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IDH-Mutant anaplastic astrocytoma, struggling with decision on next steps

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  • IDH-Mutant anaplastic astrocytoma, struggling with decision on next steps

    First off, thank you in advanced to anyone who reads or replies to this.

    Quick background: I am a 31 year old male with a biopsy confirmed IDH-mutant anaplastic astrocytoma, WHO grade III, in my left Pons, as well as other brain stem locations (details below). It is not resectable. The tumor has a non-canonical IDH1 p.R132G mutation, instead of the more common R132H mutation. I do not have 1p/19q-codeletion.

    Other specifics:

    The tumor is also present in my left cerebellar peduncle and has some extension from my left Pons into my right Pons.

    No CDKN2A/B homozygous deletion is identified, and the array pattern is not considered complex.

    The glioma cells show loss of ATRX expression, suggesting the possible presence of another, less common IDH1/ IDH2 mutation. There is widespread overexpression of p53 in the glioma cells.

    The glioma cells are negative for H3K27M, IDH1-R132H, and BRAF V600E.

    Scattered mitotic figures are identified (including 2 mitoses in a single high-power field), the Ki67 labeling index is up to approximately 5-10%, and there is no unequivocal microvascular proliferation or tumor necrosis (all immunohistochemical stains performed on block A1).

    A variant of uncertain significance was identified in gene KMT2D.

    This was first discovered in February 2019, when I was 29 years old. In that time there have been periods of slight growth, in millimeters, and also periods of no significant changes. My presenting symptom was headache from Valsalva’s maneuver, or actions similar to Valsalva’s maneuver. This prompted an MRI, which is how the tumor was found.

    Other than the headaches, at the time of initial discovery the tumor may have been asymptomatic. I now have minor symptoms, including horizontal double vision when gazing left and left sided facial weakness. The headaches are exasperated by working at a computer for extended periods of time, and long times off work (1 week or more) seem to eliminate the headache issue. In August of 2020 I was also diagnosed with a Chiari Malformation. My neurosurgeon believes the Chiari Malformation is coincidental, and my neuro-oncologist believes the tumor has caused Tonsillar Ectopia, and that the Chiari Malformation is not coincidental, but caused by the tumor. I also have head positional vertigo and balance issues, but the vertigo issue may have been caused or exasperated by a viral/bacterial infection that went around in my area around September of 2019.

    At this point my neuro-oncologist wants to begin radiation therapy, potentially proton beam therapy, and Temozolomide chemotherapy. There is not an emergency as far as starting treatment, but they would like to begin shortly after the new year. I understand that this is considered a “universally fatal” tumor, but I would like to give myself the best chance possible as far as survival.

    I have some concerns:

    First off, this has so far been a slow growing tumor. From what I have read, radiation and/or chemotherapy may hyper mutate the tumor, causing it to behave differently. I am afraid that it could become more aggressive after standard treatment. Radiation and chemotherapy may also make it more difficult for investigational drugs to work.

    Also, I don’t think my tumor needs to be shrunk. I can live with the symptoms I currently have. If I could simply limit or stop growth, that could buy me a lot of time, potentially without hyper mutating the tumor. Radiation and chemotherapy could then be saved for when there is no other choice.

    I have read about both IDH inhibitors, such as AG-881 and also PARP inhibitors. Are there any investigational drugs or clinical trials that I should look into before starting standard treatment? I want to make the right choice and I do trust my doctors, but I feel that I am simply being put through the flowchart, which starts with radiation and chemotherapy right off the bat. If that is truly the best choice, then that is what I will have to do, but I want to make sure there are no better options before doing so. I know I cannot receive medical advice on an internet forum, but any thoughts or suggestions that warrant my further investigation are appreciated.


  • #2
    Did you sign up for our patient navigation program? That is a really hard question - which is why nobody else responded. Our team of experts are good at this sort of problem.


    • #3
      Hi Al. Do you know if I can use this service if I'm not an US resident but willing to travel to anywhere in the US?


      • #4
        Yes. In the comment box tell them you are an old friend of Al Musella..


        • #5
          Thank you Al. I am currently waiting for my next MRI and hope I won't need it but I feel better knowing there is an option like this one.


          • #6
            God luck on the next MRI


            • netopolis
              netopolis commented
              Editing a comment
              Thank you!

          • #7
            I will take a look at the patient navigation program, thanks!


            • #8
              Our patient navigation program is unlike any other program in the country. It is listed as a trial on Our team of experts generate a list of the best plan options for you, and you get to decide what you want to try. Then we follow you throughout the journey and learn from every patient. So you have to agree to allow us to follow you as long as needed. You do whatever treatments you want, and we will try to help you get access to them if we can.


              • #9
                That sounds like a good program. One question, since the patient navigation program is a clinical trial, would participation disqualify me for other clinical trials? I have noticed in certain clinical trials that one of the exclusions is participation in another clinical trial.



                • #10
                  Good question. That has not happened yet. We get a lot of our patients into clinical trials and nobody every questioned that! I think they mean any other clinical trial that is treating you. Ours is more observational.


                  • #11
                    does anybody here have experience with carbon ion radiation therapy? would be great if you can share some information...


                    • #12
                      ive been told from various experts that all the early data indicate that this is a superior treatment compared to other radiation therapies.


                      • #13
                        I have read of better results with carbon ion compared to standard radiation but it is hard to get, expensive and only a little better.