Pleomorphic xanthoastrocytoma tumor (Pxa): help

Posted by lmp1 @lmp1, Jun 27, 2017

My daughter was diagnosed with a Pleomorphic xanthoastrocytoma tumor in February. She had surgery to remove it. They thought they got it all but wasn’t sure. She is 25 years old and has a 4 year old and a 1 year old. Her last app and MRI was June 21 and it seems that it is growing back. I know this a rare tumor and I’m having a hard time finding info. Any help someone has would be appreciated. Would love to hear from other survivors of this rare tumor. Thanks,
A very worried mom!

@kodabear

Thanks David! I appreciate your sharing your daughter’s experiences. It’s makes me worry a little less with her occasional headaches. Her last one which was a couple of days ago was the worst one with vomiting and a sensitive scalp, but it passed. She used Tylenol and it helped enough. Opposite of your daughter’s situation, My wife’s tumor was in her left temporal Lobe. She also uses some use of the R side limbs as well but can still walk. Her hand loses maybe 30-40% Functionality. Question. Did your daughter have any problems with her skull cap from the surgeries? Infections, etc.

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kodabear, we had no problems with her head bandaging after drain removal, other than perhaps perhaps a bit of wound 'weeping'. We were advised regular and meticulous changing is important. Sickness, usually accompanied or preceded by a headache, was a feature immediately after operations, reducing over a couple of months. She still has more headaches than the average person, but not excessively so. ATB David.

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@dgruk

kodabear, we had no problems with her head bandaging after drain removal, other than perhaps perhaps a bit of wound 'weeping'. We were advised regular and meticulous changing is important. Sickness, usually accompanied or preceded by a headache, was a feature immediately after operations, reducing over a couple of months. She still has more headaches than the average person, but not excessively so. ATB David.

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My wife had several infections making it a difficult recovery, but eventually they got it under control. Thanks for sharing about the headaches.

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@dgruk

jakejta, I have some experience similar to Audra, fingers crossed for your son's diagnosis. My daughter has had a Grade 3 PXA for 6 years and is in her 20s. She has had multiple operations, radio, chemo, targeted therapy and re-purposed drugs and is still with us albeit with relatively minor disability. When you know the situation I would be happy to share our experience if that would help. Best wishes, David.

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David, thank you so much for participating in this forum. My son Josef is 18. He had a low-grade tumor diagnosed in 2011 due to seizures, we did wait and watch till the summer of 2018 until the seizures got worse and we ran out of options. He had surgery that was meant to be epilepsy, his tumor location is complicated in the insula. we found a surgeon who specializes in that area. The surgery went very well, but seizures remained. The pathology came back great ganglioglioma grade 1 and we did not have a follow-up MRI till 7 months later as we were considering a second surgery. During the MRI they found a new tumor, this one high grade, he had emergency surgery a week later and it came back APXA. He did proton radiation together with Temodar, then 10 cycles of Temodar together with mekinist(trametinib). His tumor had EML4-BRAF fusion, CDK4/6 deletion and tert mutation. The first 2 were present in the first tumor. The fusion so far appears to be one of a kind. He did great till last week, when a 3 months MRI showed recurrence. 3 months ago everything looked great. We did stop Temodar 2 months ago, he was having low counts and had lost weight and the NO thought 10 cycles would be sufficient and he was on mekinist, which many doctors thought we should have done alone. I saw you mentioned your daughter was on Temodar, is this the first time she has been on it. Also do you think THC helps? I have been giving him CBD oil along with various supplements(curcumin, Boswellia, mushrooms, omega3, vitamind, melatonin ..) Added chia, hemp seeds, flax lots of healthy grains, does not seem to be making any difference. We are trying to figure out what to do now, the doctors do not sound too optimistic or have great options. We are trying to reach out to as many centers as we can. I wanted to see if you have any advice. I had asked if we can go back on Temodar and non of the docs so far think it is a good idea.

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Juliaf, after a couple of successful years on Tafinlar/Mekinist my daughter had a recurrence followed by (another) major operation 2 years ago. After this she was put on Temodar (for the first time). On our own initiative we saw a specialist oncologist in London who suggested trying CBD and Sativex (an active Cannabis derived drug) in parallel. Since there are no officially recognised trials of CBD/S our NO was unable to comment on taking this in parallel, but he did not object. A key thing about the CBD/Sativex treatment is she takes it 3 days on and 3 days off ie it’s not continuous/every day and the oncologist specialist says that this is important. The Temodar plus CBD/Sativex treatment in parallel worked for about 15 months before her latest occurrence found in an MRI in January of this year. It is impossible to say whether the good effect over the 15 months was in whole or in part to the Temodar, and/or whether the Sativex/CBD helped.

After her latest occurrence she is now back on Tafinlar/Mekinist. We understand this is perhaps another ‘first’ ie trying Tafinlar/Mekinist in a “re-challenge” situation. So far it seems to have had a good effect though we have no MRIs at the moment to confirm what is actually happening in her head. But when she started on this 8 weeks ago she could barely move her R arm and now she has about 80% movement restored, so it would appear that trying Tafinlar/Mekinist for a second time is working. To be clear her sequence has been Tafinlar/Mekanist (about 2 years), Temodar (15 months) and Tafinlar/Mekinist (current and ongoing, 2 months so far) and there has been no overlap of these periods. Apparently there is some evidence that going back on Tafinlar/Mekinist after a ‘holiday’ from it, of at least 6 months, can be effective (in my daughter’s case this ‘holiday’ was about 2 years).

Regarding diet, everyone we speak to says eating healthily can help the immune system. My daughter has tried some of the things you mentioned, but this has waned over time, and we are not aware of any magic bullets here.

I hope this helps in some way, ATB David.

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@dgruk

Juliaf, after a couple of successful years on Tafinlar/Mekinist my daughter had a recurrence followed by (another) major operation 2 years ago. After this she was put on Temodar (for the first time). On our own initiative we saw a specialist oncologist in London who suggested trying CBD and Sativex (an active Cannabis derived drug) in parallel. Since there are no officially recognised trials of CBD/S our NO was unable to comment on taking this in parallel, but he did not object. A key thing about the CBD/Sativex treatment is she takes it 3 days on and 3 days off ie it’s not continuous/every day and the oncologist specialist says that this is important. The Temodar plus CBD/Sativex treatment in parallel worked for about 15 months before her latest occurrence found in an MRI in January of this year. It is impossible to say whether the good effect over the 15 months was in whole or in part to the Temodar, and/or whether the Sativex/CBD helped.

After her latest occurrence she is now back on Tafinlar/Mekinist. We understand this is perhaps another ‘first’ ie trying Tafinlar/Mekinist in a “re-challenge” situation. So far it seems to have had a good effect though we have no MRIs at the moment to confirm what is actually happening in her head. But when she started on this 8 weeks ago she could barely move her R arm and now she has about 80% movement restored, so it would appear that trying Tafinlar/Mekinist for a second time is working. To be clear her sequence has been Tafinlar/Mekanist (about 2 years), Temodar (15 months) and Tafinlar/Mekinist (current and ongoing, 2 months so far) and there has been no overlap of these periods. Apparently there is some evidence that going back on Tafinlar/Mekinist after a ‘holiday’ from it, of at least 6 months, can be effective (in my daughter’s case this ‘holiday’ was about 2 years).

Regarding diet, everyone we speak to says eating healthily can help the immune system. My daughter has tried some of the things you mentioned, but this has waned over time, and we are not aware of any magic bullets here.

I hope this helps in some way, ATB David.

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HI David, thank you for your response. My son has a BRAF fusion that does not respond to the combo. We started a clinical trial of a second-generation braf inhibitor TAK-580 last week. I have talked to many parents of braf600 and maybe this will help, there is Dr. Levy in colorado childrens who is doing a trial of hydroxychloroquine( i know it sounds strange) added to the combo if it stops working and said she is seeing good results. It sounds like the combo was working for her before it may be another thing to have in your bag. Also selumetinib is now available by prescription in US and the oncologist running my son's trial mentioned that it had better results then mekinist. There is also mek-162 another newer mek inhibitor. Are you in the inhibitor buddies facebook page? It may be useful for you there are parents of kids with these types of mutation in there.

It is interesting about 3/3 days thing, I am going to give it a try although we only use CBD oil. My son also suffers from seizures and the neurologist wants him to try epidiolex, I think for seizures you are supposed to take it every day, wonder how it would work. Because we did temodar and mekinist after radiation and surgery, we do not know which of these held it at bay for 14 months we had good MRIs. We just know the tumor was back 2 months after we stopped Temodar, but every doctor we ask says its a coincidence. He did 5 day on 28 day cycles it was taking a toll on his blood counts and platelets. Did she take it the same way? They had him on a very high dose of 340 mg he is only 110lb. He lost 15 lb while on it. I hope the combo is working for your daughter. Julia

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@juliaf

HI David, thank you for your response. My son has a BRAF fusion that does not respond to the combo. We started a clinical trial of a second-generation braf inhibitor TAK-580 last week. I have talked to many parents of braf600 and maybe this will help, there is Dr. Levy in colorado childrens who is doing a trial of hydroxychloroquine( i know it sounds strange) added to the combo if it stops working and said she is seeing good results. It sounds like the combo was working for her before it may be another thing to have in your bag. Also selumetinib is now available by prescription in US and the oncologist running my son's trial mentioned that it had better results then mekinist. There is also mek-162 another newer mek inhibitor. Are you in the inhibitor buddies facebook page? It may be useful for you there are parents of kids with these types of mutation in there.

It is interesting about 3/3 days thing, I am going to give it a try although we only use CBD oil. My son also suffers from seizures and the neurologist wants him to try epidiolex, I think for seizures you are supposed to take it every day, wonder how it would work. Because we did temodar and mekinist after radiation and surgery, we do not know which of these held it at bay for 14 months we had good MRIs. We just know the tumor was back 2 months after we stopped Temodar, but every doctor we ask says its a coincidence. He did 5 day on 28 day cycles it was taking a toll on his blood counts and platelets. Did she take it the same way? They had him on a very high dose of 340 mg he is only 110lb. He lost 15 lb while on it. I hope the combo is working for your daughter. Julia

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Hi Julia, re the 3/3 approach this was recommended by a Professor Dalgleish in London. He said that the receptors in the tumour get interested in the active ingredients in cannabis extracts (Sativex and CBD combined) and when they do they pay attention to this and stop growing. But this doesn't last, in effect the receptors lose interest after a few days. So the idea is that with 3 days on the receptors get distracted and the switch off/switch off cycle distracts them the most. I'm sorry I don't have any more explanation than this and, of course, cannabis extract effect on tumours has yet to be studied comprehensively.

Re the other drugs my daughter has had 4 periods separate periods 1. Dabrafenib only 2. Dabrafenib/Trametinib 3. Temodar 4. Dabrafenib/Trametinib (currently on this). Other than D/T she has had no combination so Temodar was taken separately only. Each of these drug periods had a good effect but then wore off about 12-18 months later. She is currently 2m into D/T (ie period 4.) and this seems to have stopped the most recent deterioration.

Her weight has gone up and down, the main issue being weight gain whilst on steroids (necessary after 2 of her operations).

Fingers crossed for you. David.

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