Ivan rocking it since 2003

[Olga]

Starting Dec 2017, Pembrolizumab (Keytruda) related updates will be summarized here also - http://www.cureasps.org/forum/viewtopic.php?f=76&t=1506

Hi, for the people who doesn't know - I am Ivan's mother Olga, he is an ASPS patient - born 1986, Dx Nov.2003, small tumor (18 mm) in the right arm, no mets at the presentation. The primary was probably there since end of 2002 or beginning of 2003. Resected by a general surgeon, then a second surgery to get a negative margins by an oncologist surgeon. He removed 2 muscles and reattached the tendons to preserve the full range of movement, excellent outcome from the point of the functionality.

That was not the main problem though, 4 month later a few first lung mets were found, lungs were kept under the observation and in 6 month by the summer 2004 Ivan had about 20 visible small 2-5 mm bilateral lung mets. Our thoracic surgeon has stated that there is nothing he can do as the resection would involve loss of the significant volume of the lung tissue. After an extensive search for the second opinion we have found an advanced surgeon from Germany Dr.Rolle who uses a different technique (laser assisted resection) which allows him to save most of the lung tissue. He accepted Ivan as a prospective patient and kept him under the observation reviewing CT scans which we sent to him every 3 month. Finally in the end of the 2004 Dr.Rolle decided it is a time to have the first round of surgeries so Ivan won't loose his chance to have a clean up surgery with minimal loss of the lungs and use the benefits of the method, so we traveled to Germany.

Ivan had the first lung operated on in Feb.2005 and the second lung in May 2005, the number of the mets was huge as on the surgery it was found that in addition to the 20 visible mets in each lung there are 70-80 of the tiny ones 0.5-1 mm grain like mets which were removed using burning mode of the laser on the spot. Now it is a year an a half after the first surgery, Ivan feels very good, there are some suspicious nodules in his lungs which are a few mm in size and stable for the last few scans. Dr.Rolle keeps Ivan under the observation and is confident that if needed the next surgeries might be performed (if the condition of the patient remains as good as now and there are no progression at the other sites). Ivan feels very good, he runs, plays volleyball, cycling, plays tennis, travels a lot, he is a full time university student. The next CT scan is in the end of the September and that will be about 3 years since Dx.

Code: Select all

Timeline			
04-2003	Lump appears	R	arm
10-2003	Primary		R	initial resection of the lump in an arm (no margins, woops operation as the sarcoma was not expected)
11-2003	Primary redo	R	redo resection in an arm - 2 adjacent muscles removed, no tumor was found in the resected specimen
02-2005	Thoracotomy,   R	lung, #1 open surgery Coswig, Germany Dr.Rolle	~80 mets
04-2005	Thoracotomy 	R	lung, #2 open surgery Vancouver, BC - pneumothorax
06-2005	Thoracotomy 	L	lung, #3 open surgery Coswig, Germany Dr.Rolle	~50 mets
05-2008	Thoracotomy 	R	lung, #4 open surgery Coswig, 3y since previous thoracotomy,  Germany Dr.Rolle 3m ~30 mets
04-2009	Thoracotomy 	L	lung, #5 open surgery Coswig, 3y 10m since previous thoracotomy, Germany Dr.Rolle ~15 mets
05-2010	Cryo Detroit 	R	lung, 2y since last open surgery in Coswig, Dr.Littrup
04-2011	Cryo Vancouver	L	lung, 2y since last open surgery in Coswig, Dr.Liu
12-2011	Cryo Vancouver	L 	lung, 2y 8m since last open surgery in Coswig (later failed and needed to be redone in Detroit), Dr.Liu
05-2012	Cryo Vancouver	L	lung, 3y 2m since last open surgery in Coswig, Dr.Liu
12-2012	Cryo Detroit	L	lung, 3y 9m since last open surgery in Coswig (redo of cryo 12-2011), Dr.Littrup
04-2013	Cryo Detroit	LR	lung, 4y, 4y 11m since last open surgery in Coswig (1xL, 3xR), Dr.Littrup
06-2013	Brain resection	L	craniotomy and parietal lobe tumor resection - 8mm
08-2013	Cryo Detroit	R	adrenal gland, Dr.Littrup
08-2014	Cryo Detroit	R	IVC ablation for the previously ablated area - regional adrenal recurrence, detroit - 3cm, Dr.Littrup
05-2015    Cryo Providence L      adrenal gland, Dr.Littrup
06-2015    Cryo Providence R      lung, 6y since the last open surgery in Coswig, centrally located 2 mets, Dr.Littrup
12-2015    Cryo Vancouver	L	lung, 2 peripheral 1 cm mets, Dr.Liu
08-2016 Pancreatectomy     L      tail of pancreas, retroperitoneoscopic pancreatectomy with the spleen preservation, 25mm, Dr.Walz, Essen, Germany
02-2017    Cryo Vancouver   R      lung, 3 peripheral 1 cm mets, Dr.Liu
Nov. 2017 2 new pancreatic and 2 new heart metastases found.
Dec.2017 Keytruda started with the great response soon, less than 2 months
Feb.2020 Keytruda stopped voluntarily, due to autoimmune side effects affecting adrenals and mouth. Disease is stable with most metastases resolved or looking like scars on the scans 
May 2020 - no treatment, no changes on the scans, feels good, adrenal insufficiency side effect after Keytruda.

July 2022 - no treatment was needed in 2 years, no changes on the scans - some residual scars that are stable but are being watched. Continued adrenal failure, full hormone replacement. Good QOL regardless.

[Olga]

Hi People, Ivan's lungs CT scan was announced today and it is stable! It's about 3 years since he was Dx. 2 surgeries to remove primary, 3 lungs surgeries (2 for the laser assisted resection of the multiple lung mets right and left side separately and one for the complication - a pneumothorax) and he is doing great at the moment. He runs, plays all kind of sports, travels a lot. No other treatments done. He eats healthy, little sugar (only in the fruits and dark chocolate), little fat, has been taking Omega-3 fish oil but is having a break from it. Once in a few month we send him to Kelowna where he gets heated by the infrared Heckel unit for a few hours up to 39,5-40 grad C in attempt to activate the immune system - I have no idea if it is of any value (IN RETROSPECT, probably NOT -Ivan). Next scan in the end of January 2007 (he is on 4 month scans) so we hope to have a good Fall and a Holiday season after, get to ski, fly him to some sunny destination and let him have a normal life (besides that he is taking 5 courses at the university computer science). I am going to write to Dr.Rolle right away that the result is good and we won't see him soon

[Yosef Landesman]

Hello Ivan,
Great news! Congratulations!
Good luck at school.
Yossi

[Olga]

Hi everyone,
I have a good news to report. Finally we had a meeting with the oncologist today (Feb.16, 2007) and she was happy to tell us that Ivan's lung mets continued to be stable - the lungs CT scan from Jan. 30 has shown no change from the 4 month ago scan, his abdomen is clear and the most dreaded brain MRI is good - nothing bad is found. He had no any treatment in between these scans and it was decided that he is not going to be on any treatment for the next 4 month, as you all are very much aware there is no cure exist for ASPS, only a radical management - surgeries and local ablations - in hope to find and kill any mets before they grew to be a life treat when ever it is possible. We all hope that some of the clinical trials will be a success but at the moment we have to do what we can. Ivan's general condition is very good and his small lung metastases are not causing any respiratory symptoms. I hope that at least some of these nodules are not active metastases but the necrotic tissue after Dr.Rolle's laser assisted surgery. Ivan had hundreds of the lung metastases resected during his two bilateral lung surgeries in Germany (Feb.2005 and May 2005). It was feasible thanks to the very small size of most of the mets and he still has some number of the lung mets showing on the CT scan (do not ask me how many as I do not know at this point). His lungs condition is improving, surgical scars are dissolving and it makes the next lung surgery more real if it will be needed. Dr.Rolle keeps watching over Ivan and we stay in the close contact. We are planning to ask our oncologist about the adding of the bone scan to the usual lungs/abdomen CT next time.

[Yosef Landesman]

Dear Olga,
Very good. Very encouraging to know that the laser surgery is a good treatment option to choose.
If all looks so good, why are you on a 4 months CT scan schedule? I always tend to think that less exposure to frequent radiation is better.
Please give my Best Wishes to Ivan.
Yossi

[Olga]

Yossi, there are some number of the undetermined nodules in both Ivan's lungs. A few bigger of them (up to 5 mm) I expect to be the deep seated necrotic tissue form the laser ablation as they were found at the same size the very next scan after Ivan's lung surgery (the nodules of that size are not likely to be missed by the surgeon who palpates all of the lung tissue through) but there are also some number of the small 1-2 mm nodules which might be new mets - the ones which have been less then 0.5 mm at the time of the surgery and could have been missed easily. The goal of the scans is to closely monitor lung mets condition as from what I see in other people cases the slow growth of the ASPS mets is not an uniform fact, the growth is unpredictable and may be faster or slower at times so I do not want to miss the time when the second set of surgeries has to be done. Dr.Rolle said that the size has to be bigger then 10 mm for the second round as it was easier to find the small ones on the first surgery then now when there is a scarring but on the other hand they do not have to be bigger then optimal - so if there is a growth I am to send him a new CD to compare -which I so gladly do not have to do yet! Also when the chest CT scan is done they examine the appearance of the most of the liver, pancreas, kidneys and spine/ribs so if there is something they tell. We have already had a few false positive finding in that part which have been followed and checked on the next scans. I would be glad to move Ivan to the 6 month schedule but do not feel it is safe enough, I have seen cases when people's lung mets on the CT scans were found first resectable and then unresectable with only 5-6 month interval in between.

[Olga]

Hi folks, I just thought that I'd update on how Ivan is doing while he is away and hopefully will miss this post:(
He is still enjoying the stable condition of his residual lung mets (or whatever is getting seen on the lungs CT scan), his last scan at the end of Jan. was stable no growth and we hope that at least some of those nodules are necrotic tissue after the laser surgery, also brain MRI was done and is clear. We are now on the once in 4 month lungs CT scan. His is definitely enjoying the feeling being fit and his biggest recent achievement was his participation in the Annual 2007 Vancouver Sun Run - it is a 10 km community race when roads crossing our downtown is getting closed and 55,000 crazy people pay their own money to run without any particular goal but just to make a statement that it is a good thing to do and to feel good being able to run 10 km. He was running as a member of the super team of the Canadian Lung association dedicated to everything to improve people lungs. Running is the activity which is able to greatly improve the condition of the lungs. We are so glad that he was able to run and improve his last years result by a few minutes as the memory is still very painful and fresh - of him missing this event two years ago due to the complication, pneumothorax and the surgery to fix it so he was in the hospital at the time of the run with the tube between the ribs. But he overcame it and there is not a singe day that he does some kind of sport - this winter it was a lot of skiing, running, volleyball, gym - two years after two very extended lung surgeries - laser assisted pulmonary metastasectomies done by wonderful Dr.Rolle in Germany and a third surgery to fix a complication - he is doing well. I hope that he will be willing to do a post on the front page to attract the attention of our people being after the lung surgery/going to have one that there is a need to work on the lungs to increase its capacity. He is in Punta Cana, Dominican republic at the moment, is having a break between the winter and summer terms at the university and I hope his girlfriend and an ocean keep him enough occupied to forget about this damn sarcoma for a week.

[ellen]

Dear Olga,
It is so nice to read about your son enjoying good and stable health...I am going to post a summary of my daughter Amelis's case when we return from our oncology appointment in Boston...I have not been to the site in a couple of weeks so I was cheered to read your post and to see the video of Yossi and his beautiful daughters. Ellen

[Olga]

Hi Ellen, thank you for the kind word and I hope to see a post reg. a summary of your daughter Amelie's case, I hope that she is doing ok at the moment.

[Olga]

Hi people, Ivan's chest CT scan is "perfectly stable" and he was sent away by the oncologist until the next scan - end of September or beginning of October 2007. She said - go away, have your summer and I do not want to see you until October, so that is the plan for this summer. He is so happy (me too). We have no idea why is it stable - we do not know what these nodules in his lungs are but we take it as it is. He is not on any treatment at the moment, he only had his lung surgeries and then 3 whole body hyperthermia treatments in Kelowna when he was heated up to 39.5 grad for 2 hours - last one was about a year ago. He was taking large doses Omega-3 (fish oil supplements) for a few months but we stopped about 5 month ago. He is very active, does a lot of physical activities - to be exact he doesn't have a single day without doing some kind of sport - running, biking, volleyball, skiing, gym, at least it keeps him in a good shape and a strong spirit. We all know that it most probably has nothing to do with the stabilization of the disease, Amber said at some point to me that this sarcoma plays with the people. But it improves his quality of the life greatly as there is less of the victim and more of the survivor in him this way. Have a good summer my friends as long as you can.

[Olga]

Hi everyone, I am happy to report again that Ivan's chest CT scan has shown continued stable condition, no new mets or any growth in the existent suspicious small spots after the laser assisted surgery in Germany by Dr.Rolle - he gets to see this scan also. I am sending it to him when a CD is ready - for his records and to be included in the lecture. I hope that they also checked most of his abdominal area as upper part of the abdomen is in the area visible on a chest CT incl. ribs, spine/vertebrae. His next scan will be scheduled in a 5 month interval along with the brain MRI -his yearly brain check up. Our oncologist said that "we are slowly drifting to the once in 6 month schedule". We are extremely happy, Ivan is buying the ski pass to add to his busy sport schedule - volleyball+tennis+running+biking as all of this insane rain is bringing a lot of snow to the mountains and the season is going to be terrific.

[Olga]

Hello people. This is a long overdue update on Ivan's condition. His chest CT scans were moved to the longer schedule after Sept.2007 and the scan in the end of February 2008 (5 month interval) has detected a small growth in most of the nodules with more obvious growth in a few of them from 12 mm to 17 mm. We even didn't know he had 12 mm nodule as the last time the size was mentioned it was about 8 mm and then it were all stable scans - it means that the slow growing nodules should be compared not between the consecutive scans but given more time between them, at least 6 month then you get a real picture. Once again our local thoracic surgeon had rejected an idea about the resection and our local oncologist had given us a few very weak options like IFN or to look for the ablation of the bigger mets. We contacted Dr.Rolle asking for evaluation re. possible repeated thoracotomy to resect the recurrent nodules, after he reviewed the disc with the latest scan he accepted Ivan for one more surgery, starting from the right lung, as this is the one with the bigger mets that grown. The left one is still ok - the mets are small and almost stable.
To be eligible for the surgery, lungs have to be the only active metastatic site, so Ivan had his brain MRI and fortunately there were no brain or other mets found (no bone scan was done though, just a careful review of the chest CT scan which includes most of the abdominal organs). The surgery was done on May 7 th. It was a very difficult one, the lung was not flexible but completely attached to the pleura and pleura was attached to the chest wall so it took 2 hours only to separate the lung to start working on it. Then it took 4 more hours to find and remove 39 mets - 21 minute size mets were enucleated (burned by laser on the spot) and 18 mets were resected by a laser, a few of which were more then 10 mm and half of them were centrally located. The surgery was difficult and then there were some complications with the post-op process as one of the drains did not drain properly and as a result there is still some fluid at the base of the lung now which is very worrisome for me. The strange thing is that by some reason for Ivan it was the easiest surgery, with less pain and faster functional recovery then all 3 before - two for initial resection on each lung and one to fix the pneumothorax, a complication after the first surgery. He even started some fitness training while still at the hospital and was off restricted pain killers by the time we left for Vancouver (in 2 weeks after the surgery). Now he is slowly recovering and working on resolving multiple post-surgical abnormalities in the operated lung, his volume at the current time (a month after the surgery) is already at about 4.5 L according to a breathing trainer device that we brought home from Germany.

[Olga]

This post is about the result of the chest CT scan done in the middle of September 2008. It was the first CT scan after the second pulmonary metastasectomy on the right lung (lung mets resection) that Ivan had in Coswig in May 2008 (5 month ago as I post now, on Oct.3, 2008 and 4 month and a half before of this scan on Sept.13, 2008). There are some bad news and some good news. The bad news is that there is a growth in the metastases in the not-second-time operated left lung, they compared to the scan he had in the end of Feb.2008 (about 7 month interval). The growth is 5 mm for the biggest nodule that is measured as 15 mm now. The radiologist doesn't say what was the growth in the other nodules, he just stated that there is a growth. He also noted that there are new metastases appearing on the scan which is actually could not be a case but the metastases that were not visible on the scan of the operated lung before of the surgery and became visible after they have been ablated by Dr.Rolle (not resected but burnt and left necrotic where they were). So we have some progression in the lungs and a lot of scarring and mess after the surgery that complicated the reading tremendously. The rate of growth is 5 mm in 7 month incl. the surgery, I hope it is a little slower now as the healing is completed. We sent the CD with the scan to Dr.Rolle for re-reading but he is away for the conference and will be able to read it after he is back on the week after Oct.20.
The good news is that there are no any other abnormalities found in the chest-upper abdomen area that includes the spinal column, bones, some organs in this field such as liver. I know that it is not that reliable information though as it is relatively easy to miss something on the CT scan versus MRI. Brain MRI is not done this time, it is done only once a year so far with the last one in Feb.2008 (clean). No bone scan is ever done.The oncologist would do an additional scanning if there are any symptoms so we probably should fake one.
No treatment plan is offered to us so far in regards of the slow but nevertheless constant growth on the lung metastases. There is almost no any sarcoma clinical trials open in Vancouver and they ones that are open - are not for this type of sarcoma. They announced the trial for the drug that Paul from UK was/is on (cediranib is the name here), that they were going to try it in the combination with some other drug in Vancouver but now it is removed from the clinicaltrials.gov records as according to our oncologist who was supposed to be a principal investigator and already started to enroll patients, the company does not give the drug for the study. The only trial that appears to be open is for R1507, a Recombinant Human Monoclonal Antibody to the Insulin-Like Growth Factor-1 and it was not offered to us, I should ask why. Ivan was preapproved for the IFN-alfa before of the surgery so this option remains to be open and we are thinking about it.
I know that this growth was expected as it was detected before of the surgery on the right lung so why wouldn't the rest of the mets in the left lung progress also, he was not on any treatment, but it is hard to hear anyways. Physically he is doing very good now, recovered after the surgery completely and is able to do all his sports again. On Sept.28 he with his close friend completed 30 km hike with the elevation of 1200 m on the top of the beautiful Black Tusk mountain on the outskirts of the Garibaldi glacier that is 2 hours drive North from Vancouver. He started a job in a very nice software company. Now we wait to hear what Dr.Rolle has to say after he is back.

[Fictional]

It's good to hear that Ivan is feeling so well, Olga. I am sorry to hear of the mixed news of Ivan's latest scans.

We have found that radiologist reports are very inconsistent -even when at the same hospital. I know that you are not an MD, but I can't help thinking that it would be helpful for patients to look at their own scans because they are following it much more carefully than the formal readings by radiologists.

For almost a year we have insisted on fine cut CTs for 'K' and it has helped us map the growth of her tumors in great detail. Our first baseline scan after laser was done last week - and there is quite a lot of scarring (I know she did not have the surface burn that Ivan had), but we could discern quite a bit on the scans. I will update her latest under her section when I get a chance. We are thankful for no definite new nodules for the last 5 months. She was off ARQ197 for a total of 4 weeks for her operation - but now has been back on for over 2 weeks.

For the occurrence of new tumors in the lung, I wanted to mention the possibility of ARQ197. I know you know about it of course, but with 'K''s findings that 6 small lung nodules were completely cancer-free and larger lung nodules showed evidence of partial necrosis and fibrosis, the finding was consistent with the ability of ARQ197 to prevent new tumors from developing. We just heard that 'K''s tumor blocks are being FEDEXed to UCLA this coming week. We will be down in LA the following week for our ARQ visit, so hopefully we can look at the slides with the pathologist while we're down there. I will share any additional info that we have.

ARQ has posted the following preliminary info about possible anti-metastatic action:

"Eighteen of 19 (94.7 percent) of patients treated with ARQ 197 for 12 weeks or longer did not develop detectable new metastatic lesions. Five of 7 (71.4 percent) patients treated for 7 to 12 weeks did not develop detectable new metastatic lesions. In contrast, eleven of 19 (57.9 percent) patients treated for 6 weeks or less developed new metastatic lesions. All new metastatic lesions occurred only in organs with documented pre-existing metastatic disease, except in two patients."

I had read what I could on the early actions of cmet and I have felt that it may be more likely that ARQ will help prevent new metastases from growing and spreading (effects on FAK), than inhibiting growth of existing or large lesions. The larger ones (at least by our molecular typing) may be more sensitive to anti-angiogenesis agents like sutent. Sutent did nothing for the growth of 'K''s small lung nodules.

I guess what I am saying is that Ivan could consider entering the clinical trial in San Francisco for ARQ197. The trial is very easy because the side effects are low. We also looked at R1507, but I was discouraged by how short a time people were on that drug. I think for ASPS it would be better if we could take an effective drug for a long time - sometimes cells are only vulnerable if they are dividing, and the dividing time for ASPS is relatively slow. As far as we can tell 'K' has no side effects at all on ARQ197 + celebrex + green tea capsules. Arqule said we could take the latter two...anything as long as it is not immunosuppressive. Our time commitments now are only to fly down once a month for a blood draw, doctor visit, and pick up pills. Flights from Vancouver to San Francisco are at least down to $200 range I think, it may not interrupt his job too much and it could help him quite a bit down the road. Email offlist if you have any other specific details or questions.

[Ivan]

Thanks for your post, 'F'. We'll look into it ASAP. Very encouraging results, but I am still pretty skeptical to tell you the truth.