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http://www.uofmchildrenshospital.org/Providers/Bio/D_121128
1) Many
patients with Schwannomatosis are very interested to know, is
progress being made in the treatment and understanding
of Schwannomatosis? If you are aware of particular strategies,
medications, or practices that are increasing the quality of life for those
with Schwannomatosis, I'm sure our group members with the condition would
be very interested to know.
There is significant research underway regarding schwannomatosis
and Neurofibromatosis, type 2. The Children’s Tumor Foundation
(http://www.ctf.org/Research/Synodos.html) has initiated an NF2 “Synodos” group
that is focusing on effective treatment . According to CTF, “This group of
talented researchers come from varying backgrounds – from basic science, to
translational science, to clinicians – and have joined together to break down
barriers, proactively leverage collaboration and shared knowledge, and work
together to develop effective new treatments that will end NF2.”
In addition, there is a great deal of cross-fertilization
among researchers studying the schwann cell disorders, including NF1, NF2 and
schwannomatosis. We have elucidated some of the genes responsible for
schwannomatosis. This is the start of a process that will eventually lead to
the discovery of drugs that will effectively target these genes or their
products. This era of “targeted therapies” is a very exciting time in medicine
– people and families with schwannomatosis and related conditions should be
very excited about what’s coming down the road. It is also an important time
for effective advocacy – letting your legislators know about how important this
research is to you, telling your human stories, will preserve or increase
funding for important research regarding schwannomatosis.
2) A group
member with Schwannomatosis remarked recently that she had symptoms
such as left side headaches, facial pain, and sensations like being stabbed
with a knife above the eyebrows and on the neck. These symptoms
predominated on the left side, and to her surprise doctors found a Schwannoma
at C1, C2 on the right side. The tumor was removed and, surprisingly, her
pain and symptoms were gone. In your medical experience, is it common for
referred pain or nerve symptoms to be present this way? Her own surgeon
found this quite unusual...
It is an interesting phenomenon that we see in NF2 and
schwannomatosis. As a result of nerve
damage from schwannomas, some signals, such as pain, will be re-routed. We even
have one young lady who sweats around her ear every time she eats starchy food!
This phenomenon, known as Frey’s Syndrome is a result of the nerves stimulating
salivation being rerouted to the skin around her ear.
3) I think
many people in our group would be interested to know, how common is it that you
find that people need medication after having had even just a single
schwannoma, even when the tumor was successfully removed? For many of us,
the pain, pressure, and post op nerve symptoms can be quite significant - and
for some this persists almost indefinitely. People express dismay at
still needing pain relief. Do you find that this situation is relatively
common or uncommon?
This situation is extremely common. If a schwannoma is
removed because of pain, the pain may frequently persist for months after
surgery. We also know that schwannomas may grow at points of nerve injury
because of the basic genetic defect in schwann cell growth. Attention to means
of preventing tumor growth after nerve injury and effective treatment of post-operative
pain is an intense area of research.
Surgeons dealing with patients with schwannomatosis should be extremely
knowledgeable about the condition – an uncomfortable surgeon makes for an
uncomfortable patient. Likewise, both patient and surgeon should have
reasonable expectations for surgical outcomes. Don’t be shy about asking for
second opinions – it’s your body and your life.
4) We would
be very interested to know if many of your patients have had radio surgical
treatments such as Cyber Knife - and if you find that it is proving successful
in the cases you have seen?
I generally discourage radiotherapy for my patients unless
it is a last resort. Radiation can cause cancer and can complicate schwannomas
– especially in young people (under 60).
Cyber Knife is another word for gamma knife – both are “brand name”
forms of stereotactic radiotherapy. Tomotherapy, CMRT and proton beam therapy
are other focused forms of radiotherapy.
5) As you
might expect, we have had group members who have suffered extreme facial
paralysis as a result of surgeries for schwannomas - commonly experienced after
acoustic neuroma surgery. Do you find that there are particular
strategies for regaining and strengthening facial abilities for people who
exhibit these symptoms? What are the medical management strategies that
work best for those who have these kinds of facial paralysis?
Once facial paralysis occurs, it rarely recovers fully. Some
patients get their facial nerve (the culprit) stretched during surgery and the
post-operative facial paresis usually recovers fully. However, if the nerve is
cut or sacrificed during surgery, recovery will not happen. The surgical
statistics are pretty clear for patients undergoing surgery for
vestibular/acoustic schwannoma regarding those who are at higher risk for
facial nerve injury based on tumor size and pre-operative nerve function. The
surgeon doing the procedure should be experienced and should be able to give
you his personal statistics so that you can judge what risk you are exposing
yourself to. I make sure my patients go to teams of neurotologists and
neurosurgeons who are used to working together and have lots of experience. I
am blessed to have great teams at my institution who provide patients with very
clear evidence of outcomes. We also have a clear definition of who would
benefit most from medical therapy, such as bevacizumab (Avastin) – and can
offer other hearing preservation technologies such as cochlear implants or
brain stem implants.
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Disclaimer: Dr. Moertel and University of Minnesota Masonic Children's Hospital assume no liability whatsoever for the comments or advice offered in the content of this blog. Dr. Moertel has offered his advice generally on the issues relating to treatment of Schwannomas - however Dr Moertel and the Schwannoma Survivors & Schwannoma Fighters group always insist that a person should make all major medical decisions in consultation with one's own physician.
