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I will make this very short
If I may offer some historic information
I went to medical school and never heard about a chordoma
And I ended my specialty training here in the United States
Initially in diagnostic radiology and later in Radiation Oncology.
And my first encounter was indeed
???????? this chordoma was indeed teaching ???????
???????????????
where I was aksed to prepare some summary about a tumor type of
my choice and I pulled out of teaching ???????
the case of the patient with the lumbar chordoma and prepared myself
and so I went through the training here and came to Boston and
that's what I do now exclusively
With proton radiation therapy our own experience at Mass General Hospital
initially at the Howard Cyclotron Laboratory
and now at the Proton Center which is based at the hospital
It's a total of about 1,000 patients with chordomas in any location
skull base, mobile spine, sacral
We have treated some 600 patients with chordomas of the clivus
I recently completed a review of the 100 children with chordomas
of the clivus we have treated over the past 25 years
About 500 adult patients with chordomas of the clivus
170 patients with chordomas of the cervical spine
and some 50 patients with chordomas of the sacrum
So given the rarity of this tumor, these are incredible numbers
and this has been successful only because we have had the closest
collaboratin with other specialties
Surgery, diagnostic radiology, pathology, you name it
This is not not a stand-alone operation that one specialty can be successful
This has been only successful because we have recruited the best of
every specialty to bring to the table when it comes to the
treatment of chordoma and as a matter of fact
it starts even with things like diagnostic radiology
how can you identify without the first intervention
how can you identify chordoma?
Years ago when I came to Boston
we had treated quite a number of not only chordomas but chondrasarcomas
and they mimic each other and
when I looked at the total experiences with chondrasarcoma
it turns out that 40% of the patients we eventually treated for
low grade chondrasarcoma came from the outside
referring physician/institution with the wrong diagnosis
they were referred to as chordoma and they turned out on closer analysis
to be chondrasarcomas
So in another ???????????????????? the chordoma presents in the mobile
spine, it can right up front mimic other types of tumor
For example in the cervical spine a patient presents with neck pain
nerve deficits, slowly progressive, and the tumor's been detected
Very frequently the imaging studies the imaging studies, the MRI scans
are done and it has been found this might be a benign tumor
arising from nerve
there management is vastly different from chordoma treatment
so it just shows this should be really a joint venture
from the get go
starting with the clinical diagnosis radiographic features
planning of the biopsy, planning---
careful planning of surgical approaches in combination with
radiation therapy
the end result is really as strong as the weakest link in this entire chain
if you do poor surgery, you cannot catch up
with sophisticated radiation therapy and you can have the most brilliant
and most refined surgery and if it is not followed by adequate
post-operative management -- radiation therapy
the overall long-term result is also not as satisfactory
one message--- we have only learned from patients
you cannot go from a medical textbook for guidance
you cannot even call any physicians around the world
basically for guidance other than your colleagues and
other centers of medical excellence
we have all learned from our patients and their outcomes
they have taught us a lesson
and I'm still learning---even today
and, again, the other lesson I have learned is to seek the
broadest possible communication and collabration with other specialties
with other colleagues in the various specialties
with other centers of excellence---broadest communication
sharing information, sending them CDs, getting on the phone
they look on their computer at the same information
you do it in your own office and you come to a joint review
and you come to a come to a conclusion which is then solid
that is in the patient's best interest
so let me just show you here, Dr. Sen and Dr. Gardner mentioned the
goal when it comes to surgical managment of tumors of the crainal base
and it is the same is true for tumors in any locations
along the mobile spine and sacrum
the goal has to be gross total resection
and whenever that is feasible with acceptable morbidity
and so if that is not entirely achievable
then at least maximum debulking should be attempted
so that the patient can become a candidate for
post-operative radiation therapy
so that the target ????????????????????????? is optimal
and here youll see the typical role
when it comes to post-operative proton radiation therapy
proton radiation therapy is just one tool in the armamentarium of
radiation therapy, only if you will, you know it allows the spot where
the tumor to a much higher dose especially if it lives in the vicinity of
sensitive structures and in the brain this is true
you have multiple sensitive structures surrounding the tumor site
often above is the optic pathway
the brain, left and right sandwiching the tumor
in the back of the brain stem -- sensitive nerves, vascular structure
you want to do the least harm and for that you use indeed
a very focused type of radiation therapy and that is the unique feature
of proton radiation therapy
So one can drive the doses with proton radiation therapy
about 50% higher than one could achieve
with conventional radiation therapy and
that has, in many instances, contributed to improved long-term tumor control
so this have been clearly a joint venture
I take the time when patients come and go back through their history
and how the tumor started
what they noticed when it was detected
what the first step was
and one lesson I have clearly learned when such a tumor is detected
and it is not a medical emergency
take your time, get a second, get a third opinion
from centers of excellence
Don't let anybody rush you into decisions which are entering one-way streets,
where you cannot back off and you cannot go back and improve
since the first step --- it starts even with the consideration of the biopsy
how should it be done best, by whom, from what directions
with the least degree of harm
the same principle is true for subsequent management
surgery and radiation therapy
thank you.