3. Odontogenic Tumors Flashcards
What is the most common Epithelial Odontogenic Tumor?
Ameloblastoma
(11% of odontogenic tumors)
What is the other name for Ameloblastoma?
Adamantinoma
What population is affected by Ameloblastomas?
No one is immune
- Most cases arise on or after 3rd - 4th decade
- But can occur in younger individuals
- Equal M:F
Where are Ameloblastomas located from most to least frequently?
- Posterior Mandible (77%)
- Anterior Mandible (10%)
- Posterior Maxilla (7%)
- Anterior Maxilla (6%)
What is the clinical presentation of all Epithelial Odontogenic Tumors?
- Slow growing
- Painless
- Bony hard swelling
What is the CLASSIC radiographic appearance of an Ameloblastoma? (5)
- Multilocular RL, without RO
- Fusiform/tapered mass due to expansion
-
“Eggshell” thin cortex
- As it grows it eats away the cortex
- If advanced it can perforate the cortex
- Can displace teeth + resorb roots (80%)
- Associated with an impacted tooth, usually 3rd molar (77% mandibular)
What is the histology of an Ameloblastoma? (3 zones)
-
Peripheral Zone - Hyperchromatic columnar ameloblasts “piano keys” with:
-
Subnuclear vacuolization
- Bubbly appearing cytoplasm - defines an ameloblast
- Reverse polarity of nuclei (toward center not CT)
-
Subnuclear vacuolization
- Central Zone - of Stellate Reticulum
-
Outer Zone - of supporting stroma
- Mature, collagen producing CT, not neoplastic
What does an Ameloblastoma’s histology reproduce?
Early Enamel Organ
Not going to see enamel forming because they are neoplastic ameloblasts and enamel has to have dentin first to form.
Where are the changes occuring in the different histologic variations of Ameloblastoma?
Stellate Reticulum
Doesn’t change the way it behaves or the prognosis
Which Histologic Variation of Ameloblastoma is the conventional type?
Follicular Ameloblastoma
Which Histologic Variation of Ameloblastoma is misken for SCCA, showing invading islands of squamous metaplasia within the SR?
Acanthomatous Ameloblastoma
In which Histologic Variation of Ameloblastoma does the SR develop cells with a granular appearance?
Granular Cell Ameloblastoma
Not the same cell as a granular cell tumor or congenital epulis
Which Histologic Variation of Ameloblastoma has no SR, it just looks like a proliferation of dental lamina that hasn’t matured to the appearance of an enamel organ?
Plexiform Ameloblastoma
Which Histologic Variation of Ameloblastoma is mistaken for Metastatic Cancers and Salivary Gland Tumors?
Plexiform Ameloblastoma
What histologically occurs in Desmoplastic Ameloblastoma?
- Cells of a tumor cause the CT to become fibrous, collagenous, and dense
- The dense CT squeezes the epithelial component
Where does a Desmoplastic Ameloblastoma occur?
Anterior Maxilla
Least common area for an Ameloblastoma
How is the radiographic appearance of a Desmoplastic Ameloblastoma different from the Follicular type?
NOT Purely RL
- Has some “ground glass” opacification
- Only variant with RO
- Due to dense collagen production
What is the histogenesis of Ameloblastomas? (3)
- Originate from residual pluripotential dental lamina rests, developing enamel organ, or the lining of Dentingerous Cyst
- The cells are primitive with unrestrained growth
- They are not capable of inducing dentin or production of enamel
What other lesions do Ameloblastoma’s have a relationship with? (5)
- Basal Cell Carinoma
- Tibial Adamanthinoma
- Craniopharyngioma
- Peripheral Ameloblastoma
- Unicystic Ameloblastoma
What does a Tibial Adamanthinoma histologicall resemble?
Plexiform Ameloblastoma
But it is a malignant tumor derived from sweat glands
Why does a Craniopharyngioma precisely resemble the histo of an Ameloblastoma?
Ameloblastoma of the Pituitary Area
- It is derived from the craniopharyngeal duct from the posterior stomodeum (oral cavity) that goes upward to form the pituitary
- The surround tissue will be brain tissue, not collagen
What characterisizes the appearance of a Peripheral Ameloblastoma?
- Ameloblastoma arising in the gingiva WITHOUT primary bone involvement
- It may erode and secondarily infect bone, causing a slight RL.
- Produces a GUM BUMP (4 common in DD)
Where does a peripheral ameloblastoma arise from?
Soft tissue lesion, arising from surface epithelium
What is included in the DD for a Peripheral Ameloblastoma?
Gum Bumps
- Pyogenic Granuloma
- Peripheral Giant Cell Granuloma
- Fibroma/Fibrous Hyperplasia
- Peripheral Ossifying Fibroma
What is the treatment and prognosis for a Peripheral Ameloblastoma?
- Doesn’t act like a typical AB, it isn’t as nasty, and it isn’t very aggressive
-
Recurrence in ~17%
- Can be cured by a wider re-excision
- Recurrences must be removed
What is the histology of the Peripheral AB?
Can look like any normal subtype of AB
What is the Histogenesis of a Unicystic Ameloblastoma?
- Arises as a cyst whose lining shows:
- SR over a basal layer of hyperchromatic ameloblasts
- Not infiltrative like AB
What is the population affected by Unicystic AB?
Peaks in teenagers
Typical AB is 3rd-4th decade
Where are Unicystic ABs formed?
90% form around the crown of an unerupted mandibular molar
What is the radiographic appearance of a Unicystic AB?
-
Unilocular, well-demarcated RL
- Not multilocular like AB
- Symmetrical like a “big egg”
- Hyper-ostotic border around periphery
- Can get enormous, but is slow to invade
- Can take up entire ramus and thin out the mandible
What is the prognosis of a Unicystic AB?
30% recurrence with curettage
What are the characteristics of Ameloblastic Carcinoma? (3)
- Histo shows a growth pattern like AB but, the cells are:
- Pleomorphic
- High mitotic activity
- Fast growth
- Metastasize
- RL lesions
What are the characteristics of Malignant Ameloblastoma? (2)
- Perfectly benign histology
- But metastasizes to lungs
- This is the only way to dx it
What is the prognosis for Ameloblastoma?
- Aggressive and recurs in 55% of attempted cures, no matter what tx method was used
- > 5 yrs follow-up needed, because they are slow growing
-
Maxillary = more aggressive
- Can be mutilating and impossible to tx if it spreads from the ethmoid’s –> sinus –> brain
What is the rule for treating an Ameloblastoma of the Mandible?
-
Marginal Resection
- Resect 1 cm of radiographically normal cancellous bone and SPARE CORTEX if not involved
- Leave the integrity of the bone
-
Segemental Block Resection
- Perforated cortex mandates this
- Lose continuity of mandible
- Some may prefer to currete mandibular tumors, yeilding a smaller marginal resection
What is the rule for treating an Ameloblastoma of the Posterior Maxilla?
- NEVER Curettage because recurrence is inevittable and unmanageable
- Must resect with wide margin up to and including hemi-maxillectomy
Why is the Adenomatoid Odontogenic Tumor (Adenoameloblastoma) called the 2/3rds tumor?
- 2/3 Female
-
2/3 Maxilla (mostly anterior)
- Never past premolar region
-
2/3 10-20 y.o.
- Not seen past the age of 25
What teeth are 75% of AOTs associated with?
Impacted tooth, usually canine
What is the histogenesis of the adenomatoid odontogenic tumor? (3)
- Derived from enamel organ
- Cells programmed for necrobiosis
- Cells grow to a certain point then involute, causing self-destruction of the tumor
- Burnt-out cases
- Tumor amyloid (stains pink)
- Dystrophic calcification
What is the CLASSIC radiographic appearance of Adenomatoid Odontogenic Tumor? (4)
- Pericoronal RL around an impacted maxillary canine
- Expansile and well-demarcated
-
Originate/attaches to the tooth higher along the root surface
- Not crown-root junction like in a dentingerous cyst
- Flecks of RO - represent dystrophic calcifications from older cells dying
What is the histology of an AOT? (3)
- Sheets of cuboidal and low columnar cells, arranged in solid and hallow cell balls separated by spindly cells
- No CT or Stroma, just epithelium
- Ultimately, epithelium degenerates into amyloid and dystrophic calcification
What is the treatment and prognosis for AOT? (3)
- Simple currettage is curative
-
No known recurrences, even if incompletely removed
- Its already trying to kill itself = necrobiosis
- Most respond spontaneously, leaving only small flecks of calcification
What are the similarities between Calcifying Epithelial Odontogenic Tumor (Pindborg) and Ameloblastoma? (5)
- Infiltrative epithelial tumor, resembling Stratum Intermedium of the enamel organ
- Slow growing, painless bulge
- Can be destructive
- 3rd - 5th decade
- Equal M:F
What is the main difference between the Pindborg Tumor and AB?
Pindborg is NEVER Malignant
Where are Calcifying Epithelial Odontogenic Tumors (Pindborg Tumor) located? (3)
- Mandibular premolar region
- 30% occur in maxilla
- Often associtated with impacted 3rd molar
- Like AB
What is the radiographic appearance of the Calcifying Epithelial Odontogenic Tumor?
-
Multilocular or unilocular RL, occasionally with large RO
- Completely differs from AB
What are the histologic clues used to diagnose the Pindborg Tumor? (4)
- Mature cytoplasm
- Absence of mitoses
- Leisegang Rings (dystrophic calcifications)
- In the jaw
Infiltrative sheets, islands, and nests of pink squamous cells, showing hyperchromatis, pleomorphism, and coarse chromatin
What is the best stain to use on Pindborg Tumors, and why?
Thioflavin T Stain
Green = amyloid
- Epithelium undergoes necrobiosis and is replaced by dystrophic calcifications and amyloid
- Just like AOT
What is the treatment and prognosis for the Pindborg Tumor?
-
Block resection
- More conservative than AB
- 15% recurrence instead of 55% with AB
In what population are Calcifing Odontogenic Cysts (Gorlin Cysts) common in?
Any age or gender
Where are Calcifing Odontogenic Cysts (Gorlin Cysts) common?
Any location, preferrence for anterior areas
15% are peripheral gingival masses
What is the radiographic presentation of the Calcifing Odontogenic Cysts (Gorlin Cysts)? (5)
- CLASSICALLY a unilocular well-defined RL
-
40% have RO dystrophic calcifications
- Like AOT, Pindborg
-
1/3 associated with an unerupted tooth, usually a canine
- Like AOT
- Often causes root resorption if it gets close to another tooth
- Like AB
- 20% are associated with Odontomas
What is the histology of a Calcifing Odontogenic Cyst (Gorlin Cyst)
- Cyst lined by epithelium aka true cyst
- Ameloblastic lining with ghost (keratin) cells (have no nuclei) in stellate reticulum
Ameloblastoma with ghost cells
What other tumor most resembles the Calcifying Odontogenic Cyst (Gorlin Cyst)?
Craniopharyngioma
What is the treatment and prognosis of the Gorlin Cyst? (3)
- Enucleation (shell it out) usually curative
- Very few recurrences
- If malignant often recur and can mets
- Occasional cases are malignant but it is rare
Odontogenic Myxoma is an invasive, infiltrative tumor, derived from , resembling .
Ectomesenchyme
Dental Papilla
What is the peak age for Odontogenic Myxoma?
3rd decade
What is the location of Odontogenic Myxomas?
- Preference for mandible and posterior regions
- But may occur anywhere in tooth-bearing location
What acts like Ameloblastoma radiographically and clinically?
Odontogenic Myxoma
-
X-ray
- Multilocular or soap-bubble RL
- Fusiform expansion
- Thins cortex and may perforate
-
Clinically
- Slow, painless expansion
- Can move and resorb teeth
- Gorlin cyst also resorbs teeth
What is the gross appearance of an Odontogenic Myxoma?
- Solid, soft, gelatinous, mucoid, glary tumor,
- “Oyster”
Differs from AB, which is part cystic and part solid
What is the histological appearance of an Odontogenic Myxoma?
- Stellate fibroblast cells,“whispy”
- Widely separated by ground substance that stains for hyaluronic acid
- Alcian Blue Stain = baby blue
- Resembles histo of dental pulp, it looks like there is nothing there.
- AB ~ early enamel organ, with lots of cells
What is the treatment for an Odontogenic Myxoma?
-
Marginal or Segmental Resection
- Like the tx for AB, but not as aggressive
What is the prognosis for an Odontogenic Myxoma?
-
Goopy, gelatinous material tends to spill into the surgical bed, inviting 25% recurrences
- < 1/2 of AB
- No Malignancies
- Long-term follow-up needed > 5 yrs, due to slow growth
What does Cementoblastoma resemble histologically?
Osteoblastoma
Radiating trabeculae of parallel cementum lined by layers of plump cementoblasts
What is the peak age to find a Cementoblastoma?
2nd - 3rd decade
Male = Female
Where are Cementoblastomas located?
- Mandible, always attached to the root, of mostly 1st molars
- Occasionally in other molars or premolars
- NEVER in anterior teeth
What are the clinical symptoms of a Cementoblastoma?
-
PAIN to percussion and swelling
- One of the only ones that cause pain
- Due to being attached to the root
- Can cause expansion due to being in the area of the alveolar bone
What is the radiographic definitive diagnosis for a Cementoblastoma? (3)
- Well-demarcated, ovoid, spherical, central RO with a thin peripheral RL rim
- Surrounds and incorporates the root so that part of the root tends to disappear into the lesion
- NOT root resorption
-
Radiating “sunburst” appearance, may or may not be present
- Complex Odontomas, but they are 1st - 2nd decade
What is the prognosis for a Cementoblastoma?
- Recurrence unexpected if the tumor mass and tooth are cleanly removed
- The tooth will probs come out when the tumor mass is removed
- If the tooth remains there is a 20% recurrence, because they are true neoplasms
What is in the differential diagnosis for a RO around the roots of teeth? (3)
- Cementoblastoma
-
Condensing Osteitis
- __Also favors mand 1st molar
- Differs from CB:
- Irregular outline
- Pure homogenous RO, no RL rim or sunburst
- Root outline visable
- Tx = leave alone
-
Osseous Dysplasia
- Also a mixed RL/RO, and periapical
- Differs from CB:
- Lower anteriors, where CB never occurs
- Root outline visable
- 80% in black females > 30 yrs
List the mixed odontogenic tumors, in order of differentiation (least –> most) (4)
- Ameloblastic Fibroma
- Ameloblastic Fibro-Odontoma
- Complex Odontoma
- Compound Odontoma
What are the 2 germ layers of the mixed odontogenic tumors?
- Ectoderm
- Ectomesenchyme
What is the peak age of Ameloblastic Fibroma?
1st - 2nd decade
List the Pediatric Odontogenic Tumors. (5)
- Adenomatoid Odontogenic Tumor (AOT)
- Unicystic Ameloblastoma
- Ameloblastic Fibroma
- Ameloblastic Fibro-odontoma
- Odontomas
- Complex
- Compound
What is the location for a Ameloblastic Fibroma?
- 70% posterior mandible associated with an 75% unerupted molar
- Most others in posterior maxilla
- Uncommon in the anterior jaws
What is the radiographic appearance of the Ameloblastic Fibroma?
DD for unilocular, pericoronal RL
-
Gorlin Cyst
- Unerupted canine
-
Adenomatoid Odontogenic Tumor
- Impacted maxillary canine
-
Pindborg Tumor
- __Mandibular premolars
-
Unicystic Ameloblastoma
- Teens, unerupted mandibular molars
What is the Histogenesis of the Ameloblastic Fibroma? (3)
- The epithelium is like that of Ameloblastoma
- ~ Enamel organ
- CT is the big difference:
- Epithelium doesn’t invade the CT, the 2 germ layers grow together as a unit
- More organized and mature than AB
- The mesenchyme is like that of Myxoma
- Grows with homogenous cellular stroma
- ~ Dental papilla, no collagen
- Grows with homogenous cellular stroma
- Usually encapsulated
What is the treatment for an Ameloblastic Fibroma?
-
Marginal Resection
- Even though it is encapsulted, shelling it out doesn’t work
- Enucleation or currettage is discourage because of recurrence tendency
- Don’t mess with this tumor, get it the fuck out!
What is the prognosis for an Ameloblastic Fibroma? (2)
-
Tendency to recur, and if they do:
- CT/mesenchymal portion becomes more cellular, faster growing, and more aggressive
- But not the epithelial portion
- Recurrences are occasionally associated with transformation to Ameloblastic Fibrosarcoma
- Malignant and often fatal
- They go after vital organs
Why is the Ameloblastic Fibro-Odontoma more organized and mature compared to the Ameloblastic Fibroma?
Capable of inducing dental hard tissue (enamel, dentin, cementum)
What is the age group affected by Ameloblastic Fibro-Odontoma?
5-20 yrs old (avg age = 10)
What is the radiographic appearance of the Ameloblastic Fibro-Odontoma?
- Unerupted posterior tooth
-
Pericoronal RL with RO representing dental hard tissue
- 1st one with RO due to actual tooth structure!
- May get large
What is the Histology of the Ameloblastic Fibro-Odontoma?
Looks like Ameloblastic Fibroma, that is producing histologically recogonizable but morphologically disoriented enamel, dentin and cementum.
What is the prognosis for Ameloblastic Fibro-Odontoma?
May turn into Ameloblastic Fibrosarcoma
Remove Aggressively
What is the most common Odontogenic Tumor?
Odontomas (60%)
Characterize Odontomas. (3)
- Mixed odontogenic tumor that most closely approaches normal odontogenesis
- Dental hard tissue is formed
- The enamel epithelium that formed it involutes
- The tumor is encapsulated in a “dental follicle” just like a tooth would be
- Most are asymptomatic
- Larger cases can cause swelling and pain
- All develop in tooth-bearing areas in the 1st - 2nd decade when teeth are forming
When/How are Odontomas diagnosed?
- During investigation of a:
- Primary tooth that fails to exfoliate
- Permanent tooth that fails to erupt
What is the histology of the Complex Odontoma?
Honeycombed conglomeration of enamel matrix, dentin, cementum, and pulp
- Normal Histogenesis = recognizable enamel, dentin, and cementum
-
Abnormal Morphogenesis = disorganized anatomy
- Enamel, dentin, and cementum are not in the right place
- Doesn’t look like a tooth
Where are Complex Odontomas located?
Posterior Jaws
What is the radiographic appearance of the Complex Odontoma? (3)
- Sunburst RO, surrounded by a thin RL
- Overlying/coronal to the tooth, it won’t even look like there is a tooth there.
- Can be confused with an Osteoma or any other highly calcified bone lesion
Where are Compound Odontomas located?
Anterior Maxilla
- Opposite of Complex Odontoma
- Like the AOT and Desmoplastic Ameloblastoma
What is the histology of the Compound Odontoma?
Normal Histogenesis and Morphogenesis
What is the radiographic appearance of the Compound Odontoma?
- Multiple, smaller teeth = RO portion
- RL sac surrounds