Pleomorphic xanthoastrocytoma tumor (Pxa): help

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.

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.

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?

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

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.

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

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.

@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.

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

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!