15: Odontogenic cysts and tumors Flashcards
(189 cards)
1
Q
Gorlin syndrome aka? Mutation and gene? Symptoms?
A
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
2
Q
Gorlin cyst aka? Name if solid?
A
Calcifying odontogenic cyst; if solid = dentinogenic ghost cell tumor
3
Q
Feature of calcifying odontogenic cyst presentation
A
20% associated with odontoma; also can be AOT, ameloblastoma, ameloblastic fibroma
4
Q
Buccal bifurcation cyst location, local effect
A
Buccal of mandibular first molar; lingual displacement of roots seen on occlusal film
5
Q
Paradental cyst location
A
Distal to partially erupted third molar
6
Q
Presentation and histo of granular cell ameloblastoma
A
More aggressive, younger patients, contains lysosomes
7
Q
Dentinogenic ghost cell tumor is a counterpart of what?
A
Solid counterpart of COC
8
Q
What entity can lingually displace molar roots?
A
Buccal bifurcation cyst
9
Q
What entity can be associated with an odontoma?
A
COC
10
Q
What marker is increased in desmoplastic ameloblastoma?
A
TGFbeta
11
Q
What ameloblastoma features an increase in TGFbeta?
A
Desmoplastic
12
Q
Cytologic feature of clear cell odontogenic carcinoma
A
Glycogen-rich presecretory ameloblasts
13
Q
Glycogen-rich presecretory ameloblasts are a feature of what entity?
A
Clear cell odontogenic carcinoma
14
Q
Patterns of clear cell odontogenic carcinoma
A
monophasic, biphasic (clear/epithelial), clear cell ameloblastoma-like
15
Q
Radiographic feature of CEOT
A
Driven-snow calcifications around the crown of impacted tooth
16
Q
Driven-snow calcifications around the crown of impacted tooth are a radiographic feature of what entity?
A
CEOT (Calcifying epithelial odontogenic tumor)
17
Q
How does squamous odontogenic tumor present radiographically?
A
triangular radiolucent defect lateral to a root (similar to vertical periodontal bone loss)
18
Q
what entities can have squamous odontogenic tumor-like proliferations?
A
dentigerous and radicular cysts
19
Q
Which odontogenic entities have mx=md prevalence?
A
SOT, COC, central odontogenic fibroma, odontoameloblastoma
20
Q
Which odontogenic entities have mx prevalence?
A
desmoplastic ameloblastoma, odontomas, AOT
21
Q
Prevalent location of SOT
A
mx=md
22
Q
Prevalent location of COC
A
mx=md, anterior
23
Q
Prevalent location of odontogenic fibroma
A
mx=md
24
Q
Prevalent location of desmoplastic ameloblastoma
A
anterior mx
25
Prevalent location of odontoma
mx
26
What feature can be present in a central odontogenic fibroma
CGCG-like component
27
What odontogenic entity can have a CGCG-like component?
Central odontogenic fibroma
28
Origin and IHC profile of cells of granular cell odontogenic tumor
mesenchymal, S100-, lysosomal granules NKC13+
29
Composition and IHC of myxoma
ground substance hyaluronic acid and chondroitin sulfate. Vimentin diffuse, MSA focal (muscle-specific actin)
30
Which entity is composed of ground substance hyaluronic acid and chondroitin sulfate?
Myxoma
31
Pindborg tumor is aka
CEOT
32
CEOT is aka
Pindborg tumor
33
most common developmental odontogenic cyst
dentigerous cyst
34
how does a dentigerous cyst develop
fluid accumulates between reduced enamel epithelium and crown of tooth
35
what entity forms by accumulation of fluid between reduced enamel epithelium and crown of tooth
dentigerous cyst
36
possible relation of dentigerous cyst to tooth
central, lateral, circumferential
37
composition of wall of dentigerous cyst
glycosaminoglycan ground substance
38
what can arise from the lining of dentigerous cyst?
ameloblastoma, SCC, mucoep (latter if mucous cells are present)
39
what is the soft tissue counterpart of a dentigerous cyst?
eruption cyst
40
what happens when an eruption cyst is traumatized and bleeds?
eruption hematoma
41
what is the origin of an OKC?
dental lamina rests
42
prevalent location of OKC
posterior mandible
43
OKC age
10-40yo
44
OKC recurrence
30% (up to 10y)
45
what percentage of OKCs is associated with an impacted tooth?
25-40%
46
what malignant transformation can OKC undergo?
SCC, rare
47
how is OOC different from OKC?
clinically similar; epithelium is obvi orthokeratinized; recurrence is 2%
48
Features of BCCs in Gorlin syndrome?
less aggressive, younger age; fewer BCCs in blacks than whites
49
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)
50
origin of gingival cyst of newborn?
dental lamina rests
51
clinical presentation of gingival cyst of newborn?
multiple whitish papules (keratin filled cysts) on mx alveolar process
52
which entity presents as multiple whitish papules (keratin filled cysts) on mx alveolar process?
gingival cyst of newborn
53
how many newborns get gingival cysts
50%
54
location of gingival cyst of newborn?
mx alveolar process
55
eruption cyst is a soft tissue counterpart of what
dentigerous cyst
56
what is the soft tissue counterpart of lateral periodontal cyst?
gingival cyst of adult
57
gingival cyst of adult is a soft tissue counterpart of what?
lateral periodontal cyst
58
origin of gingival cyst of adult
rests of serres (dental lamina)
59
what cells can be present in gingival cyst of adult?
clear glycogen-rich cells
60
where can clear glycogen-rich cells be present?
gingival cyst of adult, clear cell odontogenic carcinoma
61
origin of lateral periodontal cyst
dental lamina rests
62
prevalent location of lateral periodontal cyst
mandibular canine/lat incisor/premolar area
63
what is a botryoid cyst
polycystic lateral periodontal cyst
64
what is the name for a polycystic lateral periodontal cyst
botryoid cyst
65
how many of the COCs are peripheral?
30%
66
mean age of COC
30yo
67
how many COCs are associated with unerupted teeth and which? compare to OKC
30%, canine;; OKC -- 25-40%
68
calcifications are presents in how many COCs?
30-50%
69
how many COCs are cystic or neoplastic?
98% cystic, 2% neoplastic
70
what looks like an ameloblastoma but has ghost cells and dentinoid?
dentinogenic ghost cell tumor
71
histo of dentinogenic ghost cell tumor
similar to ameloblastoma but with ghost cells and dentinoid
72
relation of dentinogenic ghost cell tumor to bone?
peripheral more common than intraosseous
73
what can arise from COC?
SCC, rare
74
aggressive variant of dentinogenic ghost cell tumor?
odontogenic ghost cell carcinoma
75
what odontogenic entity crosses md midline?
glandular odontogenic cyst
76
prevalent location of glandular odontogenic cyst
anterior md, crossing midline
77
what's the recurrence of GOC
30%
78
histo look & IHC of glandular odontogenic cyst
hobnail or papillary epithelial lining, cilia, mucous cells, spherical nodules; mucicarmine +
79
which odontogenic entities can have thickenings of epithelium?
GOC, LPC
80
what predisposes a tooth to buccal bifurcation cyst
enamel extension in bifurcation --> pocket --> cyst; or inflammatory response during eruption
81
which entities may be related to inflammatory response during eruption
buccal bifurcation cyst and paradental cyst
82
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)
83
what is the ddx for carcinoma arising in residual periapical cyst?
met vs de novo
84
origin of ameloblastoma
dental lamina, enamel organ, lining of cyst or basal cell layer of mucosa
85
which variant of ameloblastoma is preferentially in anterior mx
desmoplastic
86
radiographic look of desmoplastic ameloblastoma
mixed RL/RO
87
ddx of desmoplastic ameloblastoma
met
88
what is the effect of TGFb in desmoplastic ameloblastoma
inductive effect around islands, myxoid elsewhere
89
epidemiology of unicystic ameloblastoma
younger patients than multicystic
90
which ameloblastomas present in younger patients
unicystic and granular cell
91
histo variants of unicystic ameloblastoma
luminal, intraluminal, mural
92
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
93
presentation of peripheral ameloblastoma
nodule in posterior gingiva
94
Ddx for nodule in posterior gingiva
peripheral ameloblastoma, peripheral odontogenic fibroma (has dentin or cementum-like material, lacks reverse polarity)
95
peripheral ameloblastoma vs peripheral odontogenic fibroma ddx
peripheral odontogenic fibroma has dentin or cementum-like material, lacks reverse polarity
96
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.
97
ddx of clear cell odontogenic carcinoma
mucoep (mucicarmine +), CEOT (amyloid +), met (renal, breat, melanoma)
98
origin of adenomatoid odontogenic tumor
enamel organ
99
prevalent location of AOT
anterior mx
100
possible concurrent entities with AOT
COC, CEOT, odontoma
101
numeric rule of AOT
2/3 with tooth (usually canine), 2/3 females, 2/3 10-19yo
102
age predilection of AOT
2/3 10-19
103
sex predilection of AOT
f
104
frequency of tooth association of AOT and which tooth
2/3 or like 75%, usually canine
105
histo look of AOT
rosette-like structures with or without eosinophilic material (amyloid +)
106
rosette-like structures with or without eosinophilic material (amyloid +)
AOT
107
radiographic look of AOT if calcifications are present
snowflakes
108
AOT associated with tooth name
follicular (75%), others are extra-follicular
109
CEOT origin
enamel organ or dental lamina
110
prevalent location of CEOT
posterior mandible
111
age of CEOT
30-50yo
112
recurrence of CEOT
15%
113
special stains and histo in CEOT
amyloid-like material + for congo red and thioflavine T;; Liesegang ring calcifications within the amyloid
114
Liesegang ring calcifications
within amyloid of CEOT
115
calcifications in amyloid
CEOT; = Liesegang
116
amyloid in odontogenic entities
AOT, CEOT, maybe central odontogenic fibroma
117
origin of squamous odontogenic tumor
dental lamina or rests of Malassez in periodontal ligament
118
ddx of triangular radiolucent defect lateral to tooth root
perio dz, SOT, LPC, OKC, amelo, LRC
119
most likely odontogenic tumor to have synchronous multiple occurences
SOT
120
histo features of SOT
calcs, eosinophilic structures, amyloid -!
121
ameloblastic fibroma origin
dental papilla
122
age of ameloblastic fibroma
young patients
123
site of ameloblastic fibroma
posterior mandible
124
histo look of ameloblastic fibroma
islands of epithelium; microcystic formation uncommon (unlike ameloblastoma)
125
age of ameloblastic fibro-odontoma
young patients
126
site of ameloblastic fibro-odontoma
posterior mandible
127
histo look of ameloblastic fibro-odontoma
enamel and dentin; vs ameloblastic fibro-dentinoma in which calcifying component is dentin only
128
which component is malignant in ameloblastic fibrosarcoma?
mesenchymal
129
ameloblastic fibrosarcoma arises from
55% de novo, 45% from ameloblastic fibroma
130
which malignancy can arise from ameloblastic fibroma?
ameloblastic fibrosarcoma
131
odontogenic tumor with dysplastic dentin, enamel, and malignant mesenchymal component
ameloblastic dentinosarcoma or fibro-odontosarcoma
132
odontogenic tumor with malignant epithelial and mesenchymal components
ameloblastic carcinosarcoma
133
entity with features of ameloblastoma and complex odontoma
odontoameloblastoma
134
site of odontoameloblastoma
md=mx
135
types of odontoma
complex (mass) and compound (small teeth by the pound)
136
complex odontoma
glob
137
compound odontoma
denticles
138
odontoma with denticles
compound
139
glob odontoma
complex
140
mean age of odontoma
14yo
141
site of odontoma
mx; compound anterior, complex posterior
142
what cells can be present in complex odontomas?
20% show ghost cells
143
what can arise from odontoma lining?
dentigerous cyst(s)
144
site of central odontogenic fibroma
45% in mx (anterior to first molar);; in mandible -- posterior to first molar
145
which odontogenic entity is associated with a cleft-like tissue defect of the maxillary premolar region?
central odontogenic fibroma
146
clinical presentation of central odontogenic fibroma?
can be associated with a cleft-like tissue defect of the maxillary premolar region
147
histo types of central odontogenic fibroma
simple (myxofibroma) and WHO-type (odontogenic epithelium); can have CGCG-like component
148
ddx of central odontogenic fibroma if more collagenized
desmoplastic fibroma
149
site of peripheral odontogenic fibroma
posterior mandibular facial gingiva
150
site of granular cell odontogenic tumor
posterior mandible
151
lysosomal granules NKC13+ are characteristic of
granular cell odontogenic tumor
152
characteristic organelle of granular cell odontogenic tumor
lysosomal granules NKC13+
153
recurrence of myxoma
25%
154
what differentiates fibromyxoma or myxofibroma from regular myxoma
more collagen
155
ddx of myxoma vs chondromyxoid fibroma
chondromyxoid fibroma has cartilage
156
ddx of myxoma vs myxoid neurofibroma
myxoid neurofibroma is s100+
157
what's the ddx for myxoma
chondromyxoid fibroma has cartilage; myxoid neurofibroma is s100+
158
malignant myxoma and its histo features
myxosarcoma: marked cellularity and cellular atypia
159
myxoma with marked cellularity and cellular atypia
myxosarcoma, malignant
160
odontogenic entity with BRAF
ameloblastoma
161
odontogenic entity with PTCH
OKC
162
odontogenic cells with Wnt/beta catenin
ghost cells
163
mucoep mutation
t(11;19) CRTC-MAML2 -- Notch pathway
164
Notch pathway mutation in
mucoep
165
t(11;19) mutations
mucoep
166
CRTC-MAML2 mutation
mucoep
167
clear cell odontogenic carcinoma mutation
EWSR1-ATF1
168
EWSR1-ATF1 mutation
clear cell odontogenic carcinoma
169
mets of malignant ameloblastoma
lungs and cervical LNs
170
cysts associated with mandibular PM/M
LPC and gingival cyst
171
PTCH is which pathway
SHH
172
odontogenic tumors constitute ____% of specimens in oral path labs
<1%
173
prevalence of NBCCS
1:60,000
174
T or F, buccal bifurcation cyst is developmental
F, inflammatory
175
odontogenic cyst associated with proliferative periostitis
buccal bifurcation
176
T or F, OKCs enlarge 2/2 osmotic pressure
F, factors unknown
177
T or F, OKCs comprise 20% of all odontogenic cysts
F, 3-11%
178
Lateral periodontal cysts account for ____% of all epithelial lined jaw cysts
<2%
179
gender predilection of OOCs
2:1 M:F
180
oncoproteins in syndromic OKCs
p53 and cyclin D1 (bcl-1)
181
how many unicystic ameloblastomas are in posterior mandible
90%
182
least common ameloblastoma
basal cell variant
183
recommended surgical margins of ameloblastoma resection
1-1.5 cm beyond radiographic
184
most dangerous location for ameloblastoma
posterior maxilla
185
treatment recommendation for ameloblastic fibroma
initial conservative, aggressive for recurrence
186
average age and gender for myxoma
25-30yo, no gender predilection
187
average age of ameloblastic fibro-odontoma
10yo
188
Ameloblastic fibro-odontoma gender predilection
M:F 3:2
189
odontogenic fibromas gender predilection
F>M, 40 yo mean
