NF2 patients have different problems

[antoinette]

We are not all equal in sheltering this AN. The response to treatments could be different if you are NF2.
Radiation may provoke the worst fastest growth instead of  killing the tumor.
Surgical ablation may be followed immediately by regrowth.
And if great concern is not used right from the start of treatments, the patient may end up deaf of both ears.
Is it safe to wait as long as possible? That is not sure. Only a forum offered to NF2 patients will help them make those successive decisions. We are facing recurrences and, as a dear friend learned over 11 years, she had several successful surgeries, 7 in all, and even tried radiation, but so much was lost in the process.
We need an NF2 "main" topic.

[Windsong]

Would you be kind enough please to quote any sources for your saying these comments?  are these your opinions or are they from some recent studies?

Thank you.

[russ]

Hello;

  I have NF-2 and auditory, vestibular, and spinal tumors. Thus far my face is spared the VIIth cranial nerve not affected yet.
  It has pretty well decimated my life. Documentation at about any good web site Re NF-2 shows it more Tx resistant.
  The most likely outcome is bilateral deafness.
  I've had two I know die from this. Another is on J-Peg feeding and wheel chair bound. All three have/ had multiple surgeries as had the Wishart variety of NF-2. Mine is the Gardner variety and 'somewhat' less aggressive.
  NF-2 is always a 'wild card' in terms of AN and their TX and Px. My statements are not opinions.
  Take care.

  Russ Korte
  M.S.H.S

[Jeff]

Hi Antoinette,

You are right that NF2 is a different ballgame. After I was diagnosed with NF2, I spent a great deal of time worrying about hearing preservation, but my priorities have since shifted to preservation of facial function, learning to speech read, and other quality of life issues beyond hearing loss. The fact is, and I don't mean to downplay the life altering consequences for anyone who has to deal with an acoustic neuroma diagnosis, NF2 patients often suffer the most serious problems as a result of the various other tumors that they can have, particularly spinal tumors. Unfortunately, bilateral deafness is not often avoided. Rates for positive outcomes of interventions for vestibular schwannomas, both surgical and via radiation, are typically not as good as those for patients with unilateral tumors. For example, here is a link to a recent study regarding GK and NF2: http://jnnp.bmjjournals.com/cgi/content/full/74/9/1288 Look not only at the percentage of patients who require further intervention after GK, but note the number of patients who die (15 of 96) within 5 years of radiosurgery. Clearly, this is a daunting condition.

Because NF2 outcomes are not comparable to those with unilateral tumors, I make it a point not to share the outcomes of my treatment choices or the choices of my family members with NF2, both surgical and radiosurgical, as our outcomes could be misleading to those with unilateral tumors. I do try, however, to share my experiences in dealing with the issues caused by these tumors.

Thank you for thinking of those with NF2. You are truly kind.

Sincerely,

Jeff

[russ]

Hi Jeff and Ant
  I've asked the ANA Director to create an area on this web site just for NF-2 but do think it unlikely as may take a complete web site redesign.
  Ant has placed her wonderful concern and comment right at the top of the page in the F.A.Q. Section and I think that good. I'd like to further hi lite this area if you know how.
  I'm unsure if there are others on the board who have NF-2.
  Do take care Jeff!
  Russ

[Windsong]

Hi.

Thanks Jeff for that site link you included. I read through it all. They had a lot of detail included and it was interesting going through it all. By the end of it I saw that even with nf2 it  said that 50% who had had stereotactic treatment either had the tumour slowed or controlled.  Problem seeming to be that nf2 means other tumours growing not just the VS one. There was, of course, lots of other information interspersed throughout, hearing preservation included, but i also privately noted that with nf2 ,given the nature of it, hearing is only one thing in a whole bunch of things....



I was looking but didn't see anything about what type of stereotactic radiosurgery was used and i was hoping to see how many doses, how long, how many rads etc, but i guess that was not the purpose of that particluar abstract...

I didn't know that St Bart's was doing such close work with An's never mind NF2 so that was interesting in itself. Good that they are.  Now I wonder do they have a CK machine? Simply am curious....

My heart goes out to anyone who has nf2 and deals with not just one but more than one VS and perhaps tumours elsewhere in the body such as spinal.

 I thank you so much for taking the time to post this and the link.  I do appreciate it.

Wishing you and all others the best.

Thanks again for posting this.

[thecakes]

    Please expain to me just what NF-2 is.  I've read much about it and to me it seems that it is 1 tumor on each side of the head.  Are the signs of this condition any different than a AN? MY AN caused a fast lose of hearing.  The surgery took away all the hearing in that ear.  If I would have NF-2 I guess I would be death now.      the cakes

[Mark]

The cakes,

I know there are several members of the board community who are NF2 and can answer your question in much greater detail than I can, but I'll offer a general answer. NF2 is a genetic condition that results in the frequent formation of nerve based tumors. The large majority of AN's are spontaneous in one ear and that is it. Folks with NF2 are more prone to having AN's occur in both ears over time and also the development of tumors in the spine and other areas of the body. Again, I'm sure there are several folks who monitor this board who can give a much more comprehensive answer than that

Mark

[Mark]

One of the discussion points for those with NF2 is whether radiation is a viable treatment option. I don't have NF2 and have not researched it, so my knowledge of it is really based only on the posts I see here from those who have it. I went ahead and posted the question about radiosurgery for NF2 over on the CPSG board out of curiosity and thought I would share the response for those who are interested:

my post


Doctors,

On the acoustic neuroma patient board there has been some discussion about radiosurgery being less effective on AN's for those with NF2. I'd be interested in any opinons of what treatment guidance you would give to an NF2 patient. Is radiosurgery a viable option and are there studies that substantiate that or is surgery the preferred choice in such situations to achieve tumor removal and control

Thanks much

Dr. Medberry Response

Although you sometimes see statements that NF2 patients do not respond as well to radiosurgery, I can find little to support that contention. Pittsburgh showed 98% control and Munich showed no difference whether NF2 or not. Radiosurgery, in my opinion, remains the treatment with the least morbidity and with control rates at least as good as surgery.

Clinton A. Medbery, III, M.D.
St. Anthony Hospital Cyberknife Center
(405) 272-7311
buddy@swrads.org or cmedbery@coxinet.net

[antoinette]

It is imperative that we make room for NF2 in this forum, Not lost among other posts and getting little sparks of knowledge not necessarily applicable to their case. But the need is great to gather all the information available under one topic.
I will get some links for you to get an idea of what is known about NF2 and also what is not. I must get a bit more practice with this e mail system. I am still getting lost and wonder what I could do to improve on it. The best way would be the addition of an "main" NF2 topic designed by the management of this site.
Thanks Mark for a very good explanation. You also know there is more to it.
And we will figure a way to get all of it together.
ant

[Mark]

As a follow up to my post yesterday with Dr. Medberry's comments on effectiveness of Radiosurgery on NF2 AN's, below are some of the  studies he referenced which he was kind enough to provide. Again, just offering it as information for those who are interested

Mark

Here are a couple of references:

Wowra B, Muacevic A, Jess-Hempen A, Hempel JM, Muller-Schunk S, Tonn JC. Related Articles, Links
Outpatient gamma knife surgery for vestibular schwannoma: definition of the therapeutic profile based on a 10-year experience.
J Neurosurg. 2005 Jan;102 Suppl:114-8

Kida Y, Kobayashi T, Tanaka T, Mori Y. Related Articles, Links
Radiosurgery for bilateral neurinomas associated with neurofibromatosis type 2.
Surg Neurol. 2000 Apr;53(4):383-89; discussion 389-9

Subach BR, Kondziolka D, Lunsford LD, Bissonette DJ, Flickinger JC, Maitz AH. Related Articles, Links
Stereotactic radiosurgery in the management of acoustic neuromas associated with neurofibromatosis Type 2.
J Neurosurg. 1999 May;90(5):815-22.

Clinton A. Medbery, III, M.D.
St. Anthony Hospital Cyberknife Center
(405) 272-7311
buddy@swrads.org or cmedbery@coxinet.net

[russ]

Hello
  Re Irradiation of NF-2 mediated ANs, I know as a pt. that Mayo Clinic and The University of IA Hospitals and Clinics would beg to disagree with Dr. Medberry. So would HEI.
  My Mayo Neuro does traditional microsurgery and GK as well. He states results obtained from GK on NF-2 mediated ANs have a lower % of success.
  Two problems with radiation OR surgery of NF-2 ANs; 1. The defective chromosome with absent gene are always present within the body.
  2. Only in NF-2 caused ANs do they have the ability to impact a nerve and grow within it. This is not always visible on MRI. Unilateral ANs always grow laterally on the nerve.
  Best wishes!
  Russ

[Mark]

On the issue of radiosurgery for NF2 AN's, Dr Chang also responded to the inquiry I asked on the CPSG board which I'll post below. His experience at Stanford appears to echo some of the comments Russ shared from his Mayo and Iowa doctors

Mark

Our experience at Stanford is that the NF2 acoustic neuromas are somewhat harder to control both with surgery and radiosurgery. We use a higher dose for NF2 patients that we do with solitary acoustic neuromas and attempts to achieve tumor control.


--------------------------------------------------------------------------------
Steven D. Chang, MD
Assistant Professor
Department of Neurosurgery
Stanford University School of Medicine
300 Pasteur Dr., Room R-225
Stanford, CA 94305
Tel (650)723-5573
Fax (650)725-5032

[vjharris]

Can I ask those with NF2 if you were diagnosed because you had bilateral ANs? I have an AN and my daughter was born with a posterior subcapsular cataract. When I read of the connection, I asked three different Drs. if we could have NF2. They all said not to worry because my brain "did not look like an NF2 patient". I don't know if they meant that I didn't have two AN's. I have heard about people getting the second AN later. Anyway, my (now 20 year old) daughter just got her eyes checked and now she has a cataract on her other eye. Two of that kind of cataract means something is very wrong. I don't know how to get us tested. I'm not sure how I could get a referral from our PCP if she has no tumor symptoms.
Thanks for any input,
vjh

[antoinette]

Hi vjh,
It is not easy to point to any test proving NF2. Some exist but they are difficult. We all dream that this would be discovered soon with a simple blood test.
You must insist with your present physician to look into this possibility of NF2. Or, if necessary consult another too. And you must not worry meanwhile, it would be damaging to you and your daughter.
i will come back to you on this with some links.
antoinette