Pleomorphic xanthoastrocytoma tumor (Pxa): help

Welcome to Connect, @lmp1.
I'd like to introduce you to @audrapopp. She, too, has the same tumor type as your daughter, Anaplastic Pleomorphic Xanthoastrocytoma. In particular, I'd like you to read this post from Audra on Connect http://mayocl.in/2rX8ue2 and more about her experience with proton beam therapy here http://sharing.mayoclinic.org/2016/04/01/a-new-weapon-in-the-arsenal-for-patient-with-stubborn-cancer/. I hope Audra will return to share her experiences with you and answer any questions you may have.

In the meantime, we look forward to getting to learn more about you and your daughter. I can imagine that you are very worried. What are the planned next steps? Surgery again? Radiation? Is proton beam radiation an option?

We will see a Neuro oncologist in 3 weeks. This will be the first time she has seen one. We have seen only the surgeon so far and of course all he talks about is another surgery. She doesn't want to do that especially since it's only been 4 months since her first one. There isn't a mayo clinic near us. I so wish there was. I have read about proton beam radiation. I hope it is an option. We are waiting to see what our next step will be. I hate waiting at a time like this!
I actually had read about Audra right after my daughter was diagnosed. It would be great if she could share and answer questions with me. Thank you so much!

Three weeks must feel like an eternity. It is so hard to wait. Is there a large cancer center near where your daughter lives?
Do you live close by?

First, thank you Colleen for connecting us... because it is such a rare tumor it is difficult, if ever to connect with others and I am happy to be an advocate, support and here for any questions you or your daughter may have. First of all~ Complete surgical resection is currently the best treatment option for this tumor type, that being said~ your best defense is truly "the best neurosurgeon" you can find! If I understand correctly she has not had radiation treatment or chemotherapy of any kind yet. I'm saddened they haven't connected you with a Neuro-Oncologist yet... sorry, but that alone makes me question her neurosurgeon. Second, what is the pathology report showing from her first tumor? Is it Anaplastic (Grade 3?) I can only assume it is since it has recurred so quickly. Have they tested for the BRAF V600e mutation? Many PXA's are showing it to be positive for this mutation and there have been a few reports of using targeted mutation therapy with BRAF inhibitors that are shrinking the tumors (Tafinlar & Mekinist drug combination or Veberafinib alone.) I have a lot more info on these if you need and have recently been on them. The BRAF mutation has been found in melanoma, lung and colon cancers and these drugs have been FDA approved for Melanoma so far. Unfortunately, since PXA is so rare my insurance company would not approve the drugs for me, even with an appeal...but luckily the pharmaceutical supplier Novartis granted me a one year supply of the drugs thru the Novartis patient assistance Foundation. If your daughter goes the drug route her MD needs to get applications started ASAP. My insurance process (application, denial & appeal) took well over a quite agonizing month! If she doesn't do surgery she may want to consider a biopsy to see if her pathology has changed since her surgery 4 months ago. I can't emphasize enough that she needs to be seen sooner than later by a well qualified Neuro-oncologist to get the best treatment advice going forward. It may mean getting on a plane..but trust me, it will be worth it. Mayo has an easy referral request process. My Neuro-oncologist sees patients both in Rochester, MN and the Mayo campus in Jacksonville, FL. I hope this helps and can continue the conversation. I'm sure you have lots of questions and I'm happy to answer.
With HOPE all things are possible, Audra

Thank you, Audra!!

@lmp1, let Audra know if you need any plain language interpretation of the mutations and treatment details that she shared. It's a lot to take in.
If you wish to talk to a neuro-oncologist and get a second opinion from experts at Mayo Clinic, please call any of our 3 campuses, http://mayocl.in/1mtmR63. You will be offered an appointment within 48 hours. Be sure to mention the Brain Tumor 48-access program. They can help you navigate your healthcare journey and answer your questions about treatment options.

To my knowledge there isn't a large cancer center. 2 hours from us is the Markey Cancer Center part of the University of Kentucky hospital. The MD she will see in July is the head of the nuero oncology department. I feel like we should have already seen him months ago but that is another story. I take care of her children while she works and we live in the same area. Im trying to be her support and helping her fight this in every way I know how. That is why I am constantly researching and asking questions. It makes me feel useful also at a time I can't just make this go away.

Yes thank you Colleen. I appreciate this so much!

She hasn't seen an oncologist yet. We are finally scheduled July 19. She is supposed to see the director of the Clinical Neuro-Oncology Research Program at Markey Cancer Center. So of course no treatment other than surgery.
As far as the grade of the PXA I don't think we have ever gotten a clear answer. I have asked and the last thing I was told that they really couldn't or wouldn't give it a grade as of yet. As far as I know it hasn't been tested for the mutation. Hopefully the oncologist can give us some of the information you are referring to.
So from what you have just ask me I feel like I don't know enough and I haven't asked the right questions yet but I will be finding this out as soon as I can. I'm so grateful to have a connection to finally get the right information to help me ask questions and help her get the best care possible. I honestly feel she needs to be seen at the mayo clinic but she wants to see what happens at this next visit and go from there. The medical bills have already been so bad and her insurance starts over July 1 so it's been really hard. As you know very well insurance companies control so much when you are sick. Just a sad fact!
I will be keeping in touch. I have a starting point now and that helps so much. Thank you so very much. Audra I so hope all is well with you and your treatment. Thinking of you and many prayers!

I understand your concerns but am so glad to hear she will be seen fairly soon. Waiting is extremely hard, knowing a plan always feels better for myself... it must be very hard as a parent of a young adult in this situation, but you do need to let her have some control and make the major decisions, and if she's ok with waiting, it's okay! It really sounds like they are getting her where she needs to be now. Nice your daughter has a strong advocate and support person on her side. I saw your FB messenger post but it has disappeared, feel free to try again. Prayers for you and your family as well. I was diagnosed at age 33, but they felt my tumor likely started growing when I had my first grand mal seizure at age 29- I am now 50! PXA's typically present with seizure activity. There are finally many advances, clinical trials and new treatment options being developed in the area of brain tumors, so keep the Hope! The past 2 years in May I have gone to Washington DC to advocate with the National Brain Tumor Society (NBTS) sharing my story and fight for increased funding for brain tumor research, the cancer moonshot initiative and reduction of drug costs. The NBTS is a good contact/website to search for information and they have recently developed a clinical trial finder for brain tumors on their site which your Neuro-oncologist will likely be aware of especially since they are at a research center. Another suggestion, she may want to inquire about meeting with a social worker either locally or with her Neuro-oncology appt. to see if there are any resources for her financially and to maybe start application for social security disability if she qualifies (Anaplastic PXA diagnosis falls under compassionate use and quicker approval.) Attending a brain tumor support group in your area is another great idea, calling the American Cancer Society 800# (they may have other resources available too) and I believe support groups are also listed on the American Brain Tumor Association(ABTA) website by state. Keep making connections and enjoy your time with those grandchildren!

Audra I read you have had 6 surgeries. Is your tumor coming back in the same place? In researching this I've read it can come back in your spine and other spots on your brain. How often are you doing MRIs? Are you on any kind of treatment right now?
Also something I haven't mentioned is my daughter has another tumor they called an acoustic neuroma. They didn't seem worried about it for now.
Thank you for your help again. I'm sure I will have more questions.

I have had 6 surgeries and feel very lucky to have had that option. The most recent was March 31, 2017 and completely cleared all traces of recurrent tumor, scar tissue from previous surgery and radiation necrosis. The original plan was to resume the BRAF inhibitor drugs Tafinlar and Mekinist combination after surgery (in clinical trials these drugs are literally shrinking the tumors), but I'm currently not on any treatment as my Neuro-oncologist Dr. Buckner says there is literally "nothing to treat" and I've previously had my maximum radiation treatment. I just had an MRI Monday and excited to say everything remains clear! I will have another MRI in 3 months, and have had previous MRI's scheduled anywhere from 1-6 months all dependent upon current treatment, symptoms or tumor recurrence. For example, after this last surgery my MRI's were day after surgery, one month postop, than 2 months, now as everything looks stable will have my next in 3 months.
PXA's typically return in the same area of the brain, most tend to be near the outer edge of the brain in the parietal/temporal area. It is actually very rare for the PXA to be found in the cerebral spinal fluid, which could than place it in the spine. There are only 2 known reported cases worldwide and I actually have personally met one of them as she is being treated at Mayo in Rochester, MN too and we have the same Radiation-Oncologist Dr. Nadia Laack.
I hope all this helps.
I'm certain your Neuro-oncologist will address the acoustic neuroma as well. I hope you walk away from your July 19th appointment with all questions answered and a plan you all feel good about.