Jolie's mother from California - Dx Oct 2013

[D.ap]

Hey Jolie

So Chrisann has had about 3 doses ( 3 months)of Keytruda ?
And the tumor was around 4 cms? When discovered ?
Where was it located ? Near the inner perimeters of the frontal area ? But in the memory area, which are being affected by swelling ?

I’m so sorry to hear of .
Have you all talked of the swelling subsiding and memory and site returning ?

Hugs to you both .
Love

[Olga]

At least she should make a complain with the insurance re. incorrect scanning schedule, for ASPS it is not the sensitive scan.
Also if the local oncologist proposes the incorrect scanning schedule or type, the best way is to ask for the second opinion from the expert oncologists in ASPS.
Now she should have the chest CT and abdominal MRI to see if there are might be more silent mets.

[D.ap]

Whew
Breath in and out. Me ..

Olga is right .

Things won’t change unless we make an alert to the medical professionals .

Chrisann needs additional scans to make sure the neuropathy isn’t a result of a tumor and or other tumors issues ?

[Olga]

Debbie - it is a very good observation as I suspect the neuropathy WAS a result of the brain tumor, misdiagnosed as a side effect of Keytruda - on Keytruda the swelling/inflammation around the brain met got bigger so the symptoms appeared. neuropathy is not a frequent K side effect actually to leap to that conclusion right away - peripheral neuropathy is only registered in 1.7% of the patients on K... It is however the frequent side effect of the traditional cytotoxic chemotherapy, that regular oncologists often see in their practice so they assumed it was.

[JolieS]

I have not read scan info for exact location and measurement but I do know was in the Occipital Lobe. Her memory/speech has already improved significantly since Wednesday which I believe is due to the swelling decreasing.

So my mom got really bad neuropathy while on the clinical trial where she was receiving regular brain MRIs. I do not believe the tumor is causing this.... thoughts??

I will request the additional scans. Once she is off steroids and antibiotics we will explore another immunotherapy.

One doctor mentioned needing radiation to the brain.... I feel like it’s not necessary since it was a full resection.

[Olga]

There is always a possibility that she would fall into this small % people with that particular reaction to K.
MRI scan is pretty sensitive, so now knowing the exact location of the resected tumor, she can request the retrospective review of the previous scans to see if this met might have been there but barely visible and missed. It is not rare with ASPS. When was the last brain MRI done on a trial? 4 cm is abig brain met and usually it takes time to get to that size.
Did she have a brain MRI before of this resection? If there is nothing else, the radiation is not needed, Ivan had the single brain met resected in summer 2013 and had no other treatment after the excellent clean margin resection.

[JolieS]

Thank you Olga. I will make sure to ask this of her oncologist at UCLA when we go see him.

They did a brain MRI before the resection which showed the mass, but from my understanding it also showed bleeding so the image of the tumor is not super clear. The surgeon told us that the post OP MRI showed no evidence of tumor remaining, just some small amount of blood left in the area. She will receive another brain MRI in 2 weeks, I think. The surgeon mentioned a lumbar puncture may he indicated in a few weeks to test for signs of malignancy? Does this make any sense? It’s worth noting that her neuro surgery team are not familiar with sarcoma AT ALL. The
Pathology dept at this hospital gave an official report of “abnormal cells” for the brain tumor tissue. They have never seen ASPS in their careers and don’t know what to look for....

Ok so just to recap my game plan, I’m going to ask the UCLA specialist the following:
1. Order abdominal and pelvic MRI
2. Review previous brain MRI imaging to see if this tumor was previously present and missed
3. If we have reason to believe the tumor caused neuropathy, restart Keytruda ASAP
4. If we believe Keytruda caused neuropathy, try a different immunotherapy like Opdivo
5. Confirm no evidence of disease in brain & that brain radiation is not necessary

[JolieS]

Also... since my mom was already on an anti PD1 immuno trial, she likely will not qualify for another, correct??
What is the likelihood of getting Pfizer to give her the drug that worked so well for her on the trial 2 years ago as compassionate use?

[D.ap]

Morning Jolie,

How's your mom doing today?

Did your mom develop hyperglycemia while on the PF-06801591 trial that you are aware of?
How long was she on the trial before she had to stop because of her neuropathy?

[Olga]

Very well structured Jolie.
Add the pathology for the resected brain met clarification to your list - they can consult experts if this is to hard for them. It is important to know what this brain met was for sure - there is still some % probability of having the second malignancy and abormal does not mean malignant, i.e. the tumor consisting of inflammation response cells would also be abnormal, and besides the tissue would show a very important info re. immune system cells massively infiltrating the tumor causing it to swell - i.e. sensitivity to Keytruda. They need to refine the report. There is usually enough of the frozen tissue in a tissue bank after the surgery to get a second or third opinion and it is always paid by the insurance as it may change the patients treatment plan.
There is a difference to discontinue the drug due to an excess toxicity or due to it working on the missed brain met. Tell the insurance case manager that the brain scanning was requested on a basis of typically frequent brain metastases occurrence in ASPS patient and refused and that it caused a catastrophic event so you are aware of it and are evaluating your options.
Spinal tap were not used in Ivan's case and I am not aware of it in other cases used, may be does not make sense in ASPS versus other brain tumors like glioma etc.
No all the immune immuno trials disqualify on a basis of a previous immunotherapy, some just require a wash out period.
Pfizer's drug that your mom had on a trial, is not approved yer as far as I know (please verify) so can not be given on a compassionate use basis.
Keytruda and Opdivo are the drugs given on a compassionate use basis now to ASPS patients and technically they are the same so any should work.
Other drugs in trials that would be good to try are Atezolizumab and Avelumab.

[JolieS]

Hi guys,

My mom has been healing and has recovered an amazing amount from the brain injury caused by the brain met. She completed targeted brain radiation to the resection area last week. She’s regained 85% of her cognitive functions. She has been having random focal seizures since the surgery, and the anti seizure medicine has been affecting her mood.

However the lung spot in her right upper lobe continues to be problematic. She is in the hospital now due to coughing up scary amounts of blood. It turns out this spot has grown significantly in one month and is now blocking the major airway and beginning to obstruct the middle and lower loves as well.

Her pulmonologist wants to do another bronchoscopy to clear the airway. The last one back in November 2018 resulted in her awful lung abscess infection. I’m really worried. What could cause this tumor to start growing so rapidly?? This will be the third procedure to clear the airway from the same tumor. This tumor has also received SBRT radiation twice over the past 4 years. Why won’t it just stop coming back!!!!!!!!!???

I’m so concerned. What should we do? They say Cyro is not a good option because of the tumor location.

[Olga]

I am sorry to hear that. She should have had a surgery to remove that met a long time ago. It is an active, mutated tumor that just needs to go, even if they have to remove a part of the lobe. People only need 1/5 of their lung tissue to have the completely full functionality.

[D.ap]

Hello Jolie
I agree with Olga on pursuing surgery .
So the biopsy of the brain tumor was positive for ASPS? You all had it resected then radiated ?
I’m sure sorry to hear of the coughing . I would say no more bronchoscope as well .
In reference to anti seizure Med ,talk with doc about trying others as they truly can be mood altering.
Love

[JolieS]

The location of this tumor is too close to her heart / center for resection. It is not an option. The bronchoscopy clears the airway and stops the bleeding.

Yes her brain tumor was positive for ASPS.

Her pulmonologist here at UCLA is very good at this specialized type of bronchoscopy to “debulk” the tumor.

[D.ap]

Jolie
Is your mom on any type of treatment at this time ?