Cure Alveolar Soft Part Sarcoma International (iCureASPS)

First, i would like to apologize as I'm not a native English speaker. So please be easy on my english.. ^^

Im Rheza, 27 years old, an Indonesian. I was diagnosed ASPS a month ago. Here's how the story goes.

I was an ice skating coach during 2002 - 2008, on ice almost everyday and gotten countless falls mostly on my calf. Middle of 2007 i notice my right calf hurts and swollen. I thought it was normal and went to masseur for several times. The treatment shows no progress but as I'm too ignorant I just left it as it was. After a year my right calf is noticeably 1.5 bigger than my left, and started to hurt. So I went to a general doctor and was referenced to a Vascular Specialist. After Ct scan on my leg, the doctor diagnosed that I have AVF (Arteriovenous Fistule) and recommend coiling them. During the consultation phase, I noticed few negative changes on my bodies. First I have a small lump on my scalp (went to neurosurgeon and was diagnosed as lipoma), second I have bad cough, third my left shoulder starts to hurt when moving, and fourth after the 1st lump getting bigger, I had 2nd lump on my scalp (both of the lumps was diagnosed as an AVF too.

On March 2010. I had a leg surgery, the doctor still diagnosed it as AVF and did the coiling procedure.

On April 2010, the smaller lump on my was removed. The tumor went to biopsi and diagnosed as ASPS. After recovering from surgery I went for body CT, and founded 3 more tumors on my lung, left shoulder and right shoulder.

On May 2010, The bigger lump on my scalp is removed and here I am clueless on my next step as I doubted the doctors here. my left and right arms hurts so badly now that I have to take painkillers every day.

Can the forum please give me guideline on what I should do next step by step? Should I concentrate on my calf? (as it was the main tumor), or my arms (as it hurt so bad), or my lung? (as it's the most important body part)

Many thanks in advance. I'm thankful i found this forum. Sorry for my bad English..

Regard,
Rheza

Hi Rheza

I think your first priority should be trying to get on a Cediranib clinical trial anywhere you can. It is currently the best known systemic treatment. It doesn't cure completely but, in many cases, it can stop or even reverse tumor growth for a prolonged period of time (years). We have had many good results with it - see more details here: http://cureasps.org/forum/viewforum.php?f=45

Currently they are no longer enrolling patients into the stage 2 trial at NCI. How is your financial situation? If (when) a new trial opens are you able to travel to the US and pay for your stay there? It's also possible that Cediranib will be approved quite soon and become available.

1) Have they now confirmed that the issue with your calf is ASPS? What was done in the surgery exactly? It's possible that this is an unrelated issue and the primary is somewhere else. I kind of doubt that the surgeon would still mistake a vascular tumor like ASPS to some kind of a vein issue AFTER performing the surgery.

2) It's kind of unusual to have symmetric metastases (right + left shoulder + arm). Possible, but unusual. Bone metastases are painful, so it could be. Has everything else been ruled out?

3) How big are your lung mets? It might be a good idea to ablate them (RFA or Cryo) if there are not too many, and they are not too big.

We are happy to have you join us.

Thank for the quick reply Ivan

The Cediranib solution seems promising but I think my financial situation won't support me to go to US. Hopefully it will be available soon and I will definitely try your advice.

1) The surgery procedure on my calf was a catheter procedure. So it was non invasive. They use guiding wire to plant a coil. I haven't confirmed if it's an ASPS, so i think this would be my next step. (If it's an ASPS what should I do?)

2) I'm terribly sorry on this one. I was mistaken. It's my left and right shoulders not arms. Both are bone metastases (caput humerus). Currently I'm unable to move my left arm and suffering greatly when it comes to sleeping. I really wish to end this uncomfortable situation as soon as possible.

*additional information : According to 4th May 2010 CT, my left shoulder have a 8 x 5.5 x 6 cm tumor, and my right shoulder have a 6.5 x 3.5 x 2.5 cm tumor.

3) The 4th May CT only stated that I had multiple mets with the size of 3 - 27 mm. There's 1 crucial thing I forgot to tell. When I'm at the hospital getting ready for my bigger scalp tumor removal, I had a massive bleeding on my lung (coughing blood) it went for 1 day and lost approximately 100 cc of blood. I think this can describe how bad my lung condition is.

Thanks in advance. Sorry if I annoyed You with many questions.

Have you had CT scans of your kidneys, or any problems with them?

Check this out: cediranib trial apparently has room http://clinicaltrials.gov/ct2/show/NCT0 ... pen&rank=5. If you make it down there you do NOT need to pay for drugs, scans, or accomodation. The trial covers that. You only need to pay for TRAVEL and FOOD. You can also try to contact the makers of cediranib directly - http://www.astrazeneca.com/about-us/contact-us and ask them if they have any trial for ASPS, solid tumors, or any Phase 1 trial, in Asia which you could join. Find out about it ASAP.

If you cannot access cediranib, I think your next best option is sunitinib, which is the same class of drugs. Sunitinib is one of the prescribed drugs for similar types of tumors to ASPS, and has been used successfully for ASPS. It's been approved, so it should be more easily accessible. For ASPS it's not as effective as cediranib but we have had some success with it. It should also be available to ASPS patients under "off label use policy". This drug is available from Pfizer under the name "Sutent". Find out ASAP whether it's available in Indonesia.

How can the bone mets be so large in the humerus? If it's a bone met, that's bigger than the bone diameter. If it's a soft tissue met, it's a whole different question.

You could use http://en.wikipedia.org/wiki/Embolization or radiation therapy on your shoulder mets (your oncologist should know that anyhow) to provide some temporary pain relief immediately before systemic treatment can hopefully take effect.

Your calf - does it cause you a lot of pain right now?

2.7 cm in the lungs is becoming too large. Sometimes, the met growing can rupture small blood vessels - hence your bleeding. The small blood vessel then healed so you are OK. Anything above 1 cm you should be able to ablate, for example RFA ablation. Find out of this is offered, and consult with your doctors whether they could ablate the biggest mets in your lungs. This needs to be done ASAP.

Wow.. Your reply is such a relief. I feel much confident fighting this disease.

I've discuss with my family and I think I'm able to go US financially when it comes to only Travel and Food costs. I'll definitely go with your suggestion. As for Sutent, I have access on the medicine as my brother works for Pfizer.

About my shoulder, me myself not sure whether it's a bone metastase or not. The scan shows that I have a big tumor surrounding humerus and I can see my bone is destructed. What do you think? I can send you pic if needed.

I will consult with my doctor for embolization or radiation therapy. Greatly thanks for the advice!

My calf causes pain when in use. I'm unable to walk long distance. It also has big and numerous varices. Hope this information helps.

I will consult with my doctor ASAP about the ablation too.

Forget to tell you that I had kidney CT and it's clean.

Since this has become more a treatment thread rather than introduction I will soon move it to the "Personal Updates" section.

I've discuss with my family and I think I'm able to go US financially when it comes to only Travel and Food costs. I'll definitely go with your suggestion.

Monday is a holiday in the US. I would start calling NCI at 1-888-NCI-1937 on Tuesday morning Bethesda, Maryland time 8AM regarding enrollment in clinical study NCT00942877 ASAP. This would be the absolute best option for you. Contacting them is the most important thing you can possibly do right now.

As for Sutent, I have access on the medicine as my brother works for Pfizer.

Well that's an amazing coincidence. Find out right now (in parallel with pursuing the Cediranib trial) how fast you can access that medication. Sometimes it takes a while to set everything up, etc. So you need to have this option ready if you can't get in to the NCI cediranib trial. This is also a pretty good systemic option.

About my shoulder, me myself not sure whether it's a bone metastase or not. The scan shows that I have a big tumor surrounding humerus and I can see my bone is destructed. What do you think? I can send you pic if needed.

What did the doctors tell you it is? ASPS usually does not invade neighboring tissues but rather pushes it, because it grows so slowly. Maybe it's a soft tissue tumor, which by its growth, has crushed the humerus? Also when did you first start having shoulder pain?

My calf causes pain when in use. I'm unable to walk long distance. It also has big and numerous varices. Hope this information helps.

What does your oncologist suggest as the treatment for your calf? I guess you've already had a CT or MRI of your leg. How big is the suspected primary there? Sounds like because it's so big it's also pushing on veins or arteries there, and causing secondary issues. Again, the hope is that systemic treatment would shrink it. After that happens you would want to resect it, of course, but it's possibly too big for that right now.

I will consult with my doctor ASAP about the ablation too.

So it sounds like your doctors are at least WILLING to perform surgical treatment on you, if you request it? We here have had many instances where they don't want to and people have to fight with them to get them to do it.

Ivan wrote:Monday is a holiday in the US. I would start calling NCI at 1-888-NCI-1937 on Tuesday morning Bethesda, Maryland time 8AM regarding enrollment in clinical study NCT00942877 ASAP. This would be the absolute best option for you. Contacting them is the most important thing you can possibly do right now.

I've emailed both NCI and AstraZeneca yesterday. Will do you advice though.

Ivan wrote:Well that's an amazing coincidence. Find out right now (in parallel with pursuing the Cediranib trial) how fast you can access that medication. Sometimes it takes a while to set everything up, etc. So you need to have this option ready if you can't get in to the NCI cediranib trial. This is also a pretty good systemic option.

Yes, He even can get it a discounted price. If somehow I take sutent as my systematic treatment, should I still consult my oncologist? Or just take it anyway?

Ivan wrote:What did the doctors tell you it is? ASPS usually does not invade neighboring tissues but rather pushes it, because it grows so slowly. Maybe it's a soft tissue tumor, which by its growth, has crushed the humerus? Also when did you first start having shoulder pain?

My doctor said it was a AVF bone hemangioma (before the ASPS diagnose). I think you're right about the bone destruction is caused by the soft tissue growth. I start having left shoulder pain since December 2009, now it's immobile and painful even when moved around by other people. My right shoulder started to be painful since April 2010, It causes pain when I pick heavy stuff and getting worse now.

Ivan wrote:What does your oncologist suggest as the treatment for your calf? I guess you've already had a CT or MRI of your leg. How big is the suspected primary there? Sounds like because it's so big it's also pushing on veins or arteries there, and causing secondary issues. Again, the hope is that systemic treatment would shrink it. After that happens you would want to resect it, of course, but it's possibly too big for that right now.

Suspected primary is big u can check this pic for reference
http://sphotos.ak.fbcdn.net/hphotos-ak- ... 0454_n.jpg
My doctor said that resection is not an option and because it may causes me to unable to walk. I haven't consult with oncologist yet, because somehow I doubt the doctors here (I live in a small city in Indonesia). I might go Singapore to find better oncologist. Btw, how important is it to resect primary tumor? Is it so important that I should consider amputation as an option?

Ivan wrote:So it sounds like your doctors are at least WILLING to perform surgical treatment on you, if you request it? We here have had many instances where they don't want to and people have to fight with them to get them to do it.

I've consulted with my doctor and she said that her plan is to remove my lower left lung as the bigger mets are concentrated on that area. Is it a good option?

Thanks in advance Ivan!

Rheza, have you been able to get in touch with NCI?

Not yet.. After discussing again with my family, I think I'm unable to go US. I forget that I need a companion as I can't travel alone due to my inability to move my hand. I don't have a companion that can accompany me too long...

Btw, Yesterday I went to Jakarta (Indonesia's Capital City) went to Dharmais National Cancer Hospital for oncologist appointment. And the oncologist was clueless. She ask me to return there next week and I will be diagnosed and discussed with a group of doctors and radiologist. This prove that even the best cancer hospital in Indonesia is unfamiliar with ASPS.

So... I think my next move is Sutent.

What do you suggest?

Rheza - hi, it is normal for the institution to have a tumor board to discuss the complicated cases, and your case is not the easy one. The problem is not with the ASPS itself but with the situation overall. Did you tell them that you have an access to Sutent (sunitinib is the general name of the drug and it might even have different marketing name in Indonesia). I think that the most important thing for you now is to look for the hands situation relief - you should ask them if they can do isolated limb perfusion, embolization, soft tissue cryoablation - anything that could potentially downsize the tumor and allow for its resection to make your life better if you regain the use of the hand, because the tumor can be still in the soft tissue outside of the bone and it is destructed from the pressure outside, it may recover.

Rheza - ask them at the Dharmais National Cancer Hospital if they have any clinical trials with the tyrosine kinase inhibitors open - they are the drugs of the same class like sunitinib but some of them are more potent - they can even have cediranib on a trial or pazopanib there. I do not know if this hospital adds its participation in the trials to the general clinical trials data base (it is on clinicaltrials.gov but not all the countries add their trials there), so may be better to ask them directly.

Hi Olga. Thanks for replying!
I haven't told the Dharmais Doctor (http://www.dharmais.co.id/index.php/all-home-en.html) that I have access to Sunitinib yet, as during the appointment, the doctor seemed to be in a hurry. She asked me to go back there next Wednesday. I will surely ask them about the treatment you mentioned (limb perfusion, embolization, soft tissue cryoablation). I'll update as soon as I get the info.

You mention about downsizing the tumor for resection. Some doctor here told me that resection is never recommended. Is this true? Or is there any particular guideline for ASPS tumor resection that I need to tell them?

I'll ask them about the clinical trial for more potent treatment. Thanks for this valuable information.

Hi Rheza
By my best knowledge, when it comes to ASPS, its the best thing to go with operative removal of tumors. Since they havnt discussed your case yet, i think its odd they say, its not the way to go.

Radiation rarely works on ASP, and not all have good results with chemo... but tbh im o way near the expert on this, so ill let other people do the talking .

I was just a bit thrown of with them saying resections sint the way...

May be this article from our library might be of help:
Clinical presentation, treatment, and outcome of alveolar
soft part sarcoma in children, adolescents,
and young adults
http://cureasps.org/library/6_ASPS_MSKCC_06.pdf
They say:
"Metastasectomies have been performed in multiple long-term survivors."