Re: Neurofibrosarcoma [00054] Medicine On Line

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Re: Neurofibrosarcoma

On Thu, 21 Sep 1995 wrote:

I had a neurofibrosarcoma that was
> removed with my left jaw in Feb. 93  it reocurred as a 1 x 4 cc brain tumor
> in the V3 cranial nerve last June and was treated with gamma knife on July 17
> 1994.  Since then MRI's in Oct. 94 and Feb. 95 have indicated that the main
> tumor has stabilized or shrunk slightly but additional loss of facial muscle
> function and a MRI in Sept. indicate that fingers of the tumor continue to
> grow.  


I have Malignant Schwannoma which is another name for what you have.  
Lorne Buhle is correct in talking about a link between neurofibrosarcoma 
and Von Recklinghausen's Disease and you should be checked for that.  
Don't worry you probably don't have it, because it is a rare genetic 
disorder.  If you have any really unusual flaps of skin or "skin tags," 
then make a point of being referred to a qualified dermatologist.  Again, 
this is a very minor point, but something to keep in mind.

With Schwannoma, or any other type of rare cancer, it's important to 
become an expert and take charge of your medical treatment or find a 
trusted friend or relative to do so and do so now.  This is because 
sarcomas are nasty cancers and they need to be hit and hit very, very hard.
Now, I know the question your asking is: "Alright, WHAT do we hit it hard 
with?"  That's were it can get tricky.  The vast majority of these kind 
of sarcomas occur in the limbs so the conventional wisdom is surgery, 
followed by high intensity radiation.  But for me, who has Schwannoma in 
the trunk and you in the head, this can be problematic.

If radiation can be applied to the brain (and I don't know what the 
limitations are there) then the highest possible dose should be given, 
ie. 600 gy or so.  But again, you'll have to check with your doctors, I 
know nothing about the special circumstances of the brain in cancer 

The watchword is: "If you have to damn near kill me to keep me on the 
green side of the grass, doctor, then do it."

Some doctors get very squeamish when talking about sarcoma for several 
reasons.  First of all, it is a nasty cancer which is not very responsive 
to a lot of the standard treatments, but there is good news (see below).  
Second, and this is the real reason they are sqeamish, there is not a lot 
of data out there on Soft Tissue Sarcoma, let alone Schwannoma!  But 
here's where the good news comes into play. When I was diagnosed with 
this about three years ago, three of Canada's finest oncologists said 
"Mr. Burns, we can't be sure of many things in life but we can be sure of 
one thing: 'Chemo does not work in soft tissue sarcoma!'"

Indeed, if you look in the standard cancer textbooks chemo and soft 
tissue sarcoma come up a big, fat zero.  A few years ago, the world's 
largest study on Ifosfomide and sarcoma (only about 1000 patients, 
incredibly small compared to many studies, just because not many 
people have this cancer) there was not a shred of evidence to show any 

Except that the same chemo gave me a complete response and remission for a 
year, it worked marvelously.  I am now on another kind of chemo and it is 
working, something else that definitely wasn't supposed to happen.

In talking with several Schwannoma people, there seems to be a firm 
pattern: there is NO pattern.  So, forget the stats, ignore the naysayers 
and drive this monster out of your body.

I recommend chemo and the more the better.  The standard therapy for 
sarcoma is called MAID.  It stands for:

M esna -       a drug that protects your kidneys from Ifosfomide.
A driamyacin - one of the "big guns" in chemo for many cancers, including 
Ifosfomide   - another "big gun."
D TIC        - another chemo drug.

I think it's also important to consider high dose chemo or an autologous 
bone marrow transplant.  Roughly (and this is very rough) high dose chemo 
is where they give you a drug called Nupregen to "build up" your immune 
system after the beating it takes with high-dose chemo.  An autologous 
bone marrow transplant is where they take some of your bone marrow 
out, then they give you very high dose chemo, and put your bone marrow 
back in.  They take the bone marrow out to protect it from the chemo and 
put it back in to keep you alive.  Thousands of people have this 
procedure done every year and they're fine today.

Unfortunately, there is a problem associated with Adriamyacin, one of the 
most successful drugs in sarcoma; heart damage.  Patients can only 
recieve so much Adriamyacin in their lifetime because the effects on the 
heart are cumulative.  However, new drugs have been developed which 
protect the heart and do not dillute the power of the chemo.

This isn't a picnic, but you'll survive it, I did, with complications, 
but then I'm not promising you a rose garden; I'm just offering a 
layman's opinion, albiet a somewhat war-weary layman.

I've had:
          5 thoracic surgeries
          1 month of radiation
          2 bouts of chemo

I have some regret that I didn't hit this harder, earlier.  My doctors 
were worried about the treatments causing me problems.  If anyone tells 
you they're worried about causing you problems, George, you might want to 
tell them this: "Doctor, cancer is a problem, a big problem.  Death, on 
the scale of life's problems is right up there in terms of a fairly large 

One last piece of free advice:  You are an individual, who is on a unique 
journey like no one else's.  I know this because I've talked with several 
people from around the world with Schwannoma and they're stories are all 
different, dramatically different.

I know I need to go into some more detail because this is the briefest of 
sketches and I don't want to mislead you.  However I'm off to Memorial 
Sloan Kettering, the finest sarcoma center in the world, this week and 
I've got to prepare for it.  I'll talk to you later and ask God to give  
you strength for the journey.

Good Luck,

Evan Burns