Chapter 30.

Odontogenic Cysts and Tumors

1. Odontogenic cysts and tumors

- uncommon lesions in oral and maxillofacial region
- odontogenic : derivation from tooth-related apparatus

2. Three major tissue in odontogenesis

1) enamel organ : oral ectoderm origin
2) dental follicle : ectomesenchymal origin
3) dental papilla : ectomesenchymal origin

3. Odontogenesis
1) dental lamina : apical proliferation from oral
mucosa of epithelium
2) bud stage
3) cap stage : enamel organ formation
4) bell stage
- degeneration of dental lamina
- remained dental lamina : development of
several odontogenic cysts and tumors

4. Four epithelium of the enamel organ

1) inner enamel epithelium : ameloblastic layer
tooth enamel
2) stratum intermedium
3) stellate reticulum
4) outer enamel epithelium

5. Reduced enamel epi.

- enamel organ epi. atrophies
- accumulation of fluid between reduced enamel epi.
and enamel
dentigerous or follicular cyst

6. Cell cycle
- G1(gap 1) S(DNA synthesis)
G2(gap 2)
M(mitosis)
- G1 S : key step of progression of cyst or tumor

7. Odontogenic cysts
- epi.-lined cyst in bone
: only in the jaws
- from odontogenic epi.
- two types (Table 30-1)
i) developmental
: origin unknown
ii) inflammatory
: result of inflammation

1) Dentigerous Cyst
(1) General Features
- prevalence in unerupted Mn. 3rd molars
- other : Mx. 3rd molars, canines, Mn. 2nd premolars
- rare in primary teeth
- common in 10~30 years old,
male > female, Whites > Blacks
- well-defined unilocular radiolucency
- epi. lining surrounding crown and root
- unilocular processes
- central, lateral, circumferential variety
- D/D : enlarged dental follicle
- noninflamed dentigerous cyst : thin epi. lining
- inflamed dentigerous cyst : hyperplastic rete ridges

1) Dentigerous Cyst
(1) General Features
(2) Treatment and Prognosis
- treatment : enucleation, marsupialization(large cyst),
removal of tooth, curettage
- need for histopathologic examination
- prognosis : excellent, rare recurrence

2) Odontogenic Keratocyst
(1) General Features
- distinctive form of developmental odontogenic cyst
- derived from remnants of dental lamina
- sporadic cyst, nevoid basal cell carcinoma
associated cyst
- need for adequate diagnosis and treatment
i) aggressive
ii) high recurrence rate
iii) association with nevoid basal cell carcinoma

- prevalence in 10~40 years old, male > female,

posterior mandible and ascending ramus

2) Odontogenic Keratocyst
(1) General features (continued)
- multilocular or unilocular
- D/D : dentigerous cyst (unilocular radiolucency)
- histologic findings
i) stratified squamous epi. layer (6~8 cells)
ii) thin and orthokeratinized epi.
iii) no prominent palisaded basal layer
iv) corrugation
v) daughter cysts

2) Odontogenic Keratocyst
(1) General Features
(2) Treatment and Prognosis
- treatment : enucleation(in one piece) and
curettage
- prognosis : high recurrence rate (2.5~62,5%)
- suggested mechanism for recurrence
i) remnants of dental lamina within the jaws
ii) incomplete removal of cyst
iii) remaining rests of dental lamina and
satellite cysts

- histopathological findings : increased expression of

IL-1a, IL-6, PCNA and Ki-67

3) Nevoid Basal Cell Carcinoma Syndrome

(1) General Features

- Autosomal dominant inherited condition

- Cause: mutation in the PTCH tumor suppressor gene on ch
romosome 9q22.3-q031
- Frontal & temporoparietal bossing
- Hypertelorism
- Mandibular prognathism
- Bifid ribs & lamellar calcification of the falx cerebri
- Tendency to develop multiple basal cell carcinoma
- Pitting defects on the palms and soles
- 1st manifestation for diagnosis: discovery of multiple
eontogenic keratocysts

od

3) Nevoid Basal Cell Carcinoma Syndro

me(continued)
(2) Treatment and Prognosis
- Treatment: Marsupialization (desirable)
Resection(not recommended)
- Prognosis: recurrence is common, due
to the retained rests of the dental lamina.

4) Glandular Odontogenic Cyst

(1) General Features
- aggressive, recurrent
- pluripotentiality in spite of odontogenic origin
- most common in middleaged adults
- most common in the anterior Mn region, frequently cross
ing the midline
- either unilocular or multilocular radiographically
- D/D: mucoepidermoid carcinoma
i) glandular odontogenic cyst : typically thinner epithelial
lining
ii) mucoepidermoid carcinoma : more solid and microcyst
ic epithelial proliferations

4) Glandular Odontogenic Cyst (continued)

(2) Treatment and Progonosis
- treatment
i)
ii)

enucleation and curettage

resection, when considering the recurrence rate of t
his disease

5) Calcifying Odontogenic Cyst

(1) General features
-

histopathologically diverse and clinically variable

classified as a neoplasm by some authors
often associated with the odontoma
ghost cell keratinization & characteristic microscopic features
predominantly an intraosseous lesion
age range : from infant to elderly
COCs associated with odontomas tend to occur in younger pat
ients
- neoplastic variant of the COC tend to occur in elderly patients.
- radiophically unilocular and well-defined
- radiopaque structures within the lesion: irregular or toothlike

5) Calcifying Odontogenic Cyst (Continued)

(2) Treatment and Prognosis
- enucleation and curettage
- association with another odontogenic tumor the progn
osis tends to be the same as for the associated tumor

2. Odontogenic Tumors
- inductive interactions between odontogenic
epithelium and odontogenic ectomesenchyme
- subclassification by the tissue origin
(Table 30-3)
- geographically diverse frequency (Table 304)

1)Ameloblastoma
(1) General features
- the most common clinically significant and potentially lethal odonto
genic tumor
- origin: rests of dental lamina, epithelial lining of an odontogenic cys
t, or basal cells of oral mucosa
- 3 different variants: solid/multicystic(92%), unicystic(6%), and perip
heral(2%)
i) Solid or Multicystic Ameloblastoma
- patients over a wide age range, with no gender predilection
- a painless expansion of the jaw
- slow growth leading to facial disfigurement when untreated
- mutilocular radiolucency on radiograph
- resorption of adjacent tooth roots
- histologic patterns : follicular; plexiform; acanthomatous; granular cell; desmo
plastic; basal cell

i) Solid or Multicystic Ameloblastoma (continued)

- proliferative capacity
- recurrence high number of PCNA-positive cells and
previously unoperated ameloblastomas
- aggressive behavior overexpression of BCL2, BCLX and
expression of IL-1, IL-6
- Treatment and prognosis
highly aggressive and curable
numerous treatment recommendatoins: simple enucleation,
curettage, resection
Infiltrating feature
likely to be recurrent and persistent
resection with 1.0cm linear bony margins is recommended
any less aggressive treatment postoperative persistence

ii) Unicystic Ameloblastoma

- Histologic alterations associated with neoplastic
transformation of ameloblastomatous epithelium
Histologic changes
Hyperchromatism of basal cell nuclei of the
epithelium lining the cystic cavities.
Palisading and polarization of basal cell nuclei of
the epithelium lining the cystic cavities.
Cytoplasmic vacuolization, particularly of basal
cells of cystic linings.

ii) Unicystic Ameloblastoma (continued)

Pattern of epithelial proliferation described in dentigerous cysts of
the jaw that does not exhibit the histologic criteria for ameloblast
oma.
It may be treated more conservatively than the solid or multicystic
ameloblastoma in many cases.
The average age of patients with unicystic ameloblastomas has b
een reported as 22.1 years.
More than 90% are found in mandible(usually in the molar/ramu
s region).

ii) Unicystic Ameloblastoma (continued)

Radiograph: unilocular radiolucency, mimicking a dentigerous cyst
Three histopathologic variants
luminal unicystic ameloblastoma

confined to the luminal surface of the cyst

consists of a fibrous cyst wall with a lining that consists totally or partially of ame
loblastic epithelium.
intraluminal unicystic ameloblastoma

one or more nodules of ameloblastoma projecting from the cystic lining into the
lumen of the cyst
mural unicystic ameloblastoma

the fibrous wall of the cyst is infiltrated by typical follicular or plexi- form amelobl
astoma.

ii) Unicystic Ameloblastoma (continued)

Treatment and Prognosis
Open a cystic lesion and look for luminal proliferation of t
umor
Histopathologic examination (This is particularly impor- tant
when dealing with large cysts. )
Subclassify the variant of unicystic ameloblastoma
When confined to the lumen of the cyst with or without int
raluminal tumor extension, the enucleation has probably be
en curative treatment
When the cyst wall has been violated by the tumor as in a
mural variant of unicystic ameloblastoma, the most appropr
iate surgical management is quite controversial

 Luminal or intraluminal variant : enucleation and c

urettage
Recurrence rate : 10-20% following enucleation a
nd curettage
When recurrence arise:
recurrent unicystic ameloblastoma : more aggressive r
esection.
mural ameloblastoma : more aggressive than the lumi
nal and intraluminal variants owing to the presence of
tumor in the cyst wall and therefore closer to the surro
unding bone.
very large tumors with significant expansion : resectio
n

iii) Peripheral Ameloblastoma

Most rare variant of the ameloblastoma.
Probably arises from rests of dental lamina or the basal e
pithelial cells of the surface epithelium
Shows the same features of the intraosseous form of th
e tumor
Clinically, nonulcerated sessile or pedunculated gingival l
esions
Most <1.5cm
Wide age range (average: 52years)
Cup out bone in the jaws

 Treatment and Prognosis

Most: a wide local excision
Malignant transformation of a peripheral amelo
blas- toma is very rare.

2) Malignant Odontogenic Tumors

- very rare
- precursor cells for malignant transform
ation

The rest of Malassez

The rest of Serres in the gingiva
The lining of odontogenic cyst

- Classification (table 30-5)

2) Malignant Odontogenic Tumors (continued)

In general, all of these tumors exhibit typical mic
roscopic features of malignancy, with the except
ion of the malignant (metastasizing) ameloblast
oma and the clear cell odontogenic carcinoma.
Behaviorally, all of these tumors have the potent
ial for either regional nodal or distant metastase
s

2) Malignant Odontogenic Tumors (continued)

i) Malignant (Metastasizing) Ameloblastoma
Neoplasms that have the histologic features of benign amelob
lastoma as shown by the primary growth in the jaws and by a
ny metastatic growth.
The most common sites of metastatic disease are the lungs

ii) Ameloblastic Carcinoma

Malignant epithelial odontogenic tumors that exist in th
e background of benign ameloblastomas.
Although ameloblastic carcinomas have been reported t
o metastasize to the lungs and distant organs, many cas
es do not metastasize.
Rapid growth and pain were common symptoms.

iii) Primary Intraosseous Squamous Cell Carcinoma

Squamous cell carcinomas that are encountered in the jaws,
lack any continuity with the oral or antral mucosa, and occur
in the absence of a primary carcinoma located elsewhere
Arise from odontogenic epithelium
Typically in elderly patients and in the mandibular body regio
n.
The 5-year survival rate is 30 to 40%.
Also arise from the linings of odontogenic cysts.
Cystogenic carcinomas are seen in patients > 50 years of age
and typically occur in the mandible
Dentigerous cysts can undergo glandular metaplasia

iv) Clear Cell Odontogenic Carcinoma

Although putative odontogenic origin, histologic similari
ties to the developing tooth germ are lacking in many in
stances
Differnential diagnosis: metastasis from a distant site, es
pecially the kidney.
Chief entity to consider: clear cell variant of renal cell car
cinoma
Generally in elderly women, equally in Mx and Mn.

v) Malignant Epithelial Odontogenic Ghost Cell Tumor

Also known as dentinogenic ghost cell tumor
Solid variant of the calcifying odontogenic cyst
Both epithelial and ectomesenchymal odontogenic elem
ents are present
Only the epithelial component shows cytologic features
of malignancy.

3) Ameloblastic fibroma
The epithelial and mesenchymal tissues are both neoplas
tic.
Occur in young patients in the first two decades
70% 0f cases : post. Mn.
Radiogaraph: unilocular or multilocular lesion
Treatment and Prognosis
enucleation and curettage surgery
Resection: recurrent lesions

4) Ameloblastic Fibro-odontoma
Probably represents a hamartoma
Only a stage in the development of an odontoma and do
es not represent a separate entity.
Occurs more frequently in the posterior regions of the ja
ws
Commonly asymptomatic
Well circumscribed and appear as mixed radiopaque/radi
olucent masses.
Treatment and Prognosis
Enucleation and curettage surgery
Recurrence after this approach is very rare

5) Odontoma
The most frequently occurring odontogenic tumors
Hamartomas
Two forms
Compound
multiple small toothlike structures
predominantly seen in the anterior maxilla
Complex
irregular masses of dentin and enamel
no anatomic resemblance to a tooth
typically seen in the posterior maxilla or mandible
Treatment and Prognosis
simple enucleation and curettage

6) Odontogenic Myxoma
An uncommon benign neoplasm of the jaws
Derived from ectomesenchyme
Histologically resembles the dental papilla of the develop
ing tooth
Slow growing with a potential for aggressive behavior
High recurrence rate after subtherapeutic removal
Occur over a wide age range but seem to occur most co
mmonly in the third decade of life
The posterior mandible is most common location

6) Odontogenic Myxoma (continued)

Histologically: haphazardly arranged stellate, spindle-shaped,
and round cells in an abundant loose myxoid stroma.
Radiographically: unilocular or multilocular radiolucency that
may displace or cause root resorption of teeth in the area of t
he tumor
Pathogenetically: the proliferation and aggressive behavior(re
lated to overexpression of antiapoptotic cytokines BCL2 and
BCLX).
Treatment and Prognosis
resection with 1.0 cm bony linear margins
not encapsulated and tend to infiltrate the surrounding bone

7) Calcifying Epithelial Odontogenic Tumor

- Also known as the Pindborg tumor
- An uncommon lesion ( < 1% of all odontogenic tumors)

7) Calcifying Epithelial Odontogenic Tumor (continued)

Most patients between 30 and 50 years of age

Two-thirds of these neoplasms occur in the mandible
A painless slow-growing mass
Radiographically: mixed radiopaque/radiolucent lesion (frequently associated with a
n impacted tooth)
Histologically:
Discrete islands, strands, or sheets of polyhedral epithelial cells in a fibrous stroma
Large areas of amorphous eosinophilic hyalinized (amyloid-like) material
Calcifications: develop within the amyloid-like material and form concentric rings,
known as Liesegang rings
The precise nature of the amyloid-like material is unknown
Amyloid-like material: stained with Congo red or thioflavine T. (apple-green)

Treatment and Prognosis

Pindborg tumor is highly infiltrative and destructive and is capable of aggressive be
havior.
Recommended to be treated identically to the ameloblastoma and odontogenic m
yxoma, with 1.0 cm bony linear margins

8) Adenomatoid Odontogenic Tumor

Hamartoma
Uncommon odontogenic lesion (3 to 7% of all odontogenic tumors)
Once believed to be a variant of ameloblastoma and was previously
designated adenoameloblastoma
Limited to young patients (2/3 of all cases are diagnosed in the seco
nd decade)
Extremely uncommon (in patients > 30 years)
Predilection:

anterior region of the jaws

twice as often in the maxilla than in the mandible
Females are affected about twice as often as males

8) Adenomatoid Odontogenic Tumor (continued)

Small, rarely exceeding 3 cm in diameter
A well-circumscribed unilocular radiolucency that involves the crown of an erupted tooth,
frequently a canine.
Histologically:
Well-defined lesion (usually surrounded by a thick fibrous capsule)
Central portion of the tumor: essentially solid or varying degrees of cystic change with in
traluminal proliferation of tissue.
Spindle-shaped epithelial cells that form sheets, strands, or whorled masses of cells in a s
cant fibrous stroma
The epithelial cells may form rosette-like structures about a central space
Tubular or duct-like structures
A layer of columnar or cuboidal epithelial cells whose nuclei exhibit reverse polarization
Treatment and Prognosis
Enucleation and curettage surgery