Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi my name is Sheree and my husband Mick 2 nearly 3 years ago was diagnosed with asps. It was in his thigh they tired radiation for 5 weeks everyday and it done nothing they were hoping it would shrink but we had no luck. He had it removed in august 2005 then on a follow up scan they notice that it had spread to his lungs.
Since then til December 2006 they have stayed at the one size most of the ruffly 10mm but then in march 07 the lumps have started to grow and then on his last check up 3 months ago the lumps are now 35, 34,25 29mm the two biggest ones are on his airways and they are 35 and 34 mm and he has now developed a cough which is slowly getting worse.
He has trouble now running around with the kids he has trouble doing anything phisycal.
He has another check up on the 15th of October and i hope it good but my husband said he can now feel himself getting worse and to be honest i have notice it. He is coughing alot more getting breathless easily.
Im just after some info on what others have been through and how they have been treated to stop it from growing.
He now goes to the Mater Hospital at Newcastle in Australia...

Dear Sheree,
I am so deeply sorry for your husband's ASPS diagnosis and heartbreaking disease progression, but I am grateful that you found your way to our Web site and that you have reached out for information and support. Has he ever received any kind of treatment to try to remove, destroy, or shrink his lung mets? Unfortunately, the lung tumors are now probably too large to be successfully treated with Cryoablation or Radiofrequency Ablation, but they may be able to be treated with one of the Stereotactic Radiosurgery techniques that are described on the Home page of this Web site if you continue to scroll down until you come to the topic. Olga can probably provide information on the laser treatment which was successfully performed on her son and another ASPS patient in Germany, but again your husband's mets may now be too large for that treatment also. Paul Mavers recently made an entry on the "Other Clinical Trials" thread of this Forum regarding a new chemo treatment called AZD2171 which has apparently shown some promise in possibly slowing, stopping, reversing the growth of ASPS mets, and this might be worth you obtaining more information about. Have your husband's doctors made any treatment recommendations? Please keep us updated as you are able, and know that our special caring thoughts and prayers will be with you.
Reaching out to share special caring and continued Hope,
Bonni Hess, mother of ASPS patient Brittany

Hi, the size of these mets is not to big for a ablation - just about the critical value after which these modalities can not be used, but there are quite a few of them and they all big so it is not safe to use any radiation or ablations due to the mass effect - may be only as a palliative measure for the ones close to the airways. I do not understand why the old fashion thoracic resection was not offered/used earlier and it is certainly not late to do it right now, it will provide an immediate relief for your husband. Most of the ASPS patients have one or a few lung resection to surgically remove lung metastases and it is a recognized treatment for the ASPS lung mets. Have you met with the thoracic surgeon yet? If lung mets are the only metastatic site and they are technically resectable then this option has to be used. We have no knowledge about any chemotherapy option available for the patients with the bulky/unresectable lung metastases, in this setting all the attempts to use chemotherapy were unsuccessful. We have a few cases when cytotoxic chemotherapy was used before/after thoracic surgery with the success but not chemotherapy ONLY. ASPS is a very chemoresistant tumor due to the slow growing nature and very impaired vascular system so there is always only a partial response to the chemo (if any at all). Newer chemotherapies targeted to cut a blood supply can not be used in this case because these tumors are bulky and there might be a heavy bleeding if these drugs happen to work good. Look for the best thoracic surgeon in Australia, they all have different approach and experience so if one tells that the surgery is not feasible it doesn't mean that you won't find the other who will be able to resect them all.

Dear Sheree,
As always, Olga's advice is excellent and well informed. I agree with her that resection of your husband's lung tumors with a thoracotomy would be the best treatment option if they are located in an area where they can be safely and successfully surgically removed, because based on the detailed information you provided, the total number of tumors is only four which is certainly few enough for resection to be a viable option. I hadn't suggested a thoracotomy in my entry because I had perhaps erroneously assumed that the mets weren't resectable since this procedure hasn't already been done. Regarding the possibility of Cryoablation or Radiofrequency ablation, we were told by Dr. Peter Littrup who performed our daughter Brittany's Cryoablation procedure on her three largest and most concerning lung mets almost three years ago, that the maximum tumor size that could be treated with Cryo is three centimeters ( thirty mm.), so perhaps your husband's two smallest 25 mm. and 29 mm. mets could still be ablated, if they aren't resectable. The location of the tumors is critical as both of the ablation procedures require an adequate margin because the extent of the freeze (Cryo) and the burn (Radiofrequency) can't be completely controlled, and this has implications if the tumor is located too close to a blood vessel or another organ. I hope that you will be able to discuss these possible options with your husband's doctors as soon as possible, and then move quickly forward with treatment to prevent any further increased growth in the size of his lung mets. Please take care Sheree, and keep the Web site updated as you are able.
With special caring thoughts and continued Hope,
Bonni

HI thanks for your thoughts.. The 3 different hospitals that we have seen they have said that there are too many lumps and at that time they were too small to operate, i only gave the measurement of the biggest ones.. i think there is about 30/40 lumps all up some are still only small nut the one that are 34/35mm are on his airways.. Recently the new hospital they seem to be the only ones to explain things to us said that they wouldnt offer him radaiton as it never worked in his leg and they havent spoken about operating but we go and see them on the 15th of this month so i am taking all this info that i have found in and hopefully they can do something.. My husband thismorning said that he is really starting to feel tired all the time even just getting out of bed. He is coughing alot more than before and well im am really worried. All the doctors have said to us is the chemo that he willhave will only buy him abit of time then i read stuff on this forum and well i dont know what to think. I dont know if the doctors here in australia are as good as the ones over in america and there is no way we could afford to come there to be treated...
Well i just hope when he has his scans on the 10th it will give us good news fingers crossed.. i will let you know what happens..
Thanks again
Sheree

Sheree, the ASPS lung mets are usually very multiple but they get resected anyways but you need to look for the best thoracic surgeon there. Also the primaries of the ASPS are very radioresistant but its metastases are less resistant and some of our patients had it treated by the radiosurgery - not the radiation therapy. I have checked and there are no Novalis or Cyber Knife ot tomotherapy units are listed in Australia (which were used for lung mets here) but still there might be advanced Linac radiosurgery avail. there. I do not know if there are any cryoablation avail. there. I would insist on the lung surgery even as a palliative measure as it most probably will prolong his life and improve his current condition at least temporarily. How old is he?

I will ask the doctors when we see them. He is 29, 27 when they found it.

Hi i thought i would just let you know that my husband got his scans back and his lumps inhis chest have growen they were 3.4cm x 2.4cm three months ago but they are now 3.9cm x 2.9cm. Is this normal for these to grow like this? Does anyone know if it is good ornot? We got to the doctors on monday the 15th so i should know more then..

One of the anti-angiogenesis medications might also be considered in his situation. There is not enough experience with these new medications and ASPS, but because these tumors have lots of blood vessels and there is evidence patients have responded to these medications. They may be more effective when the metastases are small.

Surgically, if the metastases are too small, they may be too hard to see during an operation. As a result, surgeons will often have to take out an entire segment of lung. This can't be done with diffuse disease, but there is also a possibility small ones may disappear spontaneously (maybe due to the body's successful fighting of the tumor?).

For kidney cancers (some similar features to ASPS), anti-angiogenesis factors have been able to eliminate or shrink lung metastases, and in general these medications are well tolerated (some even a pill to take daily).

We have a very different situation, but we are on a trial of Sutent to see if it can shrink the tumor or initiate a partial response to make complete resection possible. It is too early to tell, but it looks as if there has been some biological response to Sutent. Sutent has been remarkably mild re: side effects for our daughter, although there are certain times she gets tired. She takes a pill at bedtime and gets her labs drawn every other week. There is also some thought that Sutent and other drugs like it may make tumors more responsive to radiation, but it is too early to tell.

Sheree wrote:Hi i thought i would just let you know that my husband got his scans back and his lumps inhis chest have growen they were 3.4cm x 2.4cm three months ago but they are now 3.9cm x 2.9cm. Is this normal for these to grow like this? Does anyone know if it is good ornot? We got to the doctors on monday the 15th so i should know more then..

Normal? I suppose, this is a NORMAL progression of the disease, but it's not something you want to happen obviously

I can't believe you have waited that long to begin seeking treatment options. Frankly, I think your oncologist should be punched repeatedly. Do you understand the scope of the problem here? Hesitation and indecisiveness can mean certain death. Make it your full time job to find a valid treatment option, starting yesterday!

I think you have to establish as quickly as possible whether surgical resection of all the large metastases is possible or not. You have to go to a large city to see a sarcoma specialist who has experience with the disease. ASPS is so rare that it's quite possible your oncologist has never had an ASPS patient.

HI we had the doctors appointment yesturday. I asked them about any drugs that my husband could take... I even asked them about surgery but they said the biggest lump has wrapped itself around the airway so he said the only way to remove it in surgery is to have a lumps transferr and they said that my husband would have a slime chance of that due to the cancer... they said that no surgen would attempt to remove the largest one because of where it was located but the other lumps they said that they could remove them when they get bigger... they said that they will give him radiaton one the biggest one when he starts to have trouble breathing or starts coughing blood but they dont want to give him anything yet as it will take his quality of life... i took the info that i have found in to them but they really didnt say much..all they say is that they dont know to much about ASPS and the info they will read up on... My husband goes back in 3 months or sooner if he feels himself getting worse...

Dear Sheree,
I am so very sorry for the disappointing outcome of your husband's doctor appointment yesterday. I am concerned about the doctors' apparent lack of knowledge about ASPS and their not having any information on the possible treatment options which were shared with you on this Forum. I would strongly recommend that you try to find an oncologist who is at least a Sarcoma specialist. I also think that you should not just wait for your husband's symptoms to progress and worsen. I feel that his condition requires an aggressive treatment approach and that he needs to receive some kind of treatment as soon as possible to try to shrink and/or destroy the tumors, instead of waiting another three months for his next scans. The largest tumor which is wrapped around his airway, seems to be the highest priority, and since it is apparently unresectable and possibly can't be ablated due to its location, perhaps one of the Stereotactic Radiosurgery options should be considered. I don't understand the doctors wanting to wait to resect the other large lung mets until they are larger. It seems that they should be removed or ablated as soon as possible to prevent any increased growth since the larger the tumor, the more lung tissue that is lost. I urge you to seek another opinion and to be very pro-active and aggressive in trying to find a treatment option before anymore tumor growth or disease progression takes place. My continued special caring thoughts and prayers will be with your husband and all of your family Sheree. Please take care and keep the Web site updated as you are able.
With special caring and continued Hope,
Bonni

Hi Sheree,
My name is Karen. I have been fighting ASPS for 7 years now. I am so sorry for what you and your husband are going through. I know how helpless it can feel. I also wanted to share with you that I have had tumors on my trachea and around it. I had one large tumor that no surgeon would touch and yet, I finally found a local surgeon who told me we "had to do it" and he successfully removed the tumor. Then last year there was another tumor in that same area that was deemed "inoperable" because of it's location. I was told by all my doctors that I needed to "get my affairs in order" and that I only had months to live. I chose to have radiation to the inoperable tumor even though it typically doesn't work on ASPS. And, thank God, it worked. The radiation caused the tumor to shrink and it is now stable.
I say all this because I wanted you to know that you are not alone in dealing with inoperable tumors in the trachea area and because I hope that you can and will cling to hope. There is hope. And on the days when you don't feel hopeful, please know that you are being thought of and prayed for. Others will continue to hope for you.
Take Care,
Karen Imm

Hi thank you all for your thoughts. My husband has been and is very positive about the whole situation which has been good for me as i have been a mess sometime.. i am getting better though... Karen are you in australia?? I just hope at our next appointment that none of the lumps have growen it would make our day... Karen how fast did your lumps grow?? How big was the one you had radation on get to? How did the radiation make you feel?? were you sick or just tired from it? My husband had the radaition o his leg before they removed the lump abd he used to get alittle tired inthe afternoon but as i said it didnt shrink it... Thank you all again for your info, thought and prayers it means a great deal knowing there is support out there with this ASPS.
Sheree and Mick

Hi Sheree,
This is Karen again. I wanted to answer some of your questions. First, I am living in the United States. My primary tumor was in my right thigh as well and I had radiation done to the leg. It was not successful. However, when the tumor by my trachea and heart appeared, it grew pretty quickly. It was about the size of a golf ball when we tried the radiation. It was near my heart, so there was some hesitation about the radiation, but we trusted our doctor to radiate the tumor without hitting the heart.
I have had over 100 treatments of radiation total and the only side effect I have ever experienced is fatigue. Sometimes it was worse than others. It also lowered my blood counts, but we kept a close eye on that. The later effects of the radiation are what I notice. The muscles in my leg are like leather and I walk with a limp because of the radiation. I have to go to physical therapy to stretch out my torso muscles and ribs because they become very constricted from the radiation. But it is totally worth it. The radiation to the tumor on the trachea worked for me. That tumor got smaller and now is just sitting there - no growth. I believe that having radiation to that area saved my life.
The doctors told me the same thing... that I would have trouble breathing, and that I would start coughing and eventually cough up blood. However, before getting to that point, I went on my own to visit with my radiation doctor and she agreed to start radiation right away. And I would do it again.
If it were my husband, I would not want to wait for some type of treatment. I would get the radiation (or another treatment) going right away for the tumor around the trachea.
Please feel free to e-mail me privately. I would love to help in any way I can.
Keep fighting and keep hoping,
Karen Imm