with nodules to see if the ARQ197 work. I underwent a thorocotomy on the 1st of June. After the surgery, I started on the ARQ197 on July 16th, 2009.

My leading doctor on the ARQ197 trial is Dr. DuBois in San Francisco. The whole team at UCSF has been really nice. I am also happy to say that I have had a somewhat positive reaction to ARQ197. My second CT scan show that my smallest nodules have actually shrink. Awesome. The bad news though is that I do have a big nodule in my left lung that is about 1cm. This nodule is the only one that grown. It has only grown slightly but nevertheless growth was seen. I go back to San Francisco on the 12th of October only to do a EKG and to do blood work and get more pills.

So there it is, sorry Its so long, I am really hoping that ARQ197 works on the big nodule too. One good thing is that so far my brain is free of disease, awesome. I sincerely hope it stays that way.

Again sorry I wrote so much and I hope that this helps everyone. I know that we are facing something that is just overwhelmingly huge. I cannot express how worry I get, or how I am scare to go into the ct scan tube. I cannot explain to you how sorry I am, that I am the reason why my mom cries all the time. I know that this disease has not only touch my life but my whole FAMILY has suffer because of this. Its just like a roller coaster you know, and to a point I think it will never end.

To all of you, I say this hang in there.

Dear Mario,
Thank you so much for joining our Discussion Board and for your thoughtful detailed sharing of the information about your ASPS journey and treatments. I am so deeply sorry for your ASPS diagnosis and for all that you have been through, but I am very grateful that you found your way to this Web site and that you have now reached out to share your story with us. On our eight year journey with our daughter Brittany's ASPS, we have found that networking and sharing information with other ASPS patients is one of our most powerful weapons in this difficult battle. If you have read the section in this Forum on ARQ-197 treatment, you are probably aware that Brittany participated in the ARQ-197 Clinical Trial at UCSF in San Francisco last year in the Fall of 2008. Dr. DuBois was also Brittany's Clinical Trial oncologist, and he was very kind, caring, compassionate,dedicated, and knowledgeable, as was his entire staff. Unfortunately, the ARQ-197 was unsuccessful in preventing Brittany's multiple mets from growing so she had to withdraw from the Trial after only two and a half months of treatment, but each patient is different, and each patient may have a different response to the same medication. It is very encouraging that you have had some shrinkage of your smallest lung mets, and only slight growth in the largest one in your left lung. My best wishes are with you for a very successful response to the treatment with continued shrinkage of your mets and stabilization of the disease. If you are not already aware of it, Dr. DuBois's patient care coordinator can help make housing arrangements for you and your family at the wonderful Family House when you are in San Francisco for your clinic visits. Family House is a very comfortable, warm, and welcoming place for out of town pediatric patients and their families to stay free of charge while undergoing treatment at UCSF. Dr. DuBois's patients are eligible to stay there even if they are over 18 because Dr. DuBois is a pediatric oncologist and Family House provides housing for his patients if arrangements are made through his office. If there are any questions that I can try to answer for you about Brittany's ARQ-197 experience or anything else, please feel free to write me on this Forum or to write to me at my personal e-mail which is BonniHess@aol.com. In the meantime, please take care Mario, know that you are not alone in your battle with this very rare disease, let continued Hope lead you through each day, and keep this Forun updated as you are able.
With special caring thoughts, healing wishes, and continued Hope,
Bonni Hess

Mario, hi, I'am Ivan's mom, Olga, you can read his topic here with his name. He is now on a trip for a few days with his GF but after he's back - I am sure that he is willing to communicate with you directly if you want to, as he is too very young - only 23 but was Dx already almost 6 years ago. He has already had 5 lung surgeries but he is very fit and athletic, plays volleyball and tennis, runs, cycles and recently added hi-rise stairs running to his routines. It is not easy for him to gain his strength back after the surgery but he works on it as he can.
I would welcome to see your mother here too if you do not mind, at least she can read everything we share as I too feel that we support each other by simple understanding what a damn disease is it, it is almost impossible to explain to any outsider.

Hi Mario,

Welcome to our community and thanks for posting. Our daughter was on ARQ197 for a total of 9 months, and we think it helped reduce the number of new metastases. We eventually left the study because of the growth of a few, but not all lung nodules, but we thankfully have not seen a new nodule since she was on ARQ.

Also our daughter's case (she's 12), we have seen some grow up to about 1 cm, then stabilize. It does happen. Also there was even one in our daughter's case that sort of quickly appeared about about 7-8mm, then started looking like a deflated balloon and now seems a bit smaller a year or so later.

Hope you enjoy the Bay area while you are there. It is a lovely place.

'F'

Hello Mario,
Just wanted to welcome you to the Board where the information and support found here is outstanding.
My son was also diagnosed in 2007 and we have been participating in the ARQ 197 study also with a
great staff in Miami since March 2009( so far stable with no real side effects). I wish you and your
family continued strength and hope. Please invite your mom to share any questions here too.
Blessings,
Cindy

Hello Casha
Welcome to the forums! I am very sorry as I am with everyone that ASPS has touched and this would include the loved ones. I am very glad you are here now and can post and talk with everyone First, i wanted to tell you that reading this made me start to cry...

cachabamba10 wrote: Again sorry I wrote so much and I hope that this helps everyone. I know that we are facing something that is just overwhelmingly huge. I cannot express how worry I get, or how I am scare to go into the ct scan tube. I cannot explain to you how sorry I am, that I am the reason why my mom cries all the time. I know that this disease has not only touch my life but my whole FAMILY has suffer because of this. Its just like a roller coaster you know, and to a point I think it will never end.
To all of you, I say this hang in there.

I want you to know what it brought back the memory of my father and I as I was about to go in to have this evil tumor removed putting are heads together and I was crying very hard and for the third time in my life I saw my father cry :*(

We feel helpless at times in this the patents and the parents and we are all in a fight to make this invader as I call it go away and sadly in this journey many tears fall from all involved! As a parent of a child that has a disability I have cried a lot also for this and felt desperate to make him better this is also what I saw in my fathers eyes...

There are a lot of parents here that are amassing and I am amassed at them and cherish the posts they make and the long hours of work and research that is done by them. They have helped me make crucial decisions and also to be informed at times more then many doctors and this is a god send! Maybe it would be a good idea for your mother to post here to them? Then she will not feel alone any longer...

I am also scarred of the CT tube I hate it and I am claustrophobic and this is why no MRI has been done on me I will need to me put to sleep to do this.

You say "Hang in there" I agree! Tell your mother this and your family and spend time doing the things together that you did before this happened. There is hope and more now with this diagnosis then before! never let hope fade and when it does take it back! You have cancer it doesn't have you! I still cant remember where I read that but it is very true! Right now I am in a low time of feeling and this is normal it is like as you said a roller coaster *sigh* But, you know that ride will also go back up again and when it does its the best

Hold onto hope and faith and love...

In healing hopes for all <3

Amanda

Thank you everyone for the replys, I really enjoyed reading them. Sadly, my mom only speaks and understands Spanish. She is trying to learn English but it will take quite some time. I have showed her the forum and the stories. She feels amazed with this site.

Bonni, Dr. Dubois told us about the housing opportunities in San Francisco. We are bless that we actually happen to have some family in Castro Valley which is located in the east bay area. I always just go and stay with them. They make my stay in San Francisco a memorable one every time. Its amazing how my family has step up and help in every way possible. I am very sorry that it took so long to write some, but sadly my boss has suffered a stroke and because of that; I have had to take care of the office. I will say something though, The ARQ197 seems to be making me really tired all the time. Since, I have started taking it. Thats the only side effect though. I go for a CT Scan on the 9th of November, and I will post the results as soon as I am able.

Cindy thank you for your response, Let me know if your son experience any other side effect. I would really like to know also how your son is doing every 8 weeks. I think that it is very important for us to communicate and let each other know of any new symptoms.

Olga, Fernett (sorry if I misspell) and Amanda thanks for your words. I have made a promise to my dad. He asked me once what I wanted to do in regard to the progression of the disease. I told him, that day, that I would fight this no matter what. I will give it my all and hopefully in the end 10 years from now I will still be here.

Thanks everyone for the Replies;
Mario Ernesto Arevalo Melendez

Dear Mario,
Thank you for your thoughtful update and kind responses to everyone's entries. I am grateful that you have caring family to stay with in the San Francisco area during your Clinical Trial visits because family love and support is so very important in this challenging battle. Brittany also experienced increased fatigue when she was taking the ARQ-197, and it seems to be a common side effect of the medication so just give yourself permission to try to get as much rest as possible to help strengthen your body to better fight the disease. My special caring thoughts and best wishes will be with you for good news results from your upcoming November 9th CT scans. In the meantime, please take care, keep your wonderful positive attitude and strong fighting spirit, and tell Dr. DuBois "HELLO " from the Hess Family,
With special caring thoughts, healing wishes, and continued Hope,
Bonni

Mario,
Just an update on our son and Arq, Last 2mos scan showed stability. He does get alittle
more tired compared to kids his same age esp. with sports but that does not stop
him. We are thrilled this drug has allowed him to stay in school
and we try to keep things as "normal" as possible between the monthly
dr. visits. All bloodwork so far has been normal too. Prayer also helps
Peace and blessings,
cindy

Dear Cindy,
I am so happy that your son's most recent two month ARQ-197 status scans showed continued disease stability, normal bloodwork, and that he seems to be tolerating the medication so well. My special thoughts, best wishes, and prayers will be with him and your family for continued treatment success. Would it be possible for you to post these and future treatment results/informaton in the ARQ-197 topic on this Board so that patients who are following ARQ-197 for possible treatment consideration can access the information there? Thank you again for your faithful updates, shared treatment results information, and caring support and encouragement of other ASPS patients on this Board which is so vitally important to everyone in this challenging battle. Take care and enjoy a most beautiful Thanksgiving season blessed with the special joy and strengthened Hope of your dear son's encouraging scan results.
With special caring thoughts and continued Hope,
Bonni

Hello everyone:

On November 9th, I had my latest check up. Sadly, though one of my tumors in my lungs has actually grow more than 20%; because of this I am no longer part of the ARQ197 trial.

It seems that ARQ197 was able to work for my smaller tumors (size from 3 to 7mm). We saw shrinkage on them, but the bigger one had no response to the drug. I am now looking into other trials. If anyone has any information that they can share that would be awesome.

It's tough right now for my family who had so much hope on this drug. Still, though we must be strong and find a solution to the problem. Please, if you have any input that could help us make our decision on whether to try for example cedinarib or sutent, let me know. We really only have two choices either surgery or we could try a new drug.

Thank you for your support and help everyone

Sincerely:

Mario Ernesto Arevalo Melendez

P.S. Cindy thank you for sharing the news about your sons stability. I do hope and pray that ARQ197 is able to help your son. It is sad that I can no longer be a part of this trial but everyones body is different so I pray for your sons positive response to ARQ197.

Dear Mario,
Thank you for your thoughtful update. As I told you in my last week's e-mail response to your personal e-mail to me, I am so deeply sorry that you had an unsuccessful response to the ARQ-197. I know and understand very well how very heartbroken and disappointed you and your family are about the negative results of your treatment, since Brittany also had more than 20% growth in her lung mets when she was on the same ARQ-197 Clinical Trial a year ago. Unfortunately, ARQ-197 has not proven to be very successful for most of the ASPS patients who I am familiar with who have tried it, but as you said, everyone's body is different and one never knows if they might respond to a certain treatment medication until they try it. Regarding your request for input about pursuing either Cediranib or Sutent treatment, I personally think that if you are able to meet the eligibility requirements and to cope with the out of state travel and housing issues of participating in the only currently recruiting Cediranib Clinical Trial at NIH in Bethesda, Maryland, this might be the best treatment approach for you since Cediranib appears to have shown more success for ASPS, especially in shrinking large mets, than Sutent has. Another option might be Pazopanib which is another anti-angiogenic drug which is similar to Cediranib, and which has apparently also shown success with ASPS. It just recently received FDA approval and is now available by prescription through your oncologist, which would eliminate having to participate in a Clinical Trial. Surgery or laser surgery to remove as many of your lung mets as possible prior to beginning a systemic treatment might be a good option, but in order to qualify for the Cediranib Clinical Trial you must have active mets, so if they are all removed with surgery, you would be ineligible for this Clinical Trial. Possibly, if you go forward with surgery and have all visible mets resected, you would not develop anymore mets, but given the metastatic nature of this disease, that is not very likely so if new mets occur you would need either more surgery to remove them (if they are in a resectable area), or a systemic treatment to try to stabilize the progression of the disease and shrink the new tumors. I know that this is a difficult decision Mario, and I wish that I could tell you the best thing to do, but since there are unfortunately no definitive answers with this unpredictable disease, you will need to base your decision on researched information, the advice of your oncologist, and which treatment best meets your personal situation and needs. My best wishes and special thoughts are with you and your family, and I am here to try to answer any questions which I am able to. Please take care, continue to stay strong, and keep this Board updated as you are able.
With special caring thoughts and continued Hope,
Bonni

Hello Everyone!!!!!!!!!!!!!!

Merry Christmas to all!!!( Sorry I know is a little bit late)

I am still waiting a response from the NIH center in Bethesda. Dr. Spunt gave me the choices that I had, and we made the decision to go to Bethesda. I do hope that I am accepted.

I hope everyone had a good Christmas and Hope you all have a Good new Years.

Sincerely
Mario Ernesto Arevalo Melendez

p.s.
Thanks for the info Bonni, I am sure I will have tons of questions once they accept me and I start to take the drug. I am hoping that I will be able to work and be on Cediranib at the same time.

Dear Mario,
It was so good to hear from you . Thank you for your update and for your good wishes for everyone. I am glad that you and your oncologist have now made a decision about the best treatment to pursue at this time, and that you have applied for admission to the NIH Cediranib Clinical Trial. Hopefully you will receive a positive response from NIH very soon of your acceptance into the Trial so that you can move forward with this promising new treatment as soon as possible. Please keep this Board updated as you are able. In the meantime, please take care, enjoy the rest of the Holidays, and have a most happy and Hopefilled start to the New Year.
With Happy Holiday best wishes, special caring thoughts, and continued Hope,
Bonni

Dear everyone:

I have spoken to my oncologist at St. Judes and have also taken into consideration what my surgeon has said. For now, I will wait and have a check up done on May the 1st. I am giving myself a 3 month break from Cediranib. The last time I took Cediranib was the last day of January, at that time the growth or progression was very minimal. Because of this, I would like to see what would be the outcome of having just a watchful eye for at least three months after Cediranib. I have read of a case that was stable 2 years after taking Cediranib with no rebound effect. Could that be the case with me? time will tell I guess.

I wasn't able to find studies on the rebound effect on Drugs like Cediranib, the only study I was able to find was done with Avastin. I will post a link here http://forum.tnbcfoundation.org/topic7564&OB=ASC.html.

I do understand that the rebound effect has happen but has yet to be proven whether its related to the drug or just the outcome of the disease. Logical thinking tells you that there should be a rebound effect. There are just so many "unknowns" with ASPS.

Also, does anyone know anything about this drug http://www.aveopharma.com/product_candi ... ib_av-951/ , it seem like a good choice since toxicity is very low and has vegf 1,2,3.

I will try to keep you all updated with my results on May, I am hoping to give you all good news. If those scans then seem to be stable I will go for another three months and then start a systematic treatment.

My goal is to go to Germany and see Dr. rolle and have him do both lungs since he said he could "clean both lungs completely".

I am praying and holding tight. We will see hopefully stable scans on May and then we will go day by day. I am looking forward to spring break and taking some vacations, yes yes yes sun and the beach all the way.

Thanks to everyone who is always watching out for me and I will try my best to keep you all updated, I am still computerless but hopefully soon I will have my gaming laptop ready from Clevo.

Sincerely;

Mario E. Arevalo