Let's move on to section two now and have a look at some epithelial odontogenic

tumours. So in this section we're going to be looking at tumours from the top part
of the classification. We're going to be looking at the Ameloblastoma and the
calcifying epithelial odontogenic tumour because the other two are relatively rare.
Ameloblastoma are not the commonest antigenic tumour, the commonest is the so
called a dontoh, but it's the commonest odontogenic tumour of any significance, and
we're going to look at it first in some detail, because it's the one that you need
to know most about, and it's the one that we can compare most of the other ones
too. You'll see that the middle Blastoise and the calcifying epithelial odontogenic
tumour are both in the top section of benign epithelial tumours, in addition to
them being benign. They are both neoplasms and you'll see also looking across the
diagram towards the third column, that they have good histo differentiation, that
is, the tissues in the middle blastoma will look like the tooth forming tissues and
the name Ameloblastoma gives you a clear clue that this is going to be a benign
neoplasm of amela blasts. So here are the two that we're going to look at. And
we're starting first with a conventional Ameloblastoma and we'll come on to its
variants later. So here's the thumbnail sketch for Ameloblastoma all facts that you
really need to be able to know and use at the end of the course. I just say that
the middle blastoma is a benign neoplasm that contains a transgenic epithelium that
looks like a meal of last. Let's look at the first line, which contains a bit of a
conundrum. It is benign but locally infiltrative. Now this is a bit of a puzzle
because you don't expect the nine neoplasms to be locally unfortunately, that's a
feature of malignant neoplasms, isn't it? So how can the Ameloblastoma is locally
infiltrative And yet it's still a benign neoplasm The answer lies in the behaviour
of adaptogenic epithelium during development. You'll remember how it burrows down
from the dental lamina and in its early developmental stages, it pushes in between
the medullary spaces of the bone and in an Ameloblastoma it does a similar thing.
So although this is classified as a benign neoplasm he does push out little islands
of epithelium into the surrounding bone. And that makes it difficult to treat as
we'll come to later. The next thing is that it's slow growing it takes several
years for an ammeter blastoma to reach five or eight centimetres in diameter. And
it's an epithelial adaptogenic neoplasm so it only consists of odontogenic
epithelium, no Meezan chi, because it has no reason time there can't be any
epithelial induction and formation of dentin or enamel so therefore you can work
out straight away that the Ameloblastoma is going to be a radiolucent lesion
because it won't make enamel dentin. The second bullet point says that it's the
commonest of antigenic neoplasm. I just said that it's not the commonest
odontogenic tumour remember that the classification contains both neoplasms
hematomas dysplasia and malignant neoplasms. So when I say that the A dontoh was
the commonest antigenic tumour that was quite correct, but the dontoh is a
hematoma. And the commonest neoplasm is this one the needle blastoma they usually
arise in middle age and they commonly Africans and Afro Caribbeans and the
commonest site is the posterior mandible on the lower ramus somewhere around the
lower third molar region. They grow painlessly to expand the mandible, and when
they start to impinge on teeth, they can resolve their roots. There are two main
variants of Ameloblastoma those which have multiple cysts within them. And those
which only have a single cyst cavity. We'll come on to the reason why this
difference is important later on. But we're going to carry on talking primarily
about the commoner solid or multicystic type. Here's a typical presentation of an
Ameloblastoma you can see that the mandible is enlarged from at least the lower
right canine posterior Li. The lower right segment teeth are displaced lingually on
the sulcus is filled out by a rounded swelling that has pushed the periosteum of
the mandible. Luckily, if you could get a finger in lingually, you would feel that
there's also lingual expansion and this is large enough to cause the patient facial
asymmetry. Note that there was no ulceration in that previous tumour nor is there
in this one because these are benign, slowly growing expense while pushing
neoplasms. This one has not yet expanded to fill the sulcus out but you can make
out the blue swelling in the mandible because of the cystic cavities that underlie
the alveolar mucosa. Here's an Ameloblastoma that's been opened up to remove the
lesion surgically you can see that several teeth have had to be sacrificed but also
that the cavity in which the Ameloblastoma lay has a smooth cortical surface around
the inside. Consistent with its benign neoplasm nature.

Typical radiographic features of the Leela blastoma are illustrated in the example
you saw earlier. These include sight, posterior mandible size, very variable but
can be very large shape multilocular outline, smooth, well defined and complicated.
Radio density radiolucent with radiopaque sceptre, and the effects on adjacent
structures include expansion of the jaw adjacent teeth displaced resorbed and
loosened this pa jaws and lower 90 degree occlusal illustrate the expansive
multilocular nature of a very large Ameloblastoma causing considerable facial
asymmetry.

These periodicals show two smaller Ameloblastoma does it still illustrate several
of the typical radiographic features

previously covered in the course. Your main radiological differential diagnosis for
the renal blastoma would be the antigenic keratocyst which you will recall is also
typically a well defined quarter cated multilocular lesion occurring in the
posterior mandible. However, unlike the Ameloblastoma, the odontogenic keratocyst
grows by borrowing through the mandible with minimal ocho lingual expansion and
virtually no effect on the adjacent teeth.

This panoramic shows that not all Ameloblastoma is present with a typical features.
The sight of this example in the anterior mandible is unusual, but the lesion is
typical in that it is multilocular expansive and there is evidence of root
resorption.

Nowadays, patients referred to hospital with suspected Ameloblastoma and other

expensive lesions would be imaged using CBCT or medical CT thereby enabling three
dimensional visualisation before biopsy.

So it will be very apparent to you by now that these Ameloblastoma as these

neoplasms are cystic neoplasms and that a lot of the space in the lesion is
occupied by cysts lumen. In order to get the diagnosis it's necessary to biopsy the
solid part of the lesion so here's an Ameloblastoma that's been opened for biopsy
and you can see the surgeon trying to find a piece of solid tumour to remove just a
piece of the stretched cyst lining may not be sufficient for diagnosis. So if
you're back working in the surgery department of the DCT and a few years, remember
that anything that cystic will need a biopsy of the solid area. Let's just take a
step back before we look at this surgical resection specimen and remember what I
said about the edge of an Ameloblastoma although it's benign, the epithelium will
infiltrate the surrounding bone or short distance. In fact, not all Ameloblastoma
do this only about 15 or 20% of them do but their empathy Liam extends in between
the two actually have the medullary bone for about four or five millimetres beyond
the edge of the cavity that you can see clinically or radiologically. This means
that if you go in and try and scoop the lesion out here islands of epithelium may
remain in the bone and they will then see the recurrence. It will also be obvious
to you that if 80% of them also don't have any of this epithelial infiltration of
the medullary cavity. It would be quite safe to correct those. But the problem with
curating Ameloblastoma is that if you get it wrong, you can have multifocal
recurrences. And the patient can end up having a bigger operation than is
necessary. This is currently a very controversial area for treatments. And this
example has been treated by the gold standard treatment, which is a surgical
resection removing one centimetre of bone all the way around the margin. This means
that the few millimetres of possible extension will be removed and there'll be a
few millimetres clearance. This resection specimen goes from the mid body from the
pre molar region to the coronoid and colonised coronoid process and condyle at the
right hand side and you can see the centre of the Ameloblastoma has been cut open.
There's a large cystic cavity and essentially within that zone of solid tumour
within which you can see lots more small cysts for me. You can also see the white
line of the ID canal that runs just below the bottom of the lesion along here. It's
been displaced inferiorly pushed down out of the way too low a border and again a
feature of a benign slowly expanding neoplasm there's a series of variants of
Ameloblastoma that we don't really have time to talk about today but details will
be found in courses essentials and they have some slight differences from the
conventional type of solid multicystic Ameloblastoma. The only one that's really of
any great significance is this last one that desmoplastic Ameloblastoma this one
has a tendency to produce a more honeycomb radiological appearance and it can be
very difficult to surgically remove. But apart from that I'll leave those to you to
collect information from textbooks and other resources. How do we know that an
Ameloblastoma he's an odontogenic tumour, well, his histological appearance gives
it away because it contains a meal oblasts This is a histological section of an
Ameloblastoma and I know that you don't all love histology but you need to get the
principles and so we'll be looking at just a little bit of histology in this
section. The histology of adaptogenic she was is recognised to be very complex.
This is a high power view of the tumour, and this is fibrous tissue in which the
tumour is growing. And this is an island of the epithelial odontogenic neoplasm. It
consists of lots of these little islands and you can see that they have some
features that resemble a little cap stage tooth germ. They have some static
reticulum in the middle and a dark line of ameloblastic running around the outside.
And in places like here you can just about make out at this magnification that
there is reverse nuclear polarity characteristic of the Miller blasts. This example
is so called a cancer mellitus that is it has keratin in the centre. So this is
some keratinization that you sometimes get.

It's of no particular significance, but just notice how two of these islands of
epithelium have started to form small sis cavities on here. One here, and you'll
remember our lectures about cysts. Me saying that once you have a cavity in
epithelium, it's bound to enlarge there's nowhere for the dead cells to go. They
break down and fall into the centre and exert an osmotic pressure and the cysts
will gradually get larger and larger is a Higher Power View. Here's the stellate
reticulum in the centre filling the centre of the island here and around the edge
of the basement membrane against which the middle of last sit and here on the
outside is the fibrous tissue that the tumour is growing in. These are the
Ameloblastoma here, you can see that the nuclei Here lie at the reverse end reverse
polarity from the basement membrane, which is here. And there are lots of vacuoles
in the other end of the Nayla blast because they're secreted B cells and they're
preparing to secrete enamel matrix onto this basement membrane for because there's
no reason time in an Ameloblastoma. The ameloblastic never differentiate any more
than this. They never make any enamel matrix and so the lesion is always
radiolucent there's nothing to mineralize the previous pattern of Ameloblastoma was
called a follicular Ameloblastoma because the epithelium forms round follicles or
islands. This is called the plexiform pattern. And it's exactly the same except
that in this one the epithelium is organised in these long thin strands. And even
at this power you can make up a lovely piano key. Emile oblasts blasts, the
difference between a plexiform and a follicular pattern of Ameloblastoma is of no
significance at all. So you don't need to know it but it does often confuse
students and you'll see examples in the online course in odontogenic tumours, it
really doesn't matter. In fact, many tumours have mixed patterns anyway, so don't
worry too much about the follicular and plexiform patterns. The key point is that
it contains lots of cysts is a big cyst here and analysis here and analysis here
are that they are there are lots of Ameloblastoma here telling you that it's an
adaptogenic tumour if you do get muddled about the difference between plexiform and
follicular, I want to come back to this lecture from the online diagnostic course.
Just think of flicker and plexiform as being mirror images of one another. On the
left if the green is epithelium and the yellow is the line of ameloblastic around
the outside. In the follicular pattern, the epithelium forms these islands with the
Ameloblastoma and the upside whereas the plexiform pattern, it forms all these
interconnected strands with the needle blast still along the edges, but in this
case, the connective tissue is in the centre of the islands, whereas in these ones
it's epithelium in the centre of the islands. I suggested that you picked up the
other variants for middle blastoma from the textbook, you don't really need to do
anything more than recognise their names as being types of Ameloblastoma. But I
thought I'd just show you briefly the desmoplastic variant because it is rather
different. In this one, the epithelium forms these fine strands that are compressed
in dense fibrous tissue. So this is epithelium here. The dark blue staining strands
are trapped in all this dense fibrous tissue desmoplastic means densely fibrous, so
histologically looks very different and when you see it radiologically it looks
different to his desmoplastic Ameloblastoma and you can barely make out its extent.
There are places where you can see these small, fuzzy cystic cavities. But it's
very difficult to tell where it starts and where it finishes. And where is normal
medullary space on where he's Ameloblastoma all these little bits of Ameloblastoma.
So that's a variant that it's worth knowing about. So let's come back to this key
fact about Ameloblastoma has been benign, and yet the epithelium extends into the
surrounding bone in about 15% of cases. Really important thing for you to know and
understand. It's very contentious at the moment about what the significance of this
is. And I think it's important to understand how it works histologically because
otherwise you don't really understand the controversy. Here is a piece of mandible
infiltrated by Ameloblastoma this bright red stuff up here is the bone you can see
the little osteocytes in the bone and you can see the lamellar structure of the
bone in several areas with resting and reversal lines. here here's the meat of last
over this is an Ameloblastoma cyst. Here's another one you can see the line of
darkly stained meal of last around the outside another cyst here, lots of tiny
little cysts. Look how many of them there are many, many hundreds of them along a
few millimetres of the margin and you can see that they've extended in between the
bony cavity and they even get into tiny little medullary spaces like this one.
There's another little bit up there. And there's more up here. It's about two or
three millimetres across this histological field. So when we say that the
epithelium permeates the bone at the edges, there's really an awful lot of it and
it can go several millimetres. So what is the current controversy about treatment
of Ameloblastoma? Well, it's how much bone you should remove to be confident of
preventing recurrence. The gold standard treatment is to excise it with a
centimetre of bone so that you remove all that epithelium all the way around the
margin.

Unfortunately, that can be a very mutilating operation. And Ameloblastoma often

extends from near the condyle to the midline, and it's difficult to reconstruct
Well, surgeons often say that enucleation can be successful and in many cases it
can because it's only in about 15% of Ameloblastoma is that the epithelium does go
into the surrounding medullary space. The problem with giving everybody a
conservative enucleation first and seeing if it works is that the failure can be
catastrophic. A large lesion in the mandible for instance, could seed small
Ameloblastoma all the way through the residual bone had a bone graft and possibly
even into soft tissue, making it very difficult to control. But in the maxilla
things are even worse, the bone is thin and the sight of the lesion is near the
base of the skull. It's not unknown for Ameloblastoma to invade through the maxilla
to the base of skull and kill the patient. Even though they're benign. So a failed
conservative enucleation meaning just scooping it out and scraping the cavity clear
is a significant risk to the patient. So surgeons will in nucleate often the ones
that they feel a well localised radiologically so small one or two system Isla
blastoma is in the mandible are often nucleated and then followed up closely to
make sure that they don't recur. If they do recur, the patient should have the gold
standard treatment which is the resection with a centimetre of bone all the way
around the edge. So moving on from the multicystic, solid multicystic or
conventional Ameloblastoma. We have a further difficulty the Unisys to Camila
blastoma you need to understand this because it affects the decision on how these
should be treated. And it also is important for differential diagnosis. So what is
a unit cystic Camila blastoma it's exactly what the name says. It's an
Ameloblastoma with a single cyst cavity and it has to have a single sis cavity both
radiologically and histologically. To meet the diagnostic criteria, sometimes you
can get an Ameloblastoma that has one very large cyst and several very small ones.
That's not even a cystic that's still multicystic This has to have just one sis
cavity. Now the importance of this is that it's probably a different entity. It's a
younger age group. We often see these in the 20 and 30 year old group. Virtually
all of them are in the mandible, usually in the lower third molar area, and they
often grow around the crown of a lower rate in a dentist rich relationship that
makes diagnosis very difficult. radiologically it's going to look like attempting
to assist and also the histological diagnosis can be difficult because the
ameloblastic are not that obvious. He therefore is distinctly different from an
ordinary multicystic Ameloblastoma. And the key feature about this is the last
thing on the slide is that you can enucleated without recurrence. In this type of
Ameloblastoma the epithelium never goes into the surrounding bone. And so you can
nucleate it and she read the cavity quite safely there's no risk of recurrence.
This is summarised in this diagram taken from courses Essentials, where this topic
is explored in some detail. The problem is when it comes to diagnosis, here's what
I've just described to you on the left eight unit cystic Camila blastoma. It looks
like a single sis cavity it has a single layer of ameloblastic around the inside,
maybe with a little bit of thickening and that's a unit cystic Ameloblastoma has
these this one number two, this has a single layer of ameloblastic in most areas,
but they thickened up to make this plexiform structure in the middle doesn't really
matter. It's still only got one cyst cavity. However, if you are trying to make a
diagnosis on a radiograph you can only see the outlines of the bony cavity and if
you have a look at this Ameloblastoma This is the outline of the bony cavity
running round the outside. It looks on a radiograph exactly like the first two on
the left, but hidden in the soft tissue and invisible radiologically are all these
little cysts down at the bottom that make this a multicystic Ameloblastoma with the
islands in the wall, sometimes called a mural Ameloblastoma doesn't really matter
the name. The point is it looks like one says radiologically but actually it's not.
And here on the right is the conventional multicystic Ameloblastoma. And both of
these types here, these two types of conventional Medulloblastomas and their
epithelium can spill off around the edge into the surrounding bone causing
recurrence. Whereas that never occurs in the two types of trouble Unisys they can
be lavastone

from what you've just heard, you would expect the unilocular Ameloblastoma to
present as a unilocular well defined quarter cated Syslog radiolucency as
illustrated by these two examples, and the 3d reconstruction given these
appearances and the relationship of the uninterrupted displaced molar in both these
examples, what's your radiological differential diagnosis?

From the lesions previously covered in the course, your main radiological
differential diagnosis would be the dentigerous cyst, which you will recall is also
typically a well defined quarter cated unilocular lesion associated with an
uninterrupted tooth, which is often displaced.

Okay, back to some pathology here. He's a Unisys, the commercial of our stoma that
has been resected you can just make out the shape of the mandible and two molar
teeth here. You can see straight away that there is a single cavity. Here's the
edge of the cavity in the bone. It's not particularly expensive. Push the ID no
doubt and you will notice that the roots of the molar have been resolved where they
impinge on the cavity. There is no solid component only a single cyst. This one has
actually been overtreated because it was a huge cystic Ameloblastoma but it could
have been treated by nucleation rather than by this resection removing a few
millimetres of bone at the margin. However, the difficulty of differential
diagnosis means that this does sometimes have to happen. And the reason that it has
to happen is that in order to make a definitive diagnosis of Unisys, the Camino
blastoma you have to have a histological specimen because you must confirm that
there is only one cyst cavity up here and that the epithelium and the ameloblastic
are limited to a single layer around the wall like a cyst and that there are no
islands of Ameloblastoma down here to make it a solid multicystic type. The
previous one could easily have been misdiagnosed as a dentigerous cyst, but if you
have a look at this one higher power, you can just about make out that there's a
few elongated palisaded cells with some vacuolation here running along the basement
membrane. This is the basement membrane here. Here are the elongated myeloblasts
Just above them, and here's some stehlik reticulum and here's the SIS lumen above
it. So the presence of these ameloblastic needs this has to be a transgenic tumour
it can't be assessed the only cysts that would have elongated cells and it is the
adaptogenic Kratos cyst, and that should have a carotid Ising corrugated surface
and other features. So it's an Ameloblastoma but it's a different animal from a
solid multicystic type. Do we know anything about the causes of Ameloblastoma as
well? Yes, we think we do. We don't know much about adaptogenic tumours in general,
but there has been quite a lot of work on Ameloblastoma. And this is a nice paper
in the Journal of pathology including Prof. Morgan from our institution. And you
can see that the message here is that 60% of Ameloblastoma is carry a BRAF V 600 e
mutation. Now, I don't expect you to know the detail of these various mutations,
but you will find a nice Wikipedia page on the V 600, a mutation of the BRAF gene
and you'll see that it's quite a common mutation in neoplasms. It's a so called
driver mutation that is it is thought to be the key causative primary mutation
causing the neoplasm. And the Wikipedia page tells you a lot about all these
different cancers and you'll notice that Ameloblastoma is on the end of the list.
So I'll leave you to just follow up with a little bit of reading on that there's
something in courses essentials, and there's more come out recently, the importance
of knowing about it is that there are anti BRAF therapy, which exists primarily to
treat unpleasant, much more aggressive malignant neoplasms but they have been tried
in Ameloblastoma. The problem is that their adverse effects are pretty severe. And
so you can only justify giving an anti BRAF treatment to a patient who is has very
extensive disease or is terminal. Obviously Ameloblastoma is a benign neoplasm and
rarely kills patients. But you do occasionally get a patient like the one in this
paper who had what's described as widely disseminated Ameloblastoma because it's
spread from their jaw to their lung even though it was benign. And in this patient
you can

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