15: Odontogenic cysts and tumors Flashcards
Gorlin syndrome aka? Mutation and gene? Symptoms?
Nevoid basal cell carcinoma syndrome; PTCH1 on 9q22.3-q31; BCC of skin, OKCs, intracranial calcifications, bifid rib, kyphoscoliosis, epidermoid cysts, hypertelorism, palmar/plantar pits, large head
Gorlin cyst aka? Name if solid?
Calcifying odontogenic cyst; if solid = dentinogenic ghost cell tumor
Feature of calcifying odontogenic cyst presentation
20% associated with odontoma; also can be AOT, ameloblastoma, ameloblastic fibroma
Buccal bifurcation cyst location, local effect
Buccal of mandibular first molar; lingual displacement of roots seen on occlusal film
Paradental cyst location
Distal to partially erupted third molar
Presentation and histo of granular cell ameloblastoma
More aggressive, younger patients, contains lysosomes
Dentinogenic ghost cell tumor is a counterpart of what?
Solid counterpart of COC
What entity can lingually displace molar roots?
Buccal bifurcation cyst
What entity can be associated with an odontoma?
COC
What marker is increased in desmoplastic ameloblastoma?
TGFbeta
What ameloblastoma features an increase in TGFbeta?
Desmoplastic
Cytologic feature of clear cell odontogenic carcinoma
Glycogen-rich presecretory ameloblasts
Glycogen-rich presecretory ameloblasts are a feature of what entity?
Clear cell odontogenic carcinoma
Patterns of clear cell odontogenic carcinoma
monophasic, biphasic (clear/epithelial), clear cell ameloblastoma-like
Radiographic feature of CEOT
Driven-snow calcifications around the crown of impacted tooth
Driven-snow calcifications around the crown of impacted tooth are a radiographic feature of what entity?
CEOT (Calcifying epithelial odontogenic tumor)
How does squamous odontogenic tumor present radiographically?
triangular radiolucent defect lateral to a root (similar to vertical periodontal bone loss)
what entities can have squamous odontogenic tumor-like proliferations?
dentigerous and radicular cysts
Which odontogenic entities have mx=md prevalence?
SOT, COC, central odontogenic fibroma, odontoameloblastoma
Which odontogenic entities have mx prevalence?
desmoplastic ameloblastoma, odontomas, AOT
Prevalent location of SOT
mx=md
Prevalent location of COC
mx=md, anterior
Prevalent location of odontogenic fibroma
mx=md
Prevalent location of desmoplastic ameloblastoma
anterior mx
Prevalent location of odontoma
mx
What feature can be present in a central odontogenic fibroma
CGCG-like component
What odontogenic entity can have a CGCG-like component?
Central odontogenic fibroma
Origin and IHC profile of cells of granular cell odontogenic tumor
mesenchymal, S100-, lysosomal granules NKC13+
Composition and IHC of myxoma
ground substance hyaluronic acid and chondroitin sulfate. Vimentin diffuse, MSA focal (muscle-specific actin)
Which entity is composed of ground substance hyaluronic acid and chondroitin sulfate?
Myxoma
Pindborg tumor is aka
CEOT
CEOT is aka
Pindborg tumor
most common developmental odontogenic cyst
dentigerous cyst
how does a dentigerous cyst develop
fluid accumulates between reduced enamel epithelium and crown of tooth
what entity forms by accumulation of fluid between reduced enamel epithelium and crown of tooth
dentigerous cyst
possible relation of dentigerous cyst to tooth
central, lateral, circumferential
composition of wall of dentigerous cyst
glycosaminoglycan ground substance
what can arise from the lining of dentigerous cyst?
ameloblastoma, SCC, mucoep (latter if mucous cells are present)
what is the soft tissue counterpart of a dentigerous cyst?
eruption cyst
what happens when an eruption cyst is traumatized and bleeds?
eruption hematoma
what is the origin of an OKC?
dental lamina rests
prevalent location of OKC
posterior mandible
OKC age
10-40yo
OKC recurrence
30% (up to 10y)
what percentage of OKCs is associated with an impacted tooth?
25-40%
what malignant transformation can OKC undergo?
SCC, rare
how is OOC different from OKC?
clinically similar; epithelium is obvi orthokeratinized; recurrence is 2%
Features of BCCs in Gorlin syndrome?
less aggressive, younger age; fewer BCCs in blacks than whites
Histo and marker features of OKCs in Gorlin syndrome?
more satellite lesions, solid islands of epithelial proliferation and odontogenic rests; overexpression of p53 and cyclin D1 (bcl-1)
origin of gingival cyst of newborn?
dental lamina rests
clinical presentation of gingival cyst of newborn?
multiple whitish papules (keratin filled cysts) on mx alveolar process
which entity presents as multiple whitish papules (keratin filled cysts) on mx alveolar process?
gingival cyst of newborn
how many newborns get gingival cysts
50%
location of gingival cyst of newborn?
mx alveolar process
eruption cyst is a soft tissue counterpart of what
dentigerous cyst
what is the soft tissue counterpart of lateral periodontal cyst?
gingival cyst of adult
gingival cyst of adult is a soft tissue counterpart of what?
lateral periodontal cyst
origin of gingival cyst of adult
rests of serres (dental lamina)
what cells can be present in gingival cyst of adult?
clear glycogen-rich cells
where can clear glycogen-rich cells be present?
gingival cyst of adult, clear cell odontogenic carcinoma
origin of lateral periodontal cyst
dental lamina rests
prevalent location of lateral periodontal cyst
mandibular canine/lat incisor/premolar area
what is a botryoid cyst
polycystic lateral periodontal cyst
what is the name for a polycystic lateral periodontal cyst
botryoid cyst
how many of the COCs are peripheral?
30%
mean age of COC
30yo
how many COCs are associated with unerupted teeth and which? compare to OKC
30%, canine;; OKC – 25-40%
calcifications are presents in how many COCs?
30-50%
how many COCs are cystic or neoplastic?
98% cystic, 2% neoplastic
what looks like an ameloblastoma but has ghost cells and dentinoid?
dentinogenic ghost cell tumor
histo of dentinogenic ghost cell tumor
similar to ameloblastoma but with ghost cells and dentinoid
relation of dentinogenic ghost cell tumor to bone?
peripheral more common than intraosseous
what can arise from COC?
SCC, rare
aggressive variant of dentinogenic ghost cell tumor?
odontogenic ghost cell carcinoma
what odontogenic entity crosses md midline?
glandular odontogenic cyst
prevalent location of glandular odontogenic cyst
anterior md, crossing midline
what’s the recurrence of GOC
30%
histo look & IHC of glandular odontogenic cyst
hobnail or papillary epithelial lining, cilia, mucous cells, spherical nodules; mucicarmine +
which odontogenic entities can have thickenings of epithelium?
GOC, LPC
what predisposes a tooth to buccal bifurcation cyst
enamel extension in bifurcation –> pocket –> cyst; or inflammatory response during eruption
which entities may be related to inflammatory response during eruption
buccal bifurcation cyst and paradental cyst
how can carcinoma happen in odontogenic entities? which cysts can give rise to carcinoma?
de novo, from ameloblastoma, from odontogenic tumor or odontogenic cyst (DC, OKC, OOC, LPC)
what is the ddx for carcinoma arising in residual periapical cyst?
met vs de novo
origin of ameloblastoma
dental lamina, enamel organ, lining of cyst or basal cell layer of mucosa
which variant of ameloblastoma is preferentially in anterior mx
desmoplastic
radiographic look of desmoplastic ameloblastoma
mixed RL/RO
ddx of desmoplastic ameloblastoma
met
what is the effect of TGFb in desmoplastic ameloblastoma
inductive effect around islands, myxoid elsewhere
epidemiology of unicystic ameloblastoma
younger patients than multicystic
which ameloblastomas present in younger patients
unicystic and granular cell
histo variants of unicystic ameloblastoma
luminal, intraluminal, mural
criteria for unicystic ameloblastoma
vickers-grolin; 1. columnar basilar cells; 2. palisading of basilar cells; 3. polarization of basilar layer nuclei away from the basement membrane; 4. hyperchromatism of basal cell nuclei in epithalial lining; 5. subnuclear vacuolization of c/pl of basal cells
presentation of peripheral ameloblastoma
nodule in posterior gingiva
Ddx for nodule in posterior gingiva
peripheral ameloblastoma, peripheral odontogenic fibroma (has dentin or cementum-like material, lacks reverse polarity)
peripheral ameloblastoma vs peripheral odontogenic fibroma ddx
peripheral odontogenic fibroma has dentin or cementum-like material, lacks reverse polarity
malignant ameloblastoma vs ameloblastic carcinoma (histo, age, mets)
malignant ameloblastoma – normal histo in both primary and met; mean age 30yo; met to lung 10y after first diagnosis;;;; ameloblastic carcinoma – cytologic features of malignancy; older pts.
ddx of clear cell odontogenic carcinoma
mucoep (mucicarmine +), CEOT (amyloid +), met (renal, breat, melanoma)
origin of adenomatoid odontogenic tumor
enamel organ
prevalent location of AOT
anterior mx
possible concurrent entities with AOT
COC, CEOT, odontoma
numeric rule of AOT
2/3 with tooth (usually canine), 2/3 females, 2/3 10-19yo
age predilection of AOT
2/3 10-19
sex predilection of AOT
f
frequency of tooth association of AOT and which tooth
2/3 or like 75%, usually canine
histo look of AOT
rosette-like structures with or without eosinophilic material (amyloid +)
rosette-like structures with or without eosinophilic material (amyloid +)
AOT
radiographic look of AOT if calcifications are present
snowflakes
AOT associated with tooth name
follicular (75%), others are extra-follicular
CEOT origin
enamel organ or dental lamina
prevalent location of CEOT
posterior mandible
age of CEOT
30-50yo
recurrence of CEOT
15%
special stains and histo in CEOT
amyloid-like material + for congo red and thioflavine T;; Liesegang ring calcifications within the amyloid
Liesegang ring calcifications
within amyloid of CEOT
calcifications in amyloid
CEOT; = Liesegang
amyloid in odontogenic entities
AOT, CEOT, maybe central odontogenic fibroma
origin of squamous odontogenic tumor
dental lamina or rests of Malassez in periodontal ligament
ddx of triangular radiolucent defect lateral to tooth root
perio dz, SOT, LPC, OKC, amelo, LRC
most likely odontogenic tumor to have synchronous multiple occurences
SOT
histo features of SOT
calcs, eosinophilic structures, amyloid -!
ameloblastic fibroma origin
dental papilla
age of ameloblastic fibroma
young patients
site of ameloblastic fibroma
posterior mandible
histo look of ameloblastic fibroma
islands of epithelium; microcystic formation uncommon (unlike ameloblastoma)
age of ameloblastic fibro-odontoma
young patients
site of ameloblastic fibro-odontoma
posterior mandible
histo look of ameloblastic fibro-odontoma
enamel and dentin; vs ameloblastic fibro-dentinoma in which calcifying component is dentin only
which component is malignant in ameloblastic fibrosarcoma?
mesenchymal
ameloblastic fibrosarcoma arises from
55% de novo, 45% from ameloblastic fibroma
which malignancy can arise from ameloblastic fibroma?
ameloblastic fibrosarcoma
odontogenic tumor with dysplastic dentin, enamel, and malignant mesenchymal component
ameloblastic dentinosarcoma or fibro-odontosarcoma
odontogenic tumor with malignant epithelial and mesenchymal components
ameloblastic carcinosarcoma
entity with features of ameloblastoma and complex odontoma
odontoameloblastoma
site of odontoameloblastoma
md=mx
types of odontoma
complex (mass) and compound (small teeth by the pound)
complex odontoma
glob
compound odontoma
denticles
odontoma with denticles
compound
glob odontoma
complex
mean age of odontoma
14yo
site of odontoma
mx; compound anterior, complex posterior
what cells can be present in complex odontomas?
20% show ghost cells
what can arise from odontoma lining?
dentigerous cyst(s)
site of central odontogenic fibroma
45% in mx (anterior to first molar);; in mandible – posterior to first molar
which odontogenic entity is associated with a cleft-like tissue defect of the maxillary premolar region?
central odontogenic fibroma
clinical presentation of central odontogenic fibroma?
can be associated with a cleft-like tissue defect of the maxillary premolar region
histo types of central odontogenic fibroma
simple (myxofibroma) and WHO-type (odontogenic epithelium); can have CGCG-like component
ddx of central odontogenic fibroma if more collagenized
desmoplastic fibroma
site of peripheral odontogenic fibroma
posterior mandibular facial gingiva
site of granular cell odontogenic tumor
posterior mandible
lysosomal granules NKC13+ are characteristic of
granular cell odontogenic tumor
characteristic organelle of granular cell odontogenic tumor
lysosomal granules NKC13+
recurrence of myxoma
25%
what differentiates fibromyxoma or myxofibroma from regular myxoma
more collagen
ddx of myxoma vs chondromyxoid fibroma
chondromyxoid fibroma has cartilage
ddx of myxoma vs myxoid neurofibroma
myxoid neurofibroma is s100+
what’s the ddx for myxoma
chondromyxoid fibroma has cartilage; myxoid neurofibroma is s100+
malignant myxoma and its histo features
myxosarcoma: marked cellularity and cellular atypia
myxoma with marked cellularity and cellular atypia
myxosarcoma, malignant
odontogenic entity with BRAF
ameloblastoma
odontogenic entity with PTCH
OKC
odontogenic cells with Wnt/beta catenin
ghost cells
mucoep mutation
t(11;19) CRTC-MAML2 – Notch pathway
Notch pathway mutation in
mucoep
t(11;19) mutations
mucoep
CRTC-MAML2 mutation
mucoep
clear cell odontogenic carcinoma mutation
EWSR1-ATF1
EWSR1-ATF1 mutation
clear cell odontogenic carcinoma
mets of malignant ameloblastoma
lungs and cervical LNs
cysts associated with mandibular PM/M
LPC and gingival cyst
PTCH is which pathway
SHH
odontogenic tumors constitute ____% of specimens in oral path labs
<1%
prevalence of NBCCS
1:60,000
T or F, buccal bifurcation cyst is developmental
F, inflammatory
odontogenic cyst associated with proliferative periostitis
buccal bifurcation
T or F, OKCs enlarge 2/2 osmotic pressure
F, factors unknown
T or F, OKCs comprise 20% of all odontogenic cysts
F, 3-11%
Lateral periodontal cysts account for ____% of all epithelial lined jaw cysts
<2%
gender predilection of OOCs
2:1 M:F
oncoproteins in syndromic OKCs
p53 and cyclin D1 (bcl-1)
how many unicystic ameloblastomas are in posterior mandible
90%
least common ameloblastoma
basal cell variant
recommended surgical margins of ameloblastoma resection
1-1.5 cm beyond radiographic
most dangerous location for ameloblastoma
posterior maxilla
treatment recommendation for ameloblastic fibroma
initial conservative, aggressive for recurrence
average age and gender for myxoma
25-30yo, no gender predilection
average age of ameloblastic fibro-odontoma
10yo
Ameloblastic fibro-odontoma gender predilection
M:F 3:2
odontogenic fibromas gender predilection
F>M, 40 yo mean