Diagnosed with Ameloblastoma

Good luck! Let us know how it goes! I'm definitely interested in which medical oncologist you see and how the meeting goes (feel free to switch to PM.)

@tomschwerdt I would definitely be interested to read your argument about the misclassification of ameloblastoma being label a "non cancer". Is there a specific time frame I can search for this information within the forum? Also, I am very interested to get a referral to MDA. I'm hoping I can get approval for this as he does have medicaid for insurance.

And thank you and @anbar04 for the advice on the surgery!

Just an update on what's been going on-

We were initially told that he had an ameloblastoma in the middle of his lower jaw, which, from what I understand, is an unusual site for this type of tumor. After that, we were referred to UTMB, where the oral surgeon suggested that it was just a cyst. We felt a lot of relief when a CT scan also noted it as a "cystic lesion."

Yesterday, we went in for surgery to remove the cyst and the adult tooth that was believed to have caused it. However, after the surgery, the surgeon informed us that it does look more like a tumor after all. So, we're back to square one with the tumor diagnosis, and it has been sent off for a biopsy.

I’m trying to stay hopeful, but I’ve read that ameloblastomas can sometimes appear benign under the microscope, even when they aren't technically benign. Does anyone know if that's true? Also, my son is quite young to have developed this, and the location of the mass seems a bit unusual. Because of these anomalies I'm quite worried about the type of ameloblastoma we are dealing with.

That being said, we’re grateful that the mass has been removed, and an extra adult tooth was saved during the surgery. He’s less than 24 hours post-op and is doing really well. His pain is being managed, and he's recoveringtomschwerdt for the advice on the surgery!

Just an update on what's been going on-

We were initially told that he had an ameloblastoma in the middle of his lower jaw, which, from what I understand, is an unusual site for this type of tumor. After that, we were referred to UTMB, where the oral surgeon suggested that it was just a cyst. We felt a lot of relief when a CT scan also noted it as a "cystic lesion."

Yesterday, we went in for surgery to remove the cyst and the adult tooth that was believed to have caused it. However, after the surgery, the surgeon informed us that it does look more like a tumor after all. So, we're back to square one with the tumor diagnosis, and it has been sent off for a biopsy.

I’m trying to stay hopeful, but I’ve read that ameloblastomas can sometimes appear benign under the microscope, even when they aren't technically benign. Does anyone know if that's true? Also, my son is quite young to have developed this, and the location of the mass seems a bit unusual. Because of these anomalies I'm quite worried about the type of ameloblastoma we are dealing with.

That being said, we’re grateful that the mass has been removed, and an extra adult tooth was saved during the surgery. He’s less than 24 hours post-op and is doing really well. His pain is being managed, and he's recovering!

Hey, @lesleylbrown

Ameloblastomas are usually benign. My understanding is that they used to be classified as a cancer, and were reclassified because of they are usually benign. "Benign but locally aggressive" is how it was originally described to me. Uncontrolled growth of cells, destroying healthy tissue locally.

However, in a small percentage of cases ameloblastoma can metastasize (invade other parts of the body) - which is the hallmark of a tumor being a cancer instead of being benign.

My understanding is that in the times it does metastasize, it's usually an ameloblastoma which has been allowed to grow enormous without treatment.

However, to know this you have to dig deep into the topic.

Most doctors will never encounter an ameloblastoma in their career. Even many oral surgeons won't ever encounter one - and oral surgeons are the ones treating the vast majority of ameloblastomas which get treatment. Dentist discovers it, refers you to the oral surgeon, who performs the standard treatment of surgery. Virtually no oncologists ever see ameloblastomas, so why would they know much about them?

Expect that the standard pathologist will end up unable to decide if it's ameloblastoma, then refer the biopsy analysis to a specialty pathologist who will take a month or more and several reminders to finalize the report. At least, that's how it happened for me both times.

Now, for the good news: It sounds like the growth/tumor/whatever was relatively small. If it is an ameloblastoma - they usually grow pretty slowly. My original tumor was removed with relatively conservative surgery (enucleation and curettage) and it was ~15 years before it showed up on a dental Xray.

In those intervening 15 years, it was determined that ~80% of mandibular (jawbone) ameloblastomas have the BRAF V600E mutation, and the FDA approved a drug treatment for tumors with that specific mutation.
https://www.hopkinsmedicine.org/health/conditions-and-diseases/braf-mutation-and-cancer

@tomschwerdt Thank you so much for sharing the information and the study! I’m going to forward it to my son’s oral surgeon to see what he thinks and whether it might lead to a referral for further treatment options.

We had a post-op visit this past Friday, and they informed us that he has a desmoplastic ameloblastoma. They explained that it’s a more aggressive type, and they’ll be monitoring him every 4-6 weeks for any signs of recurrence. They said there's about a 50/50 chance it could come back. If it does, they may need to do a small resection, possibly taking bone from his leg to reconstruct his jaw.

I also want to express my deep appreciation to everyone who has shared their experiences and information during this difficult time. Your support has been incredibly valuable and comforting as we navigate this journey.

Thank you all again.

Wow! Desmoplastic ameloblastoma is a rare variant of an already very, very rare tumor! I was diagnosed with the unicystic variant both times.

If (when) it comes back, I highly recommend getting genetic testing of the tumor to see if there is targeted therapy available. I'm not telling you to do the targeted therapy, just that it's important to know whether it is a viable option. After that you, your son and your doctors need to find the best choices for your (his) particular situation.

My personal concerns about resection are largely about long-term or permanent loss of function both from a structural perspective and from a functionality perspective (permanent nerve damage).

@tomschwerdt again for all the insight you've provided. It definitely helps us feel a little more in control of of uncontrollable situation, as far as knowing what the options are and hearing everyone's experiences.

The doctor said he thinks there is a 50/50 chance of reoccurrence. I sent a message to see what his doctor thinks about genetic testing and other treatment options.

@lesleylbrown Happy to share my experience and hope that it continues to be helpful.

~80% of mandibular amelobastomas have the BRAF V600E mutation, and there is targeted drug therapy for tumors with that mutation.

That said, it's very early days for using that therapy for ameloblastomas with that mutation.

Since all the studies ended by ~6 months of treatment and I've been on the treatment for over a year - I may well hold the "world record" for longest BRAF V600E targeted treatment of an ameloblastoma.

As of the last CT scan, it still appears to be working at shrinking the tumor and regrowing bone - just slowly.