I am an Orthopaedic Spine Surgeon...A carpenter of the body. I've missed work a total of 3 days in the last fifteen years of practice due to illness. In 6 years of residency and fellowship I missed only 4 days after having ACL reconstruction. I never get sick!

My stellar attendance record is about to change.


Recently, I have been diagnosed with Ameloblastoma...an uncommon, benign yet aggressive tumor of my jaw.

I am not a wordsmith, but I have been inspired by the few others out there who have blogged about their experiences with ameloblastoma. I look at it from a clinical perspective as a surgeon (While the diagnosis is foreign to me, the surgical management and treatments are very familiar to me.) and scientist, I am also the patient. A rare perspective that I thought by sharing, I could enlighten others with both clinical information, and personal experience.


Wednesday, December 19, 2012

Talking to the Man with the Plan

My appointment with my surgeon came up quickly. I was fortunate that Dr. W worked with Dr. M at Vanderbilt. My wife and I met with him and his chief resident late one Monday afternoon. A new panorex, a review of my medical history, and a quick exam led us to the meat of the discussion. My research really paid off. I was very prepared for our discussion.

Ameloblastoma is rare but aggressive. The most "conservative" treatment is actually the most invasive...surgery. In order to maximize the likelihood of eradication for good, we need to take out the tumor itself, as well as a good margin of normal bone to make sure we got it all. One centimeter is considered the standard for a good margin. That means that they needed to have 1cm of normal bone in front of, behind, and below the tumor (above it includes the teeth) to say goodbye to my little friend. I am fortunate that my tumor is small and is in the front part of my jaw. Most ameloblastomas are found near the angle of the jaw and can get quite large, necessitating taking out a large part of the jaw including the joint where it attaches to the skull. This makes recovery much longer, reconstruction more challenging, and risks go up.

Being in the front, however does put the mental nerve at risk. This is the nerve that gives sensation to my lip, and chin on the right side. The goal would be to take away bone whilst sparing the nerve if at all possible. Fortunately, the position of the tumor is such that the nerve may be able to be addressed and left intact. I will lose 3-5 teeth in the process, but since the bottom of the jaw is still ok, I won't likely have to have a segmental osteotomy where the entire jaw bone from teeth to the bottom of the jaw is removed. This leaves the jaw unstable, which requires plating to pieces together and wiring my jaws shut to splint the pieces. It also complicates the reconstruction. 


Reconstruction of the jaw, or rebuilding the bone that is removed is done by taking bone from my hip/pelvis and putting in the jaw. Eventually the body grows together there and makes my jaw sturdy again so that teeth can be put in place. Again, I am lucky here in that the defect will not be huge, nor segmental, making reconstruction easier. Dr. M may be able to graft it at the same time that the tumor is removed. It all depend on whether he can safely close the gum and soft tissue over the graft. The worst thing in the world is if the wound dehisces and comes apart. That really increases the chance of infection, and possibly losing the graft. 

Jargon Alert:
dehiscence /de·his·cence/ (de-his´ins) a splitting open. 
wound dehiscence  separation of the layers of a surgical wound

Surgery will take 5-6 hours. I'll be able to go home the next day. Nope....no tracheostomy since the surgery can be done from inside out. Unlikely will I need my jaws wired shut since the strongest part of my jaw will remain intact. The ability to graft will be decided at the time of surgery. Saving the nerve will be a big priority. Six weeks or so off work. Liquid or no-chew diet for 3-6 months depending on whether the graft can be done at the same time or needs to be delayed. Implants once the graft is healed and incorporated. All in all, about a year of work to re-build my mouth and restore my bite to allow me to chew properly.

Whew!

I consider myself REALLY fortunate. The lesion is small and in the front. Most ameloblastoma stories on the internet involve much bigger tumors, much bigger operations, and much longer recoveries. I am also fortunate to be in such a great community that we don't have to travel to an unknown city to find the experts who can manage this. Nashville if very fortunate to have such a place as Vanderbilt University Medical Center. I'm healthy. I have good friends and a fantastic wife. My office and practice are amazing. My patients will be understanding (although their best interest worries me the most, frankly).

Let's do it....

Saturday, December 1, 2012

ameloblastoma (ām'ə-lō-blā-stō'mə)

I am now about 4 weeks into the process. A week between tender teeth time and my dental exam. A couple of days for the endodontist. Another day til I saw Dr. W. Antibiotics and CT took another week. Biopsy and then follow up about 10 days later. And.....

The verdict is in. Last Friday night Dr. W called to tell me the news of my pathology report. "The good news is it's not cancer." Good. "The not so good news is that it is an ameloblastoma. It's benign but will need surgery. You will lose some teeth, but it will be okay" Yeesh

http://www.ajronline.org/content/197/3/W412/F4.small.gif
This is the picture that scared me a little
In all my reading before the biopsy, the one picture that stood out was the surgical specimen from resection of an ameloblastoma.  Teeth, jaw etc. sitting on a surgical towel waiting to go to pathology. Jeez I hope that isn't what I have, I had thought.  My surgeon brain then went into overdrive regarding how one puts it back together.  You would need some sort of bone graft...Iliac crest? Fibula? Rib? A plate to fix the bones would be needed. How long til it was healed? Would it look "normal" once all put back together?

Nahh, it couldn't be that I had thought.


Medium Power View
Low Power View
Medium Power View

 AMELOBLASTOMA!!!


Well, indeed it is ameloblastoma. This is an uncommon (although the most common odontogenic tumor seen in the jaw. Approximately 50% of odontogenic tumors), benign tumor that is known to be aggressive and will come back in 50-90% of cases if not fully removed. It actually comes from the cells from where the teeth originate from and it is what creates the enamel. Essentially normal cells gone wild. There are several types of ameloblastoma including:

  • Conventional/Solid or Multicystic (This is what I have)
    • 3rd - 5th decades of life
    • Located in the mandible in 85%, mostly posterior
    • Lesion is peri-apical (nearby to the tips of the roots of the teeth)
      • Follicular - 28% of conventional type
        • Islands of epithelial rests in stroma
      • Plexiform - 32% of conventional type
        • Strands and cords
      • Acanthomatous  - 12% of conventional type
        • Squamous metaplasia
  • Unicystic
    • 1st - 3rd decades of life
    • 5-22% of ameloblastomas 
    • Male:Female = 1.6:1
    • Often associated with impacted teeth
    • Lined with epithelium
    • Possible to treat with curettage alone in some cases
  • Desmoplastic
    • 3rd - 7th decades of life
    • 4-13% of ameloblastomas
    • Often involves the maxilla and the anterior mandible
    • Compressed islands and thin cords
  • Maxillary
    • Most feared type due to proximity to skull, orbits and intracranial structures
    • Males=Females
    • Sinus extension in men is 10.6%, in women is 5.9%
    • Radical surgery is mandatory
Here are some references and reviews about ameloblastoma types.

Okay, so I have ameloblastoma. I would have to say I was surprised, somewhat shocked and a bit anxious. What about treatment? The literature I reviewed, the case reports, and even the blogs out there all pointed to the need for resection of the tumor with a border of normal bone of about 1 cm on all sides. How much bone is removed depends especially on the location of the tumor. Those tumors growing in the back part of the jaw tended to get larger and needed bigger resections. Remember that photo (above) that scared me? That is a resection of the back part of the jaw including the part where the jaw attaches to the skull. Good, mine is both fairly small and in the front. 

Jargon Alert:
resect /re·sect/ (-sekt´) to excise part or all of an organ or other structure

Well....It's time to talk to the guy who will take care of my little friend. I am fortunate to live where I do in that there are some very good maxillofacial surgeons right here. Dr. W recommended that I see Dr. M at Vanderbilt. Not only the chairman of the department, but also his mentor in training. Having done my residency at the same time as Dr. W, I know about Dr. M and feel very comfortable with that recommendation.

Lucky me, he had an available appointment in just a couple of days. Honey, I've got myself a doctor's appointment.

Off to Vanderbilt....