'K' from USA - Dx 2007 at 10 years old

[Bonni Hess]

Dear 'F',
Thank you for sharing this continued very good and encouraging news. I am so happy for 'K' and your family and am very Hopeful that the Crizotonib will continue to be effective in stabilizing the growth of 'K''s lung mets and preventing new tumors. I Hope too that she will continue to tolerate the medication well and have minimal side effects, and that she will have a wonderful start to her new school year. I will be closely following this promising new drug and will be anxious to hear about the treatment results of other ASPS patients being treated with Crizotonib. Please give 'K' a high five and special hug from me. Take care.
With special caring thoughts, healing wishes for 'K', and continued Hope,
Bonni

[Fictional]

Hi everybody,

I'm sorry to have to post this - but would like to do so in case it helps someone else. 'K' is still asymptomatic and happy as usual, but we had a bad surprise last week when an ECHO (ordered for clinical trial) discovered a mass in her left atrium. The surgeons want to take it out.

Now please if you've read this far -I'd like to ask you in advance if you could NOTposting comments about how bad you feel, 'sadness', Kgal, 'heart-break' , 'shock' etc., we'd really appreciate it. We're trying to be as brave as possible and you can help if you can be brave for us too. Wish us well and say you'll be praying for us and that would be GREAT. We'd like to keep our heads as clear as possible to be helpful as we can be for Dd.

It was a bad surprise to us as the only known disease in her lungs looked like it was grinding to a halt. But that one on the left was chugging along as you remember - it was there for at least 3 years. It was missed by Jay Lee when he did conventional segmentectomy, and then by Rolle when he did Laser 1 1/2 years ago. All I can figure is that it's in a difficult place to feel and sort out from the pulmonary veins. It was clearly seen on Chest CT.

This tumor was not on top of a bronchus or on top of the hilum, or that close to the heart, so we hadn't worried about it that much. Littrup said could do cryoablation and we were planning to do that after a few more courses of Crizotinib (which we still like). Well, what that nasty tumor was doing without our knowledge was creeping along the pulmonary veins into her heart. I've since found this is not an uncommon pattern for sarcomas and other cancers in the lungs. So be careful. Maybe if tumors are 1.5 cm but within the area of the pulmonary vessels, they should be treated more aggressively with cryoablation or something else if possible - you may not see that it's directly invading, but it can also sneak into the pulmonary veins. And heart surgery is a big deal.

I will keep you posted, but I think we'll be going down to UCLA and see Fritz again and his pal Brian Reemtsen whom he described as a "rock" and "superstar". She needs to be off meds at least 2 weeks, so the earliest would be the week of Oct 10th - but I have to check Brian's schedule too.

I've been talking to fellow ASPS'er Karen Imm who had 2 heart surgeries (thankfully no return in her heart) - the last 5 years ago. She's a 10+ year survivor of ASPS and she's been wonderful giving me support.

This means we will be kicked off of Crizotinib - even though all measured lung lesions were rock stable and one shrinking - and this latest wrinkle was an incidental / accidental finding, not progression. We will try appealing the decision afterwards if we can, but first-things first. With this nasty thing in the left atrium, we're glad we opted for an anti-metastasis drug when we did.

We've also be in touch with our biotech friend who will help us molecularly profile and add new studies with this next surgery. When I have a date for surgery, I'll let you know. Kgal is strong and brave. She's great.

[Bonni Hess]

Dear 'F',
Thank you for your thoughtfulness in so graciously sharing this important information which illustrates to all of the ASPS patients and their families the importance of always being vigilant with scans, as you certainly have been, and of being aware that this disease can be very unpredictable and that mets can develop in other areas even when scans show disease stability or no increased tumor growth in the area being monitored. How large is 'K''s atrium tumor, and was an ECHO scan the only way that it could be detected? If this is so, then do you think that it would be advisable for all ASPS patients to have an ECHO in addition to their regular chest CT scans?
I am grateful that you and Brock have the experienced advice and strengthening support of Karen Imm, and the extremely well qualified, very experienced, and highly respected expertise of surgeons Fritz Eiber and Brian Reemtsen to help guide you in your treatment decision and to perform the surgery. I wish 'K' and your family the very best, am sending my most positive thoughts your way, am being brave for you and with you, and will be anxiously awaiting further updates when your time and the situation allow.
With special caring thoughts, healing wishes for 'K', and continued Hope,
Bonni

[Arch]

Dear 'F',

We are praying for 'K'.

Best wishes
Arch & Sree

[Amanda]

Hello 'F',
I am glad you found this out now and that you are being seen by such great doctors UCLA is a great place! She is yound and strong and will be back to her life i am sure alot sooner then others i know that have had heart surgery and are three times her age! I am thankful it is in an operable area also! How long are they saying recovery will be?
I had no idea that it was so dam sneaky and would go into the heart like this thank you for letting me know i have never had my heart looked at :/
I am so glad you are talking to Karin she seems like a nice and positive person
I am looking forward to the post that will say you are all home from the hospital!

Prayers of a speedy recovery healing light and hugs to 'K'!

[Fictional]

Thanks guys. Don't know if this'll be the case for her, but I've been told that kids are usually out in about 4 days. I hope that's true. They say it's not like the old days. We don't like hospitals. On the plus side too there are only single rooms and every room has a flat screen TV (renovated).

We are hoping it isn't much into the left atria. It looks like it's sticking out of the pulmonary vein. That would make resection easier - but won't know for sure until the surgeon's in there.

[Amanda]

HI 'F'
Four days is actualy about right for this now and i was also shocked!
My best friend had a rare valve condition they put in a pig valve and she was also close to an anurisim so they had to do work on that she had massive work done and they had her out in five days! I was shocked! lol I actually got mad and felt they were just pulling the insurance thing and sending her home! I was very wrong! Be ready with the body pillows it will help her be comfy after the surgery and make sure that it isnt feathers she wil hit you with the pillow if she sneezes from it... loose tops not the ones she has to pull over her head. The recovery was in some ways alot like mine i am not sure if they are opening her like they did my friend :/ the anti inflams are the most important thing for anyone with a chest surgery i have now just gone off them after almost eleven months! I cant tell you how glad i am that this was found and it is being taken care of so fast so you can all move on from this! lol at the flat screen! I still dont like hospitals and there smells but if you have to be in one UCLA is great

[Olga]

'F', I appreciate a lot that you posted this info - I know it was not easy, but information wise there is a lot to digest. Some random notes that I've taken are:
- the limitations of the scans (I knew that but not to that extend);
- ASPS ability to invade the vessels and spread along the walls in a thin layer;
- realization that there is a valid reason that ablation therapies are not recognized by the surgeons as definitive procedures - had 'K' had this met ablated by the cryo few months ago it would have been a huge mistake as by then this met already spread to the vessels and there is no chance it could be completely ablated;
-you never know what is next with the ASPS so the quality of the current life should be a priority;
-you never know what is better to do with ASPS, for example 'K' had this ECHO scan only because she was on the Phase 1 trial and they are looking into the cardiotoxicity profile, if she was on a Phase 2 trial which we usually all consider to be the better choice, she would not had this scan done. It allowed for this spread to be found early, asymptomatic is the key here, you probably already know that all surgeries that are done when asymptomatic have more chances for success.

Don't forget to tell them about exercise induced asthma that was found in Coswig, may be ask Krasler to communicate with the docs in LA re. any problems he had during the surgery, as I remember he had some situation there he told us later when we were there after you guys and they were saying how good 'K' managed after the surgery and only he mentioned that he had some problem during the surgery.
I am glad to hear that communications with Karen helped a lot, at least this goal of the board - share experiences and support each other-is functioning as intended.

[Fictional]

Thanks Amanda, Arch, Bonni, and Olga,

Yes Olga. There are definite learning points from this and I will be adding a post to the heart section. I think I'm learning a general rule that 'denial' isn't a good idea in ASPS. If you face it, you just might be able to lick it.

I tried to diagram what is happening and will attach below (I will also add to the heart mets section). I think the fact that we discovered accidentally suggests to me that others should watch out for this as a possibility. I am thinking that another friend through Sarcoma alliance (not ASPS) might have had this problem - everything look stable, then out of no where, mets to the head. I had heard that lung mets are relatively safer than other mets because the vessels in the lung are 'terminal' - the exception of course would be if the cancer travels along to pulmonary veins.

When the ECHO was ordered, Brock and I rolled our eyes - we thought it was such a stupid thing to do, waste of money etc. - but just a trials requirement, but wow glad we found it. And I have to add that this stupid chugger met on the left is the only one we thought grew on 5 months of Crizotinib (the radiologist read all as stable - but I still thought this bad boy grew a mm or so). We would like it gone.

I will also tell you I went back through our chest CTs and varied the settings to see if I could see anything out of the ordinary that would suggest this thing there - and I couldn't or at least it wasn't obvious. The ECHO picked it up and then the cardiac MRI really showed the detail. An ordinary MRI wouldn't have - you need an MRI timed to heart movement and with extra contrast dye - and you'll only get one of these if there is a high suspicion like a positive ECHO.

I have searched pulmonary vein, cancer, left atrium - and this is not an uncommon happening for mets to the lung and especially sarcoma. Now we know it can happen relatively early in the course of ASPS and its direct extension from the lung. And it is a potential risk of cryoablation. I also know that these nasty things can be successfully removed - with long term success - some patients followed up to 10 years for instance. Usually when they present, people are much sicker because it has begun to obstruct the heart.

I also don't want to worry anyone, but I think it needs to be added to the whole diverse picture of ASPS. Heart mets are apparently fairly common in patients who die from the disease (yes I know, not a good correlation), but they are also often unsuspected. If you find it, maybe you can do something about it.

Our surgeon (Dr Brian) is out of town until Monday. We decided we do not want to go to surgery until the following week because Crizotinib has a long half life and at least theoretically it could affect wound healing. We would like to ask him about resecting the left lower lobe at the same time he removes the 'thing'. Don't know if it is possible - but would be great not to contemplate 2 surgeries to do this. It is tricky though having to be on cardiac bypass and blood thinner and resecting a lung that has already been operated on (so adhesions and very vascular). I'm glad everyone says he's good.

ASPS-to-Heart.jpg (11.57 KiB) Viewed 12771 times

[Bonni Hess]

As always, you make some excellent points Olga and they are certainly very important things that all ASPS patients and their families need to be aware of. Based on 'K''s regular CT scans not showing the atrium mass, we are wondering if it would be advisable for everyone to request that an ECHO be done occassionally, and we plan to discuss this with Brittany's oncologist. This situation certainly highlights the fact that assessment of disease progression cannot be based on the results of chest CT scans alone, as we learned the hard way several years ago when Brittany's chest CT's continued to show lung met stability, but yet she had developed a liver met which was only accidentally seen because the chest CT extended to the top of her liver. Then, based on the disease progression to her liver, we requested complete scans including a brain MRI which devastatingly showed two brain mets. Continued vigilance with scans, being alert to any and all symptoms, and being as informed and pro-active as possible is essential in fighting this very unpredictable disease, and unfortunately even then, as happened with us with Brittany's spinal tumor and now with 'K''s atrium tumor, things can be and are missed, and mistakes can be and are made with accurately assessing the status of the disease.
Our deepest caring, most positive thoughts, and very best wishes continue to be with 'K', 'F', and her family as they move forward in seeking and obtaining successful treatment for this new tumor.
With special caring thoughts and continued Hope,
Bonni

[Bonni Hess]

Dear 'F',
You and I must have been posting at the same time since I just now saw your post after already submitting mine. Thank you once more for your very gracious sharing of this very signifcant and extremely important information and for your valued insights in the midst of everything that you are dealing with right now and how very busy you are with coordinating doctor appointments, researching information, etc. The information which you have provided may save another ASPS patient's Life through early diagnosis. As you have indicated and should be noted by everyone reading this, none of us can be in denial about the unpredictable nature of this disease, and unfortunately we don't have the luxury of taking a vacation from it because we never know when or where it will make an appearance. I have always equated ASPS with the Devil because it seems to play cruel and evil tricks just when we think that everything is stable. I have never understood why most oncologists continue to only follow the disease through chest CT scans alone, when they should know that ASPS can metastasize through the blood stream to almost any area of the body. We were completely shocked when Brittany was diagnosed with her breast met and then again with her pancreatic met because it is quite rare for ASPS to metastasize to these areas, but obviously it can and it does.
I am so grateful that 'K''s atrium mass was found early before it became symptomatic, and that you have the comfort and reassurance of knowing through your research and your communication with Karen Imm that heart mets can be successfully removed with long term success. I know that you are facing some important decisions in the coming week, and my very best wishes and most caring thoughts continue to be with you and your family.
Heart to heart with deepest caring, healing wishes for 'K', and continued Hope,
Bonni

[Amanda]

Hello 'F',
thank you, very much for posting this and i am going to ask to have this test done when i scan next and on a yearly basis with the brain CT ..
I hope that will be a good time frame.
I am so glad this was found and early what a blessing!

[Olga]

'F', why are you going to ask for a left low lobe resection during the same surgery? As I remember there is only that one met that was growing? There is a technique of the intraoperative ablation use that might be tried to save that lobe, as I understand Dr.Suh works at the same institution? My concern is the ability of the remaining lobe to expand to fill all that space after being already operated on twice, did 'K' have a lot of resection on the upper lobe?

[Fictional]

Thanks again everybody,

Olga, thanks too for the reminder about exercise-induced asthma. We started her back on Pulmicort and Albuterol (she had basically stopped using her inhalers unless I reminded her) in prep for surgery. She also had an orthodontic appliance that we had removed in prep for surgery (she is happy about this - but she might have to have it put back in at some point).

We are asking about intraoperative cryoablation with Robert Suh. Before her cardiac MRI, Fritz said - just do the heart thing because the lung would have a great deal of adhesions from prior surgery (would have to be completely freed0 and she would have to be on bypass and heparinized. Not a simple job, but now we know the tumor in the lung is connected by the pulmonary vein to the heart. The lower inferior pulmonary vein (LIPV) looks nearly occluded.

I have read about surgeries in which just the pulmonary vein was resected and any involvement of the heart if seen (especially as it is in the left atrium) - with the stump just tied off... and others in which it was combined with a pulmonary resection...usually lobectomy. The way they do it is they do the heart first and then they reverse the heparinization and do the lung. I think Karen had a combined procedure for one of her heart surgeries. Dd's poor little left lung has had left anterior and lingular segmentectomies plus wedge resections, then Rolle's laser. Because the LIPV is totally occluded, I don't know how much air is getting exchanged in the lower lobe anyway. I know all this doesn't sound good, but at least I know she has been running 1.5 miles per day (in addition to rowing machine and bicycle). I also don't know if that left lower lobe is exchanging much air now because it is extending tumor into the LIPV - also has clogged all the little veins heading to the LIPV with tumor. Some of that might be able to be killed off with cryo though. If we could save breathable lung, it would be worth it. Also it is possible that some of the clogging was worse before she was heparinized. If cryoablation - I would think the lung would not have to be completely freed.

p.s. Bonni, I have thought about this cancer as work of the devil as well. Evil thing. Maybe if anyone with ASPS is considering ablation around the hilum, they should have an ECHO checked first. We could do a case report and it might help other people in the future. One study I read said that when mets to the heart were looked for in sarcoma in a large path series it was 20%! (and they suggested it contributed significantly to morbidity and mortality) Well, wouldn't it be nice if doctors looked before their patients died...especially if something could be done about it? Especially as the ECHO is non-invasive, slap some gel on and look inside.

[cachabamba10]

Dear 'F':

I am praying for you and your whole family. Also,thank you for sharing this information on the board. Keep us updated as you are able 'F'.

Sincerely;

Mario E. Arevalo