Nhi from NY - Dx June 2016

[Bonni Hess]

Hello again dear Nhi and thank you for the additional shared information about your scan regimen. I am grateful that you are now receiving abdominal/pelvic MRI's instead of CT scans which pose such a dangerous risk of radiation exposure to your vital organs. Brittany has undergone three Cryoablation procedures with Dr. Littrup to treat dangerously located lung mets, an RFA for a liver met, an RFA for a tibia met, and RFA for lung mets. Although both the Cryo and RFA procedures were thankfully successful in shrinking and destroying all of Brittany's respectively ablated mets, I would personally recommend that you consult with Dr. Littrup regarding the possibility of Cryoablation of your manubrium met. Dr. Littrup is extremely knowledgeable and experienced which is critically important for a successful outcome with Cryoablation procedures.We have always suspected, but cannot validate, that Brittany's lung met RFA at The University of Pittsburgh may have caused seeding and dissemination of her tumor cells through the pathway of the heated RFA probe since shortly following Brittany's lung met RFA, she was devastatingly diagnosed with previously undetected mets in her pancreas, duodenum, and small bowel intususception. It is critically important to research and pursue the best treatment available performed by the most knowledgeable and experienced interventional radiologist. In the meantime, I will be anxiously awaiting the results of your today's abdominal/pelvic MRI which will Hopefully bring good news of no mets and of disease stability. Additionally, I again encourage you to request and schedule a full body bone scan to ensure that there are no other bone mets. Heart to heart with special caring thoughts, healing wishes, and continued Hope, Bonni,

[ntran727]

So I have more bad news. MRI abdomen pelvis shows a 1.9cm bone met in my L5 spine, 0.7cm in my L4, and right iliac lesion (near my primary). This is not good at all. Seems like retrospectively they found the 1.7cm in my L5 from the last scan in May 2018. I am getting a PET scan on 3/7. I don’t even know what to do at this point because it seems that it has really progressed :/ are the spine Mets going to be treatable?

[Olga]

Nhi - I think it is a very alarming development. Ask the oncologist to contact Dr.Wilky from the ASPS clinical trial in Miami and ask her if there were any good results with the bone/spine mets on the Keytruda trial. Also there are few other ICI trials going on incl. atezolizumab for ASPS - may be the oncologist could find out what trial might be more promising for the bone mets. We need the inside information.
In the meantime ask him to consult re. possible radiosurgery. If he could get Keytruda off label as Ivan is getting or Opdivo as Josh is getting, it would be beneficial to combine one of the Keytruda or Opdivo treatments with the radiosurgery. May be there are promising combination treatments that aim to potentiate ICI efficacy. If you go on a clinical trial, you can try to treat one bone met before starting the trial.
If the iliac bone met is small, it might be treated with the cryosurgery as well.
Also might be make sense to start bisphosphonates to try to slow down the bone mets growth?
Although I do not know about possible ICI and bisphosphonates interaction, even after a single shot they stay in the system for a very long time

[Bonni Hess]

Dear Nhi, I am so deeply sorry for the heartbreaking results of your abdominal/pelvic scans. Unfortunately, based on my 17 and a half years of ASPS experience, this is the cruel nature of this indolent ,but very unpredictable, disease which can appear to be stable for a period of time and can then become more aggressive with disease progression. I agree with Olga's suggestions, and am wondering what treatment recommendations your oncologist has made. Thankfully the new Immune Checkpoint Inhibitors (ICI) like Keytruda , Opdivo , and Atezolizumab are showing very encouraging success for several ASPS patients, and I join Olga in encouraging you to pursue possible treatment with one of these promising new drugs. Addituonally, based on your somewhat aggressive disease progression, I strongly encourage you to schedule a brain MRI as soon as possible even though your yearly brain MRI is not due for several months. Based on my extensive observations of ASPS patients treated with Sutent , Sutent may provide initial met shrinkage and disease stabilization as it did for you, but unfortunately, eventually the patient develops disease progression. I was SO Hopeful that this would not be the case for you, as every patient can have a different response to the same treatment. It is very difficult to determine if your recent disease progression is the result of your reduced Sutent dosage, or just the historical nature of ASPS patients to develop resistance to the Sutent, but it does not seem that remaining on Sutent is a good treatment plan at this time. I know that you have many treatment decisions to make, but please stay in touch and keep this Board updated as you are able. Holding you very close in my heart, sharing your sorrow and concern, and sending special caring thoughts, healing wishes, and continued Hope, Bonni

[ntran727]

Thank you everyone for your support.

I don’t think the sutent is working, and not because the dose was lowered because they saw the bone changes in the L5 on the last scan in May 2018, and it was 1.7cm. Now it is 1.9cm 7 months after. They just didn’t mention it on the last scan. The right iliac one is 0.7cm and they didn’t comment on the size of the one on L4. Seems like that has been happening a lot with these findings that aren’t being mentioned until later. I think I’ve had disease progression in the past 8-10 months but just hasn’t been caught. I am going to ask about immunotherapy as I am sure he can get it off label for me, also in conjunction with radiotherapy for my bone mets. I wonder what this means with me only getting bone Mets and no other Mets and why my lung is stable. Maybe those lung Mets have been necrotic and just scarred, but I think I would rather have Mets somewhere that’s not in my bone and especially the spine :( because it makes it harder to treat. I am asking for brain MRI because lesions don’t always show up on PET scans. I see my oncologist on 3/7 to discuss. I will keep you all updated.

[khp]

Hello Nhi, I'm so sorry to read about your bone mets. My wife Pam was in the Axitinib plus Pembrolizumab study in Miami with Dr. Wilky from June 2016 until March 2017. Pam had numerous bone mets throughout her spine and other parts of her body. Pam had to leave the trial due to her liver mets increasing in size. The Keytruda did, however, kill all of her bone mets and a great number of lung
mets of which none have returned. Pam is now in an Anlotinib trial which she started in August 2017.
Dr. Wilky is not in Miami as she moved to the University of Colorado Anschutz Medical Campus School of Medicine in Aurora Co. in late October of 2018 her number is 303-724-6429 or Fax 303-724-3889. I encourage you to take Olga's advice and pursue the Keytruda as well as contacting Dr. Wilky, She is a wonderful person and great doctor. We wish you the best and you will be in our prayers.

[D.ap]

Nhi
I’m so sorry for your findings ..
As you pursue further treatment I want to extend a big hug your way.

Ps Karl
News of Pam is so great to hear!:)

Much love

[Bonni Hess]

Dear Nhi, I am very perplexed and frustrated, as I know that you must be too, as to why the radiologist and oncologist inexplicably and I feel inexcusably failed to tell you about your new mets when they were seen on your scans 8+ months ago in May, thus allowing you to erroneously/ naively believe that your disease was stable! The 1.7 Cm. spinal met on your L-5 should have definitely been reported and addressed at that time. I am very thankful that it only increased in size to 1.9 Cm. during the past 8+months since it was observed on your scans, and perhaps that relatively small amount of growth can be attributed to the effect of the Sutent. However, I agree with you that the Sutent is apparently not working to stabilize your disease progression, even though it seemed to have stabilized your lung met growth. I think that you need to aggressively pursue another systemic treatment, and I am very encouraged by Karl's gracious sharing about his dear wife Pam's very successful response of her numerous bone mets throughout her spine and other parts of her body to the Pembrolizumab (Keytruda) and Axitinib combination Clinical Trial treatment. Although Dr. Wilky is no longer practicing at the University of Miami, I strongly encourage you to contact her with the information that Karl thoughtfully provided as Dr. Wilky is very knowledgeable and passionate about ASPS patients and treatments. I am grateful that you are requesting a critically important brain MRI, and I will be anxious to hear the outcome of your March 7th oncologist appointment. Take care, stay strong, be as well informed and pro-active as possible, let Hope continue to lead you through each day, and know that you have the special caring and support of those of us in this ASPS Community who qll share your challenging battle with this insidious disease. With gentle hugs, deepest caring, healing wishes, and continued Hope, Bonni

[Ivan]

Well goddamn. Radiologists like these make me absolutely furious. Lazy excuse of a human who absolutely 100% fails at their job and should be held criminally liable for missing a >1.5 cm tumor.

Tom has a long standing battle with a spinal tumor, and he's been stable on PD-1 for 2 years now, I believe.

[Bonni Hess]

Dear Nhi, In thinking about your disease progression other than just the known nature of this disease to eventually progress, and knowing that Sutent has unfortunately not been shown to provide long term sustained disease stability for the majority of ASPS patients, and since your new mets apparently initially appeared about 9 months ago, I am wondering if there is a connection between your disease progression and the hormones which you had to take to facilitate egg retrieval for the invitro fertilization procedure for your surrogate? Based on our extensive 17 and a half years of personal ASPS observations, research, and networking we have always felt that there is a connection between increased hormones and ASPS disease progression , which is why oral contraceptives, ,pregnancy and hormone replacements have not been options for Brittany. If the hormones required for your egg retrieval might be related to your disease progression, this may mean that the Sutent was possibly working, but just not strong enough to overcome the effects of the high dose hormones. There are certainly so many unknowns and variables with this unpredictable disease, but this might be something to discuss with your oncologist at your appointment on Thursday. With special caring thoughts, healing wishes, and continued Hope, Bonni

[ntran727]

Hi Bonnie,

I did the egg retrieval before even starting the sutent in December 2016. It was 10 days of hormones only and after I started the sutent, my lung tumors decreased in size and stayed around 3-4mm (9mm was the largest before that). I have not been on any other hormones since then including birth control due to the fear of hormonal effects on the cancer. We did lower the sutent dose to the lowest dose which has been for the past 10 months though and I am wondering if that’s what led to the disease progression. I also am now thinking that this L4 spine met has been there for a much longer time and just was not noted on the scan. I have copies of all the scans which I will look through myself to see if it was visible and the plan is to speak with my oncologist regarding the unacceptable misses from these radiologists. This is 3 misses, starting with the lung Mets that were retrospectively there, the manubrium met which was retrospectively there 2 scans ago, and now this L4 one that’s is now 1.9cm in size. There has been a different radiologist reading it each time and looks like many residents/fellows as well with the report being signed off by the attending radiologists. I am very upset that they missed this as it would’ve affected my treatment. My oncologist is heading the trial for Tecentriq in New York and most likely that’s what I will be starting. Which met should I try to treat with radiotherapy? Should I try to address the 1.9cm mass in the L4 spine first? Manubrium and right iliac are still <1cm but better location for radiotherapy likely but I’m worried about the size of the L4 met. Any advice would be appreciated. I am getting a PET scan same day as my oncology appointment on 3/7 and MRI brain scheduled for 3/16 as they didn’t have anything sooner. What do you think about the PET scan in terms of detecting further Mets? I’m so afraid there are more Mets in areas that we don’t scan, but not sure if bone scan would be better or PET scan. My onco thinks PET is more useful.

[Olga]

When I was asking an advice from the various big names radiologists what met to choose to treat in conjunction with the immunotherapy, the advice was to pick the one that is less dangerous to irradiate, i.e. less damage to surrounding tissue or the one that really needs to be treated ASAP - i.e. the clinical benefit is expected from its treatment. I would meet with the best radiation oncologist you can get there and discuss this subject with him. Combining SBRT with the immunotherapy is a popular topic now. The large met in L4 is of course concerning but you need to ask the spinal surgeon to review it to define its location better - is it in a soft tissue or what part of the vertebrae or elsewhere so then the immediate danger can be assessed, as basically you will have to decide if you can wait for the immune therapy to work, or the risk of damage is justified. The spine SBRT risks are significant - the treated or the next vertebrae could break, weaken/fracture, damage to the spinal nerves etc. It all depends on the location. So basically you need the extensive workout before the making an informed decision.
The other very important subject is timing, check with the clinical trial what is the latest treatment time before of the trial start is allowed.

[Bonni Hess]

Hello again dear Nhi, Thank you for your very prompt response and the additional shared information regarding the time frame during which you took hormones for egg retrieval. I am grateful that you are aware of the concerning effect of hormones on cancer. I am grateful too for your knowledgeable and pro-active approach to fighting your disease, and I share your great frustration and anger for the radiologists and oncologist repeatedly missing/failing to report your spinal and manubrium mets. Sadly, despite our VERY pro-active approach to fighting Brittany's ASPS, we too were the victim of radiologists missing Brittany's clearly obvious spinal met two scans in a row, and it was consequently not found until it had grown large enough to become symptomatic, and was then devastatingly threatening paralysis or death. We have ALWAYS insisted on personally looking at the scans and reviewing them with the oncologist, but we had temporarily been assigned to an over scheduled interim oncologist who dismissively said he didn't have time to review the scans with us, and he irresponsibly relied on the inadequately done radiologist report which erroneously stated that there were no new tumors and indicated that Brittany's disease was stable. These kind of "mistakes" are inexcusable, and put patients' Lives at risk, but unfortunately they seem to occur more often than people realize. That is why it is vitally important to be adamant about demanding to personally look at and review the scans at least with the oncologist, as well as with the radiologist if possible, and to obtain CD copies of the scans for your personal records and review. In the case of Brittany's large spinal met, it was clearly visible on the scans even to us as lay people when we retrospectively went back and looked at the scans from the previous 6 months, but the radiologist's unacceptable excuse was that his orders were to evaluate the chest CT for lung mets, and not the spinal area!!... Regarding your situation and your questions, Olga has offered you some excellent advice which I agree with. It is very important for you to prioritize the treatment plan for your mets based on the criteria that Olga has provided, and I strongly encourage you to contact Dr. Littrup for consultation as soon as possible. I think that pursuing systemic treatment with the promising new immunotherapy drug Tecentriq (atezolizumab) is a good option, but also think that treatment of your bone mets needs to be addressed and evaluated before you begin your Tecentruq treatment . I am so deeply sorry and saddened for your heartbreaking new challenges in the midst of what should be such a joyous time with the expected May arrival of your precious twin babies, but I am grateful that there are promising new treatments now available. Take care dear Nhi, stay strong, keep in touch, and let Hope lead you through each day.
With gentle hugs, deepest caring, healing wishes, and continued Hope, Bonni,

[Bonni Hess]

Dear Nhi. I am thinking about you and sending best wishes and most positive thoughts today as you have your PET scan and meet with your oncologist to discuss treatment options. Based on my extensive personal experience, observations, and research, I disagree with your oncologist about a PET scan being more useful than a bone scan. PET scans can be, and in our experience with Brittany, are unreliable and sometimes erroneous in showing false positives or false negatives. In 2005, a specialized PET scan was done which alarmingly showed brain tumor activity in Brittany"s previously resected parietal tumor bed. Based on the what we later learned were totally erroneous results of the brain PET scan, Brittany underwent what proved to be a completely unnecessary Interstitial brain procedure in Boston in which no sign of tumor reoccurrence was found by the surgeon!! I have known of other cases where the PET scan failed to show new mets which were consequently then not found until they became symptomatic /or other scans detected them. PET scans MIGHT... be helpful as part of a diagnostic regimen, but I personally strongly feel that the regular scans should be the primary diagnostic tool. I will be anxiously awaiting your next update as I continue to hold you very close in my heart and my most caring thoughts with healing wishes and continued Hope, Bonni

[ntran727]

Yes, I agree, probably wasn’t necessary. The PET Showed the manubrium met (which was recently biopsied and showed more activity because of that), and the L4 lesion, which means it picks up the large ones. He wants to start me on Keytruda or Opdivo off label, sent by Merck for free anticipatig that my insurance would likely not approve the medication for me. We went with Keytruda. He said the trial for Tecentriq was on hold because they needed to addend something and that I wouldn’t qualify anyway because bone mets don’t count as part of the Mets included in the criteria, which have to be >1cm in size in any soft tissue or spread to any organs besides bones. Weird. He also said there was no difference between either Keytruda, Tecentriq, or Opdivo in terms of its efficacy on a specific type of met like bone mets vs other Mets etc. because we just don’t have enough data right now. I asked about usefulness of cryoablation/RFA and he said he would rather first see how the Keytruda works alone first and then think about that option later if it doesn’t shrink enough. So I think I will wait also before having a dangerous procedure done to potentially unsafe places. He does think that maybe lowering the dose of sutent caused a change in growth of the tumors. There is just no way to tell now. He is going to have a sit down with the radiologist to see how long the now 1.9cm lesion in the L4 spine has been there to check the rate of growth. They will send the bone met for testing. Do you all think I should still contact Dr. Littrup or to wait to see how Keytruda works first?