2002：I was diagnosed ASPS about 4x3x2cm at left thigh in 2002. A surgery was taken to removed the tissue. A wide range excision surgery (16x10x2 cm) was taken to insure no tumor cells were left. After the 2nd surgery, the doctor also arranged me to take radiation therapy 30 times, with total 6,000 cGy at left thigh.
2014：2 lung mets(around 3~5 cm) were found at bilateral lungs. A VATs was taken to remove them.
2019：3 organ meta were found at the ER CT scan due to my chest pain.
(1) Heart：inside the heart(left ventricle), a 6.7cm x 4cm tumor were found and resected by an experienced heart surgeon in Aug 2019 (see attached picture).
(2) Pancreas：a 2.6cm tumor was found at the CT scan, planning to have a laparoscopic surgery to resect it soon.
(3) Bilateral lungs：multiple(5~10) mets were found, size up to 0.7cm, no clear plan how I can do with it yet.
=======================
Update(2019/8/20)：
I talked with my doctor, he still suggested me to resect the pancreatic tail meta (2.6cm) first before Keytruda because he is afraid if Keytruda doesn't work well for me, I will lose the chance to resect the pancreatic tail meta.
As for my lung mets, do you think I shall consider Dr.Rolle's laser-assisted surgery for my 5-10 lung mets (size up to 0.7cm) after my pancreatic tail met is resected and before I take Keytruda?
Sam from Taiwan since 2002 (now 37 years old)
Sam from Taiwan since 2002 (now 37 years old)
- Attachments
-
- ASPS_heart meta_resected
- ASPS heart meta.jpg (766.23 KiB) Viewed 4236 times
Last edited by kn0227 on Mon Aug 19, 2019 7:20 pm, edited 2 times in total.
Re: Sam from Taiwan since 2002
Sam, thank you for posting your story.
It was a very long uneventful period between your primary resection/diagnosis and the unfortunate discovery that your sarcoma has now metastasized to your lungs years. It means your ASPS is a slow growing so you should usually have enough time to react and do something with the proper scanning regimen.
The pattern of metastasis you got reminds me of my son's Ivan case - but in addition he had 1 brain met and metastases to both adrenal glands, so I really suggest you to pay more attention to the brain (MRI at least yearly) and to adrenals (CT scan or better MRI).
Every time the new problem appears, you should evaluate all the options available as every one of the have the pluses and minuses, i.e. every treatment comes at the specific cost of the damages to your body that are different depends on the location, experience of the surgeon and often just on your luck.
It is good that you have found a good quality surgeon that agreed to perform the cardiac resection, it is usually hard to find for the patient with the metastatic stage of sarcoma. I hope you are recovering well.
Lets talk about the pancreas situation. If the metastasis located in a pancreatic tail, the resection is possible but it is preferable if it is done from the back as to access to that part from the front, the surgeon has to preform a very big surgery moving all the organs out before accessing the met, so you should ask if the surgeon is going to access it from via Retroperitoneal approach, also if the surgeon is planning to use VATS (as Ivan had - the Retroperitoneoscopic pancreatectomy with Dr.Walz in Germany, you can read more here (its long):
viewtopic.php?f=81&t=1260
Also the very important thing to discuss is if the surgeon is planning to save the vessels that run along the pancreas to feed the spleen that is located at the end of pancreas like a small hat, and to save the spleen. If you are planning to have an immunotherapy later, your chances to have a response to it might be greatly reduced or lost if the spleen is removed.
So the risks of having the distal pancreatectomy is the loss of vessels and spleen.
In Ivan case the surgery was done with the vessels and spleen preservation, but unfortunately during the recovery, the feeding vein collapsed and subsequently became non-functional. The artery survived - I found later that it is not rare as the vein has less robust blood flow and scarring from the surgery can get it blocked more easily than the artery. Ivan's body somehow dealt with it and developed accessory veins to feed the spleen so now his blood supply is pretty unusual. The spleen is enlarged but functional, which is a gift.
Because his main success came from the immunotherapy treatment he had to start after more pancreatic and heart metastases were found, two years ago. His multiple metastases have almost completely regressed and I want to point out that his 2 unresectable pancreatic metastases that were found a year after the pancreatic surgery are now not visible on the scans - they say it is hard to see if there is anything left.
Overall, I would investigate the immunotherapy first. The response was very fast in Ivan's case, just in few months of treatment. Talk to the surgeon re. Ivan's case and if he is confident he can save the vessels and the spleen based on the need for you to have an immunotherapy at some point for the lung mets anyways (although you can go to Germany to Dr.Rolle successor who now work with the laser).
Keytruda is not available for ASPS in Canada, the same like in any other country - it is not approved for ASPS yet. But we have applied to insurance, they refused to pay, we wrote an appeal and they agreed . You can discuss that with your oncologist and with the Merck or Bristol Meyers representatives (they make Opdivo which is the same) in your country or look for the clinical trial for atezolizumab (another immunotherapy drug of the same type) or any of them. Sometimes they give the drug to a patient off label for free.
It was a very long uneventful period between your primary resection/diagnosis and the unfortunate discovery that your sarcoma has now metastasized to your lungs years. It means your ASPS is a slow growing so you should usually have enough time to react and do something with the proper scanning regimen.
The pattern of metastasis you got reminds me of my son's Ivan case - but in addition he had 1 brain met and metastases to both adrenal glands, so I really suggest you to pay more attention to the brain (MRI at least yearly) and to adrenals (CT scan or better MRI).
Every time the new problem appears, you should evaluate all the options available as every one of the have the pluses and minuses, i.e. every treatment comes at the specific cost of the damages to your body that are different depends on the location, experience of the surgeon and often just on your luck.
It is good that you have found a good quality surgeon that agreed to perform the cardiac resection, it is usually hard to find for the patient with the metastatic stage of sarcoma. I hope you are recovering well.
Lets talk about the pancreas situation. If the metastasis located in a pancreatic tail, the resection is possible but it is preferable if it is done from the back as to access to that part from the front, the surgeon has to preform a very big surgery moving all the organs out before accessing the met, so you should ask if the surgeon is going to access it from via Retroperitoneal approach, also if the surgeon is planning to use VATS (as Ivan had - the Retroperitoneoscopic pancreatectomy with Dr.Walz in Germany, you can read more here (its long):
viewtopic.php?f=81&t=1260
Also the very important thing to discuss is if the surgeon is planning to save the vessels that run along the pancreas to feed the spleen that is located at the end of pancreas like a small hat, and to save the spleen. If you are planning to have an immunotherapy later, your chances to have a response to it might be greatly reduced or lost if the spleen is removed.
So the risks of having the distal pancreatectomy is the loss of vessels and spleen.
In Ivan case the surgery was done with the vessels and spleen preservation, but unfortunately during the recovery, the feeding vein collapsed and subsequently became non-functional. The artery survived - I found later that it is not rare as the vein has less robust blood flow and scarring from the surgery can get it blocked more easily than the artery. Ivan's body somehow dealt with it and developed accessory veins to feed the spleen so now his blood supply is pretty unusual. The spleen is enlarged but functional, which is a gift.
Because his main success came from the immunotherapy treatment he had to start after more pancreatic and heart metastases were found, two years ago. His multiple metastases have almost completely regressed and I want to point out that his 2 unresectable pancreatic metastases that were found a year after the pancreatic surgery are now not visible on the scans - they say it is hard to see if there is anything left.
Overall, I would investigate the immunotherapy first. The response was very fast in Ivan's case, just in few months of treatment. Talk to the surgeon re. Ivan's case and if he is confident he can save the vessels and the spleen based on the need for you to have an immunotherapy at some point for the lung mets anyways (although you can go to Germany to Dr.Rolle successor who now work with the laser).
Keytruda is not available for ASPS in Canada, the same like in any other country - it is not approved for ASPS yet. But we have applied to insurance, they refused to pay, we wrote an appeal and they agreed . You can discuss that with your oncologist and with the Merck or Bristol Meyers representatives (they make Opdivo which is the same) in your country or look for the clinical trial for atezolizumab (another immunotherapy drug of the same type) or any of them. Sometimes they give the drug to a patient off label for free.
Olga
Re: Sam from Taiwan since 2002
Hi Sam
You’ve had quite the journey the last 17 years .
And as Olga has said , you have an awesome surgeon(s)? to aid you in that journey.
37 currently . You were 20 when diagnosed ?
Was the heart met sent to pathologist?
Left or right ventricle ?
Wow the growth was truly large .
My and my family’s love and prayers here and in the future .
Love,
You’ve had quite the journey the last 17 years .
And as Olga has said , you have an awesome surgeon(s)? to aid you in that journey.
37 currently . You were 20 when diagnosed ?
Was the heart met sent to pathologist?
Left or right ventricle ?
Wow the growth was truly large .
My and my family’s love and prayers here and in the future .
Love,
Debbie
Re: Sam from Taiwan since 2002 (now 37 years old)
Hi, Olga:
Thank you very much for the detailed explanation.
Now I understand why I should make this decision whether to resect the pancreatic tail tumor very carefully (needs to preserve the spleen function) since I plan to have immunotherapy for my 5-10 small lung mets afterwards.
I have a question that since my insurance wouldn't pay for Keytruda for me and the chance to change it is pretty low. So it will take a lot of money for me to have Keytruda treatment. I think I can afford Keytruda for at least two years, but I am worried about "what about it after 2 years?"
Do you have any suggestion on this? Thank you very much!
And, what are the main side effects has Ivan had in the past 2 years of having Keytruda?
Best,
Sam
Thank you very much for the detailed explanation.
Now I understand why I should make this decision whether to resect the pancreatic tail tumor very carefully (needs to preserve the spleen function) since I plan to have immunotherapy for my 5-10 small lung mets afterwards.
I have a question that since my insurance wouldn't pay for Keytruda for me and the chance to change it is pretty low. So it will take a lot of money for me to have Keytruda treatment. I think I can afford Keytruda for at least two years, but I am worried about "what about it after 2 years?"
Do you have any suggestion on this? Thank you very much!
And, what are the main side effects has Ivan had in the past 2 years of having Keytruda?
Best,
Sam
Re: Sam from Taiwan since 2002 (now 37 years old)
Sam, you should not settle for insurance not paying and you paying for Keytruda yourself - first the ASPS patients have a very strong case for the insurance appeal on a basis of the rare disease status and multiple documented cases of the excellent response to Keytruda, and second - the companies producing the drug often provide a financial assistance to the patients that have to pay all the cost without the support from insurance. But it takes persistence to figure out the contacts and rules in your country, and you need a help of at least one oncologist anyways to get it prescribed.
re. pancreatic tail surgery - even with the best surgeons, there is a chance that the spleen will not survive the surgery, you need to discuss that with the surgeon and explain it to him why do you need to keep the spleen and ask how confident he is that it is going to survive, as the immunotherapy works based on its functions (I might be wrong and the immune cells that are needed might be also produced elsewhere, need to consult with the immunologist).
If Keytruda works, it may take care of all locations, lungs and pancreas and the ones that you do not know yet about. You do not need to be on it for long, the immune system just need to get the chance to recognize the tumor and to form a tumor specific killer cells with the memory - the same like we form the virus specific immunity after the childhood vaccinations. In some people the response is very long lasting, and in some locations the mets might be gone forever. There are cases when the recurrence happens later in the locations that are easy to treat by the cryoablation and an easy surgery for example. The insurance does not usually pay for more than 2 years, all the clinical trials are limited by that. Ask re. clinical trial? to be accepted you need to have at least one bigger met, the ones in the lungs smaller than 10 mm would not qualify.
re. pancreatic tail surgery - even with the best surgeons, there is a chance that the spleen will not survive the surgery, you need to discuss that with the surgeon and explain it to him why do you need to keep the spleen and ask how confident he is that it is going to survive, as the immunotherapy works based on its functions (I might be wrong and the immune cells that are needed might be also produced elsewhere, need to consult with the immunologist).
If Keytruda works, it may take care of all locations, lungs and pancreas and the ones that you do not know yet about. You do not need to be on it for long, the immune system just need to get the chance to recognize the tumor and to form a tumor specific killer cells with the memory - the same like we form the virus specific immunity after the childhood vaccinations. In some people the response is very long lasting, and in some locations the mets might be gone forever. There are cases when the recurrence happens later in the locations that are easy to treat by the cryoablation and an easy surgery for example. The insurance does not usually pay for more than 2 years, all the clinical trials are limited by that. Ask re. clinical trial? to be accepted you need to have at least one bigger met, the ones in the lungs smaller than 10 mm would not qualify.
Olga
Re: Sam from Taiwan since 2002 (now 37 years old)
Hi, Olga：
I talked with the pancreas surgeon, he said the spleen won't be saved at the pancreatic tumor resection surgery.
So now immunotherapy has become my first option.
But the doctor had me have a gene test-MSI(microsatellite instability)
And the result shows that I am not MSI-High.
Will it affect the effectiveness of my immunotherapy of taking Keytruda?
Best,
Sam
I talked with the pancreas surgeon, he said the spleen won't be saved at the pancreatic tumor resection surgery.
So now immunotherapy has become my first option.
But the doctor had me have a gene test-MSI(microsatellite instability)
And the result shows that I am not MSI-High.
Will it affect the effectiveness of my immunotherapy of taking Keytruda?
Best,
Sam
Re: Sam from Taiwan since 2002 (now 37 years old)
Hi Sam
Msi as I understand it, is not a TOTAL factor in our SLOW indolent sarcoma .
Light reading :/
https://www.ncbi.nlm.nih.gov/m/pubmed/14562278/
Typically the majority of adults with asps , are on the low scale of Micro-instability. The tumors are rather large and have been “suppressed “ of oxygen and metabolic activity ?
On the down side of of progression ? Increases as the soft tissue is causing issues of pressure , late in life ?
As Olga has offered, immuno therapy truly is the key.
I bet Olga will have input.
Love
Msi as I understand it, is not a TOTAL factor in our SLOW indolent sarcoma .
Light reading :/
https://www.ncbi.nlm.nih.gov/m/pubmed/14562278/
Typically the majority of adults with asps , are on the low scale of Micro-instability. The tumors are rather large and have been “suppressed “ of oxygen and metabolic activity ?
On the down side of of progression ? Increases as the soft tissue is causing issues of pressure , late in life ?
As Olga has offered, immuno therapy truly is the key.
I bet Olga will have input.
Love
Debbie
Re: Sam from Taiwan since 2002 (now 37 years old)
Sam,
first of all do not be discouraged by the surgeon saying he is not going to be able to save the spleen - you might find some other surgeon who could using some other very specific approach from the back. For example Dr.Walz who has done the surgery for Ivan might be interested to review your scans remote. His surgery is less than $30,000 (which is a lot anyways but 10 times less than in US for example.)
But you really should try immunotherapy first and see in few months how does it go. Let us know if you need more articles re. its success in ASPS. They mean that Keytruda is approved for the MSI-High cancers, but I told you right away that it is not approved for ASPS yet as the clinical trials are still not finished but the good interim results were already reported and are widely known in the medical society. ASPS is typically responds to Keytruda, Opdivo or atezolizumab and ASPS is not typically MSI-High.
first of all do not be discouraged by the surgeon saying he is not going to be able to save the spleen - you might find some other surgeon who could using some other very specific approach from the back. For example Dr.Walz who has done the surgery for Ivan might be interested to review your scans remote. His surgery is less than $30,000 (which is a lot anyways but 10 times less than in US for example.)
But you really should try immunotherapy first and see in few months how does it go. Let us know if you need more articles re. its success in ASPS. They mean that Keytruda is approved for the MSI-High cancers, but I told you right away that it is not approved for ASPS yet as the clinical trials are still not finished but the good interim results were already reported and are widely known in the medical society. ASPS is typically responds to Keytruda, Opdivo or atezolizumab and ASPS is not typically MSI-High.
Olga
Re: Sam from Taiwan since 2002 (now 37 years old)
Hi, Olga：
Yes, I plan to try Atezolizumab asap.
Unfortunately, one brain met was found yesterday (5mm in size).
Does Atezolizumab work on brain mets as well?
Best,
Sam
Yes, I plan to try Atezolizumab asap.
Unfortunately, one brain met was found yesterday (5mm in size).
Does Atezolizumab work on brain mets as well?
Best,
Sam
Re: Sam from Taiwan since 2002 (now 37 years old)
Atezolizumab is one of the immune checkpoint inhibitors, it is currently on trial for ASPS specifically. We have one of the members on this trial in US and one in Canada (may be we have more I do not remember right now).
viewtopic.php?f=4&t=270
The ICI drugs are not much different one from another. But I have to say that 5 mm brain met is very easily treatable with the radiosurgery and many of our patients including Ivan had a radiosurgery to one of their mets with the goal to improve the response to immunotherapy - it is usually done in a following order: the radiosurgery is done first and the immunotherapy is started right after the radiosurgery - the next day. Is atezolizumab going to be given on a clinical trial or off label? there are limitations for what can be done during/before of the trial
viewtopic.php?f=4&t=270
The ICI drugs are not much different one from another. But I have to say that 5 mm brain met is very easily treatable with the radiosurgery and many of our patients including Ivan had a radiosurgery to one of their mets with the goal to improve the response to immunotherapy - it is usually done in a following order: the radiosurgery is done first and the immunotherapy is started right after the radiosurgery - the next day. Is atezolizumab going to be given on a clinical trial or off label? there are limitations for what can be done during/before of the trial
Olga
Re: Sam from Taiwan since 2002 (now 37 years old)
This is the link to atezolizumab clinical trial in US
https://clinicaltrials.gov/ct2/show/NCT ... 4#contacts
they say:
Patients with known primary central nervous system (CNS) malignancy or symptomatic CNS metastases are excluded, with the following exceptions:
Patients with asymptomatic untreated CNS disease may be enrolled, provided all of the following criteria are met:
Evaluable or measurable disease outside the CNS
No metastases to brain stem, midbrain, pons, medulla, or cerebellum
No history of intracranial hemorrhage or spinal cord hemorrhage
No ongoing requirement for dexamethasone for CNS disease; patients on a stable dose of anticonvulsants are permitted
No neurosurgical resection or brain biopsy within 28 days prior to cycle 1, day 1
Patients with asymptomatic treated CNS metastases may be enrolled, provided all the criteria listed above are met as well as the following:
Radiographic demonstration of improvement upon the completion of CNS-directed therapy and no evidence of interim progression between the completion of CNS-directed therapy and radiographic screening for the current study
No stereotactic radiation or whole-brain radiation within 28 days prior to cycle 1, day 1
Screening CNS radiographic study >= 4 weeks from completion of radiotherapy and >= 2 weeks from discontinuation of corticosteroids
https://clinicaltrials.gov/ct2/show/NCT ... 4#contacts
they say:
Patients with known primary central nervous system (CNS) malignancy or symptomatic CNS metastases are excluded, with the following exceptions:
Patients with asymptomatic untreated CNS disease may be enrolled, provided all of the following criteria are met:
Evaluable or measurable disease outside the CNS
No metastases to brain stem, midbrain, pons, medulla, or cerebellum
No history of intracranial hemorrhage or spinal cord hemorrhage
No ongoing requirement for dexamethasone for CNS disease; patients on a stable dose of anticonvulsants are permitted
No neurosurgical resection or brain biopsy within 28 days prior to cycle 1, day 1
Patients with asymptomatic treated CNS metastases may be enrolled, provided all the criteria listed above are met as well as the following:
Radiographic demonstration of improvement upon the completion of CNS-directed therapy and no evidence of interim progression between the completion of CNS-directed therapy and radiographic screening for the current study
No stereotactic radiation or whole-brain radiation within 28 days prior to cycle 1, day 1
Screening CNS radiographic study >= 4 weeks from completion of radiotherapy and >= 2 weeks from discontinuation of corticosteroids
Olga
Re: Sam from Taiwan since 2002 (now 37 years old)
Hi, Olga：
As for the immunotherapy, now I have two choices：Atezolizumab(PD-L1) vs Keytruda (PD-1).
My insurance won't pay for either one.
And I might have a chance to get Atezolizumab with 1/3 of the price of Keytruda for the next coming 2 years.
But I care about the effectiveness the most.
So is there any research that compares PD-1 with PD-L1 drugs to let us know which has higher response rate and works better on ASPS?
Can I ask my doctor to do a PD-1, PD-L1 check with my recently resected heart ASPS metastasis tumor so that I can know if my immunotherapy will work before I take it?
Best,
Sam
As for the immunotherapy, now I have two choices：Atezolizumab(PD-L1) vs Keytruda (PD-1).
My insurance won't pay for either one.
And I might have a chance to get Atezolizumab with 1/3 of the price of Keytruda for the next coming 2 years.
But I care about the effectiveness the most.
So is there any research that compares PD-1 with PD-L1 drugs to let us know which has higher response rate and works better on ASPS?
Can I ask my doctor to do a PD-1, PD-L1 check with my recently resected heart ASPS metastasis tumor so that I can know if my immunotherapy will work before I take it?
Best,
Sam
Re: Sam from Taiwan since 2002 (now 37 years old)
If the testing is free, go for it - it is interesting. But it will not change your treatment plan at all, as both negatively and positively tested tumors may respond to this treatment and at about the same rate:
Efficacy of PD-1 or PD-L1 inhibitors and PD-L1 expression status in cancer: meta-analysis
https://www.bmj.com/content/362/bmj.k3529
and I do not know how to pick the more promising drug as we do not have any statistics for PD-1 versus PD-L1, we just have more people on the PD-1 because they started earlier, and we have good reports in both groups. What about Opdivo? it is basically the same drug as Keyruda PD-1 but from the diff. maker.
And if you are going to have it not on clinical trial, I would strongly suggest to start from the radiosurgery to a small brain met as adding the radiosurgery to the ICI treatment greatly increases the chances of efficacy, helping the body to recognize the tumor.
Efficacy of PD-1 or PD-L1 inhibitors and PD-L1 expression status in cancer: meta-analysis
https://www.bmj.com/content/362/bmj.k3529
and I do not know how to pick the more promising drug as we do not have any statistics for PD-1 versus PD-L1, we just have more people on the PD-1 because they started earlier, and we have good reports in both groups. What about Opdivo? it is basically the same drug as Keyruda PD-1 but from the diff. maker.
And if you are going to have it not on clinical trial, I would strongly suggest to start from the radiosurgery to a small brain met as adding the radiosurgery to the ICI treatment greatly increases the chances of efficacy, helping the body to recognize the tumor.
Olga
Re: Sam from Taiwan since 2002 (now 37 years old)
Concurrent Immune Checkpoint Inhibitors and Stereotactic Radiosurgery for Brain Metastases in Non-Small Cell Lung Cancer, Melanoma, and Renal Cell Carcinoma.
https://www.ncbi.nlm.nih.gov/pubmed/29485071
we use RCC as the closest to ASPS in terms of radio- and chemoresistance and overall metastatic pattern, there is even sub type of RCC with the same translocation but unbalanced
https://www.ncbi.nlm.nih.gov/pubmed/29485071
we use RCC as the closest to ASPS in terms of radio- and chemoresistance and overall metastatic pattern, there is even sub type of RCC with the same translocation but unbalanced
Olga
Re: Sam from Taiwan since 2002 (now 37 years old)
Hi, Olga：
Does the following statement mean that PD-1 has better response rate and efficacy?
Conclusions
PD-1 or PD-L1 blockade therapy is a preferable treatment option over conventional therapy for both patients that are PD-L1 positive and PD-L1 negative. This finding suggests that PD-L1 expression status alone is insufficient in determining which patients should be offered PD-1 or PD-L1 blockade therapy.
https://www.bmj.com/content/362/bmj.k3529
Best,
Sam
Does the following statement mean that PD-1 has better response rate and efficacy?
Conclusions
PD-1 or PD-L1 blockade therapy is a preferable treatment option over conventional therapy for both patients that are PD-L1 positive and PD-L1 negative. This finding suggests that PD-L1 expression status alone is insufficient in determining which patients should be offered PD-1 or PD-L1 blockade therapy.
https://www.bmj.com/content/362/bmj.k3529
Best,
Sam