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!

Counseling has always helped me. This is a nasty beast. I'm so glad you have good doctors. So sorry she had an infection. It's bad enough to have to just go through surgery! Good luck and prayers coming your way.

Liked by kodabear

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Hi everyone. Hoping to find some info on here. My best friend's daughter has a PXA grade 3 – not responding to treatment. Has had two tumor removal surgeries and is on nivolumab as last effort and that doesn't seem to be working. Very aggressive tumor. Radiation/Chemo – nothing worked. Anyone have any thoughts on last ditch ideas. She is at Children's Hospital of Philadelphia and they are talking about hospice. This beautiful kid just turned 18 and has brilliant future ahead.

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@ejacobs16

Hi everyone. Hoping to find some info on here. My best friend's daughter has a PXA grade 3 – not responding to treatment. Has had two tumor removal surgeries and is on nivolumab as last effort and that doesn't seem to be working. Very aggressive tumor. Radiation/Chemo – nothing worked. Anyone have any thoughts on last ditch ideas. She is at Children's Hospital of Philadelphia and they are talking about hospice. This beautiful kid just turned 18 and has brilliant future ahead.

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Hi @ejacobs16 and welcome to Connect. You'll notice that I moved your message to this exisiting discussion group where you can meet other members talking about pleomorphic xanthoastrocytoma tumor (Pxa), like @bjh369 @lmp1 @daniellemarie @dgruk @kodabear and others. If you click VIEW & REPLY you will be taken to this message in the discussion and you can scroll through past messages.

This must be so difficult for you, your friend and her whole family. Has she had proton beam therapy?

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@colleenyoung

Hi @ejacobs16 and welcome to Connect. You'll notice that I moved your message to this exisiting discussion group where you can meet other members talking about pleomorphic xanthoastrocytoma tumor (Pxa), like @bjh369 @lmp1 @daniellemarie @dgruk @kodabear and others. If you click VIEW & REPLY you will be taken to this message in the discussion and you can scroll through past messages.

This must be so difficult for you, your friend and her whole family. Has she had proton beam therapy?

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Thank you, Colleen. So yes she had proton beam therapy – and we still find ourselves in this situation. So sad and desperate.

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My daughter was in a similar situation with the same type of tumour aged 21 at the time. Check if they have tested for the biomarker BRAF V600E (https://en.wikipedia.org/wiki/Cancer_biomarker). Apparently PXA tumours often have this and if so there are drugs that might help. My daughter was put on Dabrafenib (another drug, like Nivolumab, originally developed for Melanoma) which worked very well, giving her several years good quality life without serious side effects. We have some difficultines now but she is nearing 5 years from when it was originally found. Irrespective do keep advising what drug treatment they propose or have discounted in case I or anyone on this forum has heard of other possibilities. All the best with it anyway. David.

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@dgruk

My daughter was in a similar situation with the same type of tumour aged 21 at the time. Check if they have tested for the biomarker BRAF V600E (https://en.wikipedia.org/wiki/Cancer_biomarker). Apparently PXA tumours often have this and if so there are drugs that might help. My daughter was put on Dabrafenib (another drug, like Nivolumab, originally developed for Melanoma) which worked very well, giving her several years good quality life without serious side effects. We have some difficultines now but she is nearing 5 years from when it was originally found. Irrespective do keep advising what drug treatment they propose or have discounted in case I or anyone on this forum has heard of other possibilities. All the best with it anyway. David.

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Thank you for reaching out. So she has v600g if that means anything to you?

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I am not an expert but had a quick look on the web and it seems that V600G is a different, possibly rarer, form of mutiation than V600E. I found this document which talks about the V600G mutation in Melanoma patients: https://mc.vanderbilt.edu/ckm/gl/mutation_profiles/pdfopen.php?fnp=melanoma/content/BRAF/Melanoma_and_BRAF_V600G.pdf Near the end it asks: "Are there targeted therapies for BRAF V600G?" and the answer seems to imply Dabrafenib may be relevant so it's probably worth asking about based on this citation. Also Vemurafenib is another similar drug I should have perhaps mentioned too (though my daughter had unsustainable side-effects from that when she tried it). ATB David.

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@kodabear

Hey @Imp1 how is your daughter doing on the clinical trial? My wife has PXA grade 3 and finished her radiation and temodar treatment. mRI shows it recurring and I’m looking for other treatments. She was feeling pretty good during the treatment but now that she’s been off of it for. Few weeks, her initial symptoms are coming back, And in addition she is experiencing constant dizziness. I don’t know what to do about the dizziness. Thanks to all of you for sharing your stories in these feed. It helps to know we are not alone in this. 😢

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@kodabear , I’m sorry to hear about your wife. I don’t get on here often because I stay busy. My daughter is doing very well. She is on the maintenance part of the clinical trial right now and her tumor is stable. She has Avastin every 2 weeks and the clinical drug once a month now, (dsp-7888)is the name of the trial drug. This is a type of immunotherapy. It can affect your heart and kidneys so she has to be watched closely with EKGs and blood work but so far she has been ok. Radiation and tremador did not help her at all. Also when she was 1st diagnosed they called her tumor a PXA but when it came back 6 months later it was GBM grade 4. With the Avastin you can’t have surgery at least a month after taking it because of risk of bleeding. I hope this helps you. As of right now she is doing so great and of course we know that can change any day but we just take one day at a time and hold our breath in between MRI. She has them every 2 months.

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Here's an update on my daughter. She has been taking Temozolomide (TMZ) together with cannabis extracts (Sativex and CBD oil) for the last 4-5 months and in her last scan we finally had some better news – her tumour has reduced. We are not getting carried away but it was lovely to hear something positive after the incessant negatives and stressparticularly over the last year or so. Her next scan is now planned for 3 months which will hopefully give us some breathing space. FYI in the 28 day TMZ cycle she has a week or so at the start where she feels very tired, and tummy troubles can arise, but the rest of the time she feels pretty good so overall it's ok. Also FYI she was put on steroids for 3-4 months, as a precaution against swelling/pressure effects in her head, but has now come off them. The steroids caused her body to swell, for example she got a much rounder face, and clothes became tight fitting. But she has now migrated off these and is hopefully returning to normal. But, it's worth noting that during the phased withdrawal from steroids she had fairly bad headaches, so if you or yours are suggested to go on these, or are on and need to come off, be advised of this possibility. Fingers crossed for all of you. David R

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Imp1 and David, I’m glad both of your daughters are doing well. My wife, I am glad to say is fairly stable too. She had a heck of a time recovering from her last round of surgeries, but is now trying to figure out what she can and can’t do both mentally and physically. Thanks for sharing. It’s good to know what my wife is going through is common like the weight gain from the steroids. She’s been off of them for a little less than a month but still swollen looking around the face and the neck. Has headaches occasionally, gets confused from time to time and has to just stop and refocus. My wife just had an MRI last night. Hoping that the results are the same. Ever since January she has become a hard stick. Everyone has an extremely hard time drawing blood or putting in an IV. That is a nightmare for her in itself. I guess it is from the Temodar? I hope to eat more good reports from you guys. Makes me feel hopeful and very happy. I will try to do the same. My wife is supposed to take her third round of Temodar in a few days. She gets extremely tired too after a few days of taking it and lasted for about a week and a half. Her energy levels are better now, but I need to remind her to eat. She would sleep for half the day if she could. 😉 the best to you all!

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@ejacobs16

Hi everyone. Hoping to find some info on here. My best friend's daughter has a PXA grade 3 – not responding to treatment. Has had two tumor removal surgeries and is on nivolumab as last effort and that doesn't seem to be working. Very aggressive tumor. Radiation/Chemo – nothing worked. Anyone have any thoughts on last ditch ideas. She is at Children's Hospital of Philadelphia and they are talking about hospice. This beautiful kid just turned 18 and has brilliant future ahead.

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Sorry to hear that eJacobs. My wife’s situation is similar to David’s daughters one. She is still in the Temodar phase. I hope your friend can find something that works, we all do. All the best, Koda.

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Hey has anyone had problems with a wound not healing up? And CSF accumulating in the head causing ballooning or pouching? Just curious if there are any methods used that worked for them. Thanks.

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@ejacobs16

Hi everyone. Hoping to find some info on here. My best friend's daughter has a PXA grade 3 – not responding to treatment. Has had two tumor removal surgeries and is on nivolumab as last effort and that doesn't seem to be working. Very aggressive tumor. Radiation/Chemo – nothing worked. Anyone have any thoughts on last ditch ideas. She is at Children's Hospital of Philadelphia and they are talking about hospice. This beautiful kid just turned 18 and has brilliant future ahead.

Jump to this post

@ ejacobs16 hello I am so sorry your best friends daughter and the family have to go through this horrible nightmare. My daughter Danica has just passed January 28, 2019 age 10 years old from PXA grade 3. She had 14.5 hour surgery the first attempt to remove and they could only get 97% out due to it being on a blood vessel. Then she did radiation for 5 weeks and it still grew back. Then she was put on tafinlar chemo for 9 months and it shrunk to nothing but her next mri which she had every three months came back that it had grown back. We had to have a meeting with her team from Oakland children’s hospital to see what her next options were and the plan was a biopsy and try to remove what little they could since it was growing from vessel area and to make sure it had not changed. Then they would start her on two other chemos together. So October of 2018 Danica has her surgery set and days before that she had several quick scans and mri’s because she was getting nauseous like she had in the start of it all. Well scans showed bad news and it had doubled in size and she ended up having a 10.5 hour surgery and her removal was 75% we went home grateful she was alive and had no complications from another long brain surgery. But inside we New our options were down to this last treatment that were the two chemos. Sadly to say Danica’s rumor was out of control. We were sent home on hospice from a check up after being on the treatment for 2 1/2 months and nothing changed with medications. Our world crumbled and Danica’s pain at home was not able to be controlled we ended up back in Oakland children’s hospital for her last 8 days of her life and she passed. We caught with her for two years and it has torn me and my family up. I pray for your friends child and the family for strength love and a miracle with this kind of cancer because it is very rare and has no cure yet. Breaks my heart that our children have to go through this. 💔😭🙏🏻

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@daniellemarie

@ ejacobs16 hello I am so sorry your best friends daughter and the family have to go through this horrible nightmare. My daughter Danica has just passed January 28, 2019 age 10 years old from PXA grade 3. She had 14.5 hour surgery the first attempt to remove and they could only get 97% out due to it being on a blood vessel. Then she did radiation for 5 weeks and it still grew back. Then she was put on tafinlar chemo for 9 months and it shrunk to nothing but her next mri which she had every three months came back that it had grown back. We had to have a meeting with her team from Oakland children’s hospital to see what her next options were and the plan was a biopsy and try to remove what little they could since it was growing from vessel area and to make sure it had not changed. Then they would start her on two other chemos together. So October of 2018 Danica has her surgery set and days before that she had several quick scans and mri’s because she was getting nauseous like she had in the start of it all. Well scans showed bad news and it had doubled in size and she ended up having a 10.5 hour surgery and her removal was 75% we went home grateful she was alive and had no complications from another long brain surgery. But inside we New our options were down to this last treatment that were the two chemos. Sadly to say Danica’s rumor was out of control. We were sent home on hospice from a check up after being on the treatment for 2 1/2 months and nothing changed with medications. Our world crumbled and Danica’s pain at home was not able to be controlled we ended up back in Oakland children’s hospital for her last 8 days of her life and she passed. We caught with her for two years and it has torn me and my family up. I pray for your friends child and the family for strength love and a miracle with this kind of cancer because it is very rare and has no cure yet. Breaks my heart that our children have to go through this. 💔😭🙏🏻

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I'm sorry about what happened … My son died in 2016 from the same tumor, but not in all cases the same thing happens. There are patients with a very good prognosis. Since 2012 I have a blog where I publish information about this tumor. https://xantoastrocitoma.blogspot.com/

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@lmp1

I haven’t been on in a long time. Wanted to check see how everyone is doing? @daniellemarie how is your daughter?
Update on my daughter: her tumor is growing back for the 3rd time. The Temador and Radiation didn’t stop it. So now she is starting a clinical trial. She is doing well. Because she had a hole in her brain from her other 2 surgeries the tumor has empty space to grow in to. Her symptoms hasn’t started like before yet. All our hope is in in this clinical trial. We pray this will stop the tumor growing. It’s a combination of Avastin and another trial drug, if she gets the trial drug. They pick who gets at random by a computer. It isn’t a blind study so once she starts we will know if she gets both drugs. The Avastin could stop growth. Sure wish there was more paths to take to stop this cancer. It is evil.

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@lmp71 hello I am so sorry I have not been on in a while. My world has been shattered and I am trying to cope. January 28,2019 I lost my daughter Danica this evil pxa tumor. Danica ran out of options and what medicines that have been known to help shrink these kind of tumors. Hers just got overly aggressive and took over. She had tumor all over by the time she was sent home on hospice and having swelling nausea and losing right side functioning since her tumor was on left and front. She was sent home from Oakland children’s hospital on hospice on a Friday which hospice was not coming till that following Monday so she had been sent home with a couple strong hard core pain medications that I had to give her since her headaches became consistent and very painful. By Sunday morning at 3am I had to make the decision to load her up with my other children husband and my good friend and her daughter that Danica has over for sleep over even in the pain and condition she was in and I was letting her do and have what ever she wanted knowing her days were numbered. We got to Oakland and they admitted her and started her on pain meds and asked what we wanted as in her being home after they get pain controlled to pass there or hospital and we picked hospital because of so many reason. First it’s where she had her care, they can control her pain and her comfort and team would be there with us. Danica fought 8 long days till she passed at 2:49am on January 28, 2019. It was the worse and long 7hrs of my life that I had to watch and go through due to her labor breathing and sounds and being so powerless and hit with reality that my baby was leaving me forever. Today I am still not emotionally ok. My 7 year old who’s been here in home and shared a room with her is having a hell of a time and he is in grieving therapy. My 19 year has checked out and became completely detached as well as I have. I have to drag my self and push to get through each day for my kids and it has taken its toll. My life with out her is hell and I can’t believe she is gone.

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