My six-year-old son, diagnoed with NF1 several years ago, developed a rapidly growing brain tumor from May 2000 (negative scans) to November 2000 which showed a chiasmatic/hypothalamic mass. I understand this to be an optic glioma of the left optic nerve, progressive, also written as optic pathway tumor/chiasmal glioma. (I have all copies of medical reports).
This is stated:
"...neurofibromatosis of overwhelming pathology....it is benign infiltration of tumor along the optic pathway system. Since it is infiltrative, it is not surgically amiable to biopsy or resection without causing significant morbidity and potential mortality."
It was told to us also that radiation would not be an alternative as this will lead to leukemia in an NF patient.
I understand optic gliomas (low grade astrocytomas) are rather slow growing and tend not to create any huge problems, although in my son's case, we have definite radiographic progression since may of 2000 until now, yet his vision checks all look good except for the fact that he has developed a left eye exotropia approximately one-two years intermittently prior to tumor diagnosis (or present when all brain scans were negative for tumors) and just before the november, 2000 tumor diagnosis his right eye began to intermittently wander out which never occurred before that.
My question is, in looking at this brief description and knowing what you would about optic gliomas, the optic pathway, progresive tumors, etc...and a sudden development of the right eye exotropia although no visual loss at this point - 20/30 in both eyes approx. 4 months since diagnosis - what would your prognosis for a situation such as this be?
I understand we have an option of chemotherapy at this point, but with it or without it, what would be your answer to my question in this case?
Please don't ask me to refer it back to my son's specialists as I have done that as well as constant study and research. I find many different situations concerning optic gliomas...in this case, with infiltration and progression, what is your opinion? (Let me add that we have been told that with treatment and barring any complications the time we have with our son is approximately 5 years). Would you agree or disagree with this? (Let me add the number itself is insugnificant but approximates are ok and prognosis from someone who does not know of nor has ever seen my son).
It is very much appreciated as we are dealing with multiple medical problems with our son and the decision on chemotherapy alone is one that is being taken very seriously due to his many problems.
I appreciate your quick response and thank you for your time in reading my post.
I am sorry to hear about your son. I am not sure what you want from me, as it sounds like you have your mind made up, the diagnosis in hand, researched the problem, and talked tomany people concerning the problem. Opinions are a dime a dozen and you will find conflicting ones with any diagnosis.
I would treat the optic glioma when it began to compromise vision. Radiation question would not be out, one can consider the gamma knife and many times radiation is center specific. I would never tell anyone they have 5 years or what ever, I am not God and know no one who is (although many claim to be). I don't trust most and would view the MRI myself and have those I trust to give me their opinion. I can't tell you the prognosis-your information is imcomplete.
I appreciate your reply. I suppose I thought I would receive one such as yours, but with all going on was looking for some hope in this whole scheme of things.
In fact they have wanted to begin treatment as far back as December on this but we have decided to treat this conservatively as our son's vision is still good although the exotropia problems are there.
I suppose the first sense of any real change on scans (as he is scanned and observed opthomology and neurological frequently), we will then decide to go with the chemotherapy and that is dreaded.
I see you have mentioned radiation, but I am hearing from peds quite often that they really don't like to use it in an NF patient although effective, because of the long term effects and the leukemia it causes in NF children.
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