Optic Nerve Sheath Tumor

[Andrea Havill]

My 55 year old mother had a second crainiotomy to remove her optic nerve (left eye) which housed a benign tumor in November.  Her first surgery was 2 1/2 years ago, left her blind in the left eye and partial vision in the right.  Apparently a substantial amount of the tumor was not removed during the first surgery. (Symptoms such as pressure, dizziness and protruding eyball led us to a new set of doctors and thus second surgery.) The first surgeon called the tumor a "benign meningioma", the second an "optic nerve sheath tumor".  Is there a difference?  Further, her doctors within the last week have determined that a small portion of the tumor remains behind the eyeball and that radiation should be the next step.  I trust her current doctor, but feel in dire need of good reference books, articles, etc. that that would help explain her tumor including diagnosis, appropriate therapies, quality of future life, etc as well as allow me to ask more informed questions. Do you have any suggestions?  And finally, the dizziness which began about 6 weeks prior to the second surgery persists; in particular, when she gets out of bed or stands up quickly (same as before surgery).  The doctors don't have an explanation; Do you?  Any comments, suggestions or advice is greatly appreciated.  Thank you.

[CCF Neuro[P] MD, RPS]

Dear Andrea Havill:

I am not sure what to tell you as the diagnosis of the tumor dictates what I tell you.  A meningoma is a possibility but these are usually singular in nature and would only be in one area.  The vast majority of the time they do not require removal of the eye.  What you are describing to me sounds more like a glioma, such as a pilocytic astrocytoma.  That would cause a sheath tumor, or something like a oligodendroglioma.  Find out the exact pathology of the tumor resected.  Meningomas grow so slowly that repeat surgery so soon would not be the usual course unless it has turned malignant (only in a small number of cases).  Find out the pathology and let me know.

Sincerely,

CCF Neuro MD

[Andrea Havill]

Sorry about the confusion.  It is a meningioma that happened to be located in the optic nerve. It was not malignant and the second surgery was the result of the first surgeon removing only a small portion of the tumor, located close to where the optic nerves cross, 2 1/2 years ago.  Why, I'm not sure.  But symptoms such as pressure behind the eyeball, proptosis and dizziness caused my mother to be concerned, switch to a new set of doctors (opthamologist and neurosurgeon) and thus the discovery that a large part of the tumor, located directly behind the eyeball, had not been removed.  During the second surgery in November, the nuerosurgeon reomoved the entire optic nerve which housed the tumor.  A recent MRI showed that part of the tumor remained behind the eyeball and he recommended radiation. Back to my concerns. I think I've answered my own question concerning the "difference"; I would still be interested in good reference material specifically geared toward her type of tumor located in the optic nerve,i.e. what is the prognosis considering they removed the entire optic nerve, what does this mean for her right eye (which has about 85% of vision), what is the chance of recurrence?,etc. I would also like to know the general effectiveness of the proposed radiation (they have talked of using a gamma knife).  And finally, the dizziness that began before surgery that still persists 2 months post-op is a concern.  I hope this clears your questions.  Again, sorry for the confusion and any comments are greatly appreciated.

[CCF Neuro[P] MD, RPS]

Dear Andrea:

A meningoma is a very slow growing tumor.  The fact that it returned or presented with symptoms so soon after surgery would mean that either they did not get it all or it turned malignant. I would think that it is the former from what you have indicated.  This is a very difficult to remove all the tumor cells.  What did your neurosurgeon tell your mother?  What sort of prediction did he/she give?  You are putting me on the spot without any films, path reports etc.  The opinion of the surgeon is far more valuable than my internet opinion.  Didn't he/she provide your mother with information?  You can go to any medical library and find the basic information in any text on pathology and neurosurgury.  Meningomas occur in a variety of areas, one of which is in the optic chiasm.  They are singular in nature, meaning that they do not form in multiple areas unless you have radiation or another syndrome (such as NF-2).  They are not invasive but they can become maligant.  There are specific types of meningomas that contain steriod receptors which some think may lead to their growth.  However, whether you mother's is this type, only the pathologist can tell you (what did they say?).  If the tumor is steriod responsive then growth may be controlled by anti-steriod treatments(estrogens or progesterone).  Gamma knife may be an alternative, but I am not sure if the recurrence data is well known.  A lot depends on where and who does the gamma knife treatment.  Since surgery could not get rid of the meningoma, I am not sure what to tell you about gamma knife surgery.  Certainly, it will help but whether the degree of tumor removal will be great is unknown to me, it depends on what they see on MRI.

sorry, I'm not much help, but my hands are tied.

Sincerely,

CCF Neuro MD

[C Jenkins]

Dear Andrea,

I have NF-2 and have had schwannoma removed from my optic nerve and the orbital floor of my left eye.  A craniotomy was performed 8 mos ago.  Proptosis remains and I pray it is attributed to scar tissue and not the other.  I too, have dizzy spells, which I think are common when your eyes are messed with, more especially when I first wake up in the morning and when I am tired.  I hope all goes well with your mother and pray everything turns out for the best.  My thoughts are with you.

Sincerely,

C Jenkins
(***@****

[CCF Neuro[P] MD, RPS]

thanks for your comments (as usual)

CCF Neuro MD

[Troubled]

Dear CCF Neuro RPS:

I'm troubled.  (as usual)  My mailbox is full.  Most are troubled people.  Troubled with earthquakes and aftershocks.  They've tried to post questions but have given up hope of ever doing so.  If it isn't dealing with what to expect post-viral, it's about their pre-craniotomy fears or post-craniotomy nightmares.  I don't have a problem with explaining to a worried son about the step-by-step "what-to-expects" of his mother's up-coming craniotomy.  In fact, it helps me... and it's even better still hearing that the surgery went flawlessly and that his mother isn't going to go blind.  Or... the question, "I can't walk straight!  Why can't I walk straight?  I stumble.  Can't pick up my feet!  Did something go wrong?"  So... (as usual) those with NF seem to litter the mailroom floor wanting to trade experiences or delve deeper with an array of questions about their own up-coming tumoral excision problems.  They know that I'm not a physician, so they lean for support... and I'll continue to support because they need it.  If every one of those people could have gotten through to post a question about their troubles or to inform they were troubled you would have been troubled.  I thank my lucky stars everyday for being given experience because it helps all the way down to what medications are decent for controlling pain.  Therapy is therapy, no matter.  I'm there.

Christine

[CCF Neuro[P] MD, RPS]

thanks for the note.

CCF Neuro MD

[David Loud]

A colleague of mine has recently suffered from a tumour to the petuitary gland. The swelling caused by the tumour has resulted in permanent damage to the optic nerve causing complete loss of vision in both eyes.

Is there anything that can be done to try and repair damage to the nerve.

This is a guy that went to bed with a headache and work up blind. Any detail of possible remedies would give hope.

David

[CCF Neuro[P] MD, RPS]

Dear David:

Alot would depend if the optic nerve changes were due to edema or the tumor itself.  If the nerves were compressed by the edema, there might be hope for some recovery,  However, if many months have passed then likely not.  If the tumor had actually invaded the optic nerve, then likely there will be little recovery.  sorry about the news.

Sincerely,

CCF Neuro MD

[C Jenkins]

Hey Doc...

I'm at a crossroads.  I couldn't have the implant surgery done due to insurance discrepancies.  Is it or is it not "cosmetic".  Is it or is it not "pre-existing".  All I know is my left eye doesn't close properly... and would if these jackasses would come to their senses.  I made good arguement the other day about NF and it's standing in the "pre-existing" department.  I cannot help that NF is pre-existing.  Since my eye didn't close properly before the craniotomy... the situation is considered "pre-existing".

So... here I sit, right now, looking through  a watery mess caused by erythromycin antibiotic ointment and healthy doses of Lotemax drops.  I'm forced to wear my eyepatch.  Cute as it is (I made it myself... there's a rose on it), it's frustrating.  Try patching up your eye and watch (1st hand) your peripheral vision and depth of field disappear right before your eye.  Forget driving.  I tried.  Ever heard the word "dangerous"?  It is.

I really don't have a question save for how long will it take to totally wreck my cornea to the point where the insurance company refuses to fund a transplant?  I guess I'm just angry and frustrated because I need the implant every bit as bad as we humans need water!  I scratch my eyeball at least twice a week.  The scratch feels like there's something stuck in my eye and at one point not too long ago, I had to wear a contact in order for the corneal "scratch" to heal properly.  As a physician, you know "that ain't good"... especially when it happens time and again.  I'm doing all that I can... and correctly, to lessen the damage.  Proptosis isn't an asset, it's a liability.  More eye surface is exposed.

Now, my situation is to prove "pre-determination".  Oh... you better believe I'm determined to have this done.  I hope I haven't bored you to tears.  Lord knows I have enough of them running unconsciously down my cheek.  Anyways Doc... thanks for allowing me to vent my frustration.  

Truly sincere,

Christine

[CCF Neuro[P] MD, RPS]

Dear Christine:

Sorry to hear about your problems.

CCF Neuro MD

[C Jenkins]

Dear Doc RPS,

I know you are.  Thanks for the shoulder to lean on and ears to hear the frustrated "babble".  You're a great Doc.

Truly sincere,

Christine

[CCF Neuro[P] MD, RPS]

your welcome.

CCF Neuro MD

[Karen very worried Mom]

Hi,
Never done this before.  Let me get to it.  My 3year old daughter has NF-=1.  Just diagnosed with an optic glioma.  I have three Dr.s telling me three different things.  But they all end up saying "Don't Worry, very rarely does anything happen." I really need to know if I don't need to worry.  I am very scared and my husband is very nervous.  I don't know much about optic gliomas and what they really can do.  How rare is rare if something can go wrong?

We would appreciate any advice.
Thanks,
Karen

[CCF Neuro[P] MD, RPS]

Dear Karen:

Sorry to hear about your daughter.  We are a center for NF-1 patients so we see alot of children and adults with this disorder.  There is a spectrum of optic gliomas in NF-1.  Most are benign (a loose term in this case meaning not an aggressive tumor) pilocytic astrocytoma.  There are very, very slow growing tumors and usually we just watch them. Usually a yearly MRI. There is a very small minority that can transform into a more aggressive tumor and these can cause severe problems.  We have no way to detect which ones will transform.  So, likely all three opinions were is some way correct.

Sincerely,

CCF Neuro MD

[MN_Chu]

A related discussion, 9 years old boy with Bilateral Optic Nerve Glioma was started.