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OP - Yield > 15: Odontogenic cysts and tumors > Flashcards

15: Odontogenic cysts and tumors Flashcards

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

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2
Q

Gorlin cyst aka? Name if solid?

A

Calcifying odontogenic cyst; if solid = dentinogenic ghost cell tumor

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3
Q

Feature of calcifying odontogenic cyst presentation

A

20% associated with odontoma; also can be AOT, ameloblastoma, ameloblastic fibroma

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4
Q

Buccal bifurcation cyst location, local effect

A

Buccal of mandibular first molar; lingual displacement of roots seen on occlusal film

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5
Q

Paradental cyst location

A

Distal to partially erupted third molar

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6
Q

Presentation and histo of granular cell ameloblastoma

A

More aggressive, younger patients, contains lysosomes

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7
Q

Dentinogenic ghost cell tumor is a counterpart of what?

A

Solid counterpart of COC

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8
Q

What entity can lingually displace molar roots?

A

Buccal bifurcation cyst

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9
Q

What entity can be associated with an odontoma?

A

COC

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10
Q

What marker is increased in desmoplastic ameloblastoma?

A

TGFbeta

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11
Q

What ameloblastoma features an increase in TGFbeta?

A

Desmoplastic

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12
Q

Cytologic feature of clear cell odontogenic carcinoma

A

Glycogen-rich presecretory ameloblasts

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13
Q

Glycogen-rich presecretory ameloblasts are a feature of what entity?

A

Clear cell odontogenic carcinoma

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14
Q

Patterns of clear cell odontogenic carcinoma

A

monophasic, biphasic (clear/epithelial), clear cell ameloblastoma-like

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15
Q

Radiographic feature of CEOT

A

Driven-snow calcifications around the crown of impacted tooth

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16
Q

Driven-snow calcifications around the crown of impacted tooth are a radiographic feature of what entity?

A

CEOT (Calcifying epithelial odontogenic tumor)

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17
Q

How does squamous odontogenic tumor present radiographically?

A

triangular radiolucent defect lateral to a root (similar to vertical periodontal bone loss)

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18
Q

what entities can have squamous odontogenic tumor-like proliferations?

A

dentigerous and radicular cysts

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19
Q

Which odontogenic entities have mx=md prevalence?

A

SOT, COC, central odontogenic fibroma, odontoameloblastoma

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20
Q

Which odontogenic entities have mx prevalence?

A

desmoplastic ameloblastoma, odontomas, AOT

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21
Q

Prevalent location of SOT

A

mx=md

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22
Q

Prevalent location of COC

A

mx=md, anterior

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23
Q

Prevalent location of odontogenic fibroma

A

mx=md

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24
Q

Prevalent location of desmoplastic ameloblastoma

A

anterior mx

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25
Q

Prevalent location of odontoma

A

mx

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26
Q

What feature can be present in a central odontogenic fibroma

A

CGCG-like component

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27
Q

What odontogenic entity can have a CGCG-like component?

A

Central odontogenic fibroma

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28
Q

Origin and IHC profile of cells of granular cell odontogenic tumor

A

mesenchymal, S100-, lysosomal granules NKC13+

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29
Q

Composition and IHC of myxoma

A

ground substance hyaluronic acid and chondroitin sulfate. Vimentin diffuse, MSA focal (muscle-specific actin)

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30
Q

Which entity is composed of ground substance hyaluronic acid and chondroitin sulfate?

A

Myxoma

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31
Q

Pindborg tumor is aka

A

CEOT

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32
Q

CEOT is aka

A

Pindborg tumor

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33
Q

most common developmental odontogenic cyst

A

dentigerous cyst

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34
Q

how does a dentigerous cyst develop

A

fluid accumulates between reduced enamel epithelium and crown of tooth

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35
Q

what entity forms by accumulation of fluid between reduced enamel epithelium and crown of tooth

A

dentigerous cyst

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36
Q

possible relation of dentigerous cyst to tooth

A

central, lateral, circumferential

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37
Q

composition of wall of dentigerous cyst

A

glycosaminoglycan ground substance

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38
Q

what can arise from the lining of dentigerous cyst?

A

ameloblastoma, SCC, mucoep (latter if mucous cells are present)

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39
Q

what is the soft tissue counterpart of a dentigerous cyst?

A

eruption cyst

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40
Q

what happens when an eruption cyst is traumatized and bleeds?

A

eruption hematoma

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41
Q

what is the origin of an OKC?

A

dental lamina rests

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42
Q

prevalent location of OKC

A

posterior mandible

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43
Q

OKC age

A

10-40yo

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44
Q

OKC recurrence

A

30% (up to 10y)

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45
Q

what percentage of OKCs is associated with an impacted tooth?

A

25-40%

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46
Q

what malignant transformation can OKC undergo?

A

SCC, rare

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47
Q

how is OOC different from OKC?

A

clinically similar; epithelium is obvi orthokeratinized; recurrence is 2%

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48
Q

Features of BCCs in Gorlin syndrome?

A

less aggressive, younger age; fewer BCCs in blacks than whites

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49
Q

Histo and marker features of OKCs in Gorlin syndrome?

A

more satellite lesions, solid islands of epithelial proliferation and odontogenic rests; overexpression of p53 and cyclin D1 (bcl-1)

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50
Q

origin of gingival cyst of newborn?

A

dental lamina rests

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51
Q

clinical presentation of gingival cyst of newborn?

A

multiple whitish papules (keratin filled cysts) on mx alveolar process

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52
Q

which entity presents as multiple whitish papules (keratin filled cysts) on mx alveolar process?

A

gingival cyst of newborn

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53
Q

how many newborns get gingival cysts

A

50%

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54
Q

location of gingival cyst of newborn?

A

mx alveolar process

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55
Q

eruption cyst is a soft tissue counterpart of what

A

dentigerous cyst

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56
Q

what is the soft tissue counterpart of lateral periodontal cyst?

A

gingival cyst of adult

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57
Q

gingival cyst of adult is a soft tissue counterpart of what?

A

lateral periodontal cyst

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58
Q

origin of gingival cyst of adult

A

rests of serres (dental lamina)

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59
Q

what cells can be present in gingival cyst of adult?

A

clear glycogen-rich cells

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60
Q

where can clear glycogen-rich cells be present?

A

gingival cyst of adult, clear cell odontogenic carcinoma

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61
Q

origin of lateral periodontal cyst

A

dental lamina rests

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62
Q

prevalent location of lateral periodontal cyst

A

mandibular canine/lat incisor/premolar area

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63
Q

what is a botryoid cyst

A

polycystic lateral periodontal cyst

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64
Q

what is the name for a polycystic lateral periodontal cyst

A

botryoid cyst

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65
Q

how many of the COCs are peripheral?

A

30%

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66
Q

mean age of COC

A

30yo

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67
Q

how many COCs are associated with unerupted teeth and which? compare to OKC

A

30%, canine;; OKC – 25-40%

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68
Q

calcifications are presents in how many COCs?

A

30-50%

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69
Q

how many COCs are cystic or neoplastic?

A

98% cystic, 2% neoplastic

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70
Q

what looks like an ameloblastoma but has ghost cells and dentinoid?

A

dentinogenic ghost cell tumor

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71
Q

histo of dentinogenic ghost cell tumor

A

similar to ameloblastoma but with ghost cells and dentinoid

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72
Q

relation of dentinogenic ghost cell tumor to bone?

A

peripheral more common than intraosseous

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73
Q

what can arise from COC?

A

SCC, rare

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74
Q

aggressive variant of dentinogenic ghost cell tumor?

A

odontogenic ghost cell carcinoma

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75
Q

what odontogenic entity crosses md midline?

A

glandular odontogenic cyst

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76
Q

prevalent location of glandular odontogenic cyst

A

anterior md, crossing midline

77
Q

what’s the recurrence of GOC

A

30%

78
Q

histo look & IHC of glandular odontogenic cyst

A

hobnail or papillary epithelial lining, cilia, mucous cells, spherical nodules; mucicarmine +

79
Q

which odontogenic entities can have thickenings of epithelium?

A

GOC, LPC

80
Q

what predisposes a tooth to buccal bifurcation cyst

A

enamel extension in bifurcation –> pocket –> cyst; or inflammatory response during eruption

81
Q

which entities may be related to inflammatory response during eruption

A

buccal bifurcation cyst and paradental cyst

82
Q

how can carcinoma happen in odontogenic entities? which cysts can give rise to carcinoma?

A

de novo, from ameloblastoma, from odontogenic tumor or odontogenic cyst (DC, OKC, OOC, LPC)

83
Q

what is the ddx for carcinoma arising in residual periapical cyst?

A

met vs de novo

84
Q

origin of ameloblastoma

A

dental lamina, enamel organ, lining of cyst or basal cell layer of mucosa

85
Q

which variant of ameloblastoma is preferentially in anterior mx

A

desmoplastic

86
Q

radiographic look of desmoplastic ameloblastoma

A

mixed RL/RO

87
Q

ddx of desmoplastic ameloblastoma

A

met

88
Q

what is the effect of TGFb in desmoplastic ameloblastoma

A

inductive effect around islands, myxoid elsewhere

89
Q

epidemiology of unicystic ameloblastoma

A

younger patients than multicystic

90
Q

which ameloblastomas present in younger patients

A

unicystic and granular cell

91
Q

histo variants of unicystic ameloblastoma

A

luminal, intraluminal, mural

92
Q

criteria for unicystic ameloblastoma

A

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
Q

presentation of peripheral ameloblastoma

A

nodule in posterior gingiva

94
Q

Ddx for nodule in posterior gingiva

A

peripheral ameloblastoma, peripheral odontogenic fibroma (has dentin or cementum-like material, lacks reverse polarity)

95
Q

peripheral ameloblastoma vs peripheral odontogenic fibroma ddx

A

peripheral odontogenic fibroma has dentin or cementum-like material, lacks reverse polarity

96
Q

malignant ameloblastoma vs ameloblastic carcinoma (histo, age, mets)

A

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
Q

ddx of clear cell odontogenic carcinoma

A

mucoep (mucicarmine +), CEOT (amyloid +), met (renal, breat, melanoma)

98
Q

origin of adenomatoid odontogenic tumor

A

enamel organ

99
Q

prevalent location of AOT

A

anterior mx

100
Q

possible concurrent entities with AOT

A

COC, CEOT, odontoma

101
Q

numeric rule of AOT

A

2/3 with tooth (usually canine), 2/3 females, 2/3 10-19yo

102
Q

age predilection of AOT

A

2/3 10-19

103
Q

sex predilection of AOT

A

f

104
Q

frequency of tooth association of AOT and which tooth

A

2/3 or like 75%, usually canine

105
Q

histo look of AOT

A

rosette-like structures with or without eosinophilic material (amyloid +)

106
Q

rosette-like structures with or without eosinophilic material (amyloid +)

A

AOT

107
Q

radiographic look of AOT if calcifications are present

A

snowflakes

108
Q

AOT associated with tooth name

A

follicular (75%), others are extra-follicular

109
Q

CEOT origin

A

enamel organ or dental lamina

110
Q

prevalent location of CEOT

A

posterior mandible

111
Q

age of CEOT

A

30-50yo

112
Q

recurrence of CEOT

A

15%

113
Q

special stains and histo in CEOT

A

amyloid-like material + for congo red and thioflavine T;; Liesegang ring calcifications within the amyloid

114
Q

Liesegang ring calcifications

A

within amyloid of CEOT

115
Q

calcifications in amyloid

A

CEOT; = Liesegang

116
Q

amyloid in odontogenic entities

A

AOT, CEOT, maybe central odontogenic fibroma

117
Q

origin of squamous odontogenic tumor

A

dental lamina or rests of Malassez in periodontal ligament

118
Q

ddx of triangular radiolucent defect lateral to tooth root

A

perio dz, SOT, LPC, OKC, amelo, LRC

119
Q

most likely odontogenic tumor to have synchronous multiple occurences

A

SOT

120
Q

histo features of SOT

A

calcs, eosinophilic structures, amyloid -!

121
Q

ameloblastic fibroma origin

A

dental papilla

122
Q

age of ameloblastic fibroma

A

young patients

123
Q

site of ameloblastic fibroma

A

posterior mandible

124
Q

histo look of ameloblastic fibroma

A

islands of epithelium; microcystic formation uncommon (unlike ameloblastoma)

125
Q

age of ameloblastic fibro-odontoma

A

young patients

126
Q

site of ameloblastic fibro-odontoma

A

posterior mandible

127
Q

histo look of ameloblastic fibro-odontoma

A

enamel and dentin; vs ameloblastic fibro-dentinoma in which calcifying component is dentin only

128
Q

which component is malignant in ameloblastic fibrosarcoma?

A

mesenchymal

129
Q

ameloblastic fibrosarcoma arises from

A

55% de novo, 45% from ameloblastic fibroma

130
Q

which malignancy can arise from ameloblastic fibroma?

A

ameloblastic fibrosarcoma

131
Q

odontogenic tumor with dysplastic dentin, enamel, and malignant mesenchymal component

A

ameloblastic dentinosarcoma or fibro-odontosarcoma

132
Q

odontogenic tumor with malignant epithelial and mesenchymal components

A

ameloblastic carcinosarcoma

133
Q

entity with features of ameloblastoma and complex odontoma

A

odontoameloblastoma

134
Q

site of odontoameloblastoma

A

md=mx

135
Q

types of odontoma

A

complex (mass) and compound (small teeth by the pound)

136
Q

complex odontoma

A

glob

137
Q

compound odontoma

A

denticles

138
Q

odontoma with denticles

A

compound

139
Q

glob odontoma

A

complex

140
Q

mean age of odontoma

A

14yo

141
Q

site of odontoma

A

mx; compound anterior, complex posterior

142
Q

what cells can be present in complex odontomas?

A

20% show ghost cells

143
Q

what can arise from odontoma lining?

A

dentigerous cyst(s)

144
Q

site of central odontogenic fibroma

A

45% in mx (anterior to first molar);; in mandible – posterior to first molar

145
Q

which odontogenic entity is associated with a cleft-like tissue defect of the maxillary premolar region?

A

central odontogenic fibroma

146
Q

clinical presentation of central odontogenic fibroma?

A

can be associated with a cleft-like tissue defect of the maxillary premolar region

147
Q

histo types of central odontogenic fibroma

A

simple (myxofibroma) and WHO-type (odontogenic epithelium); can have CGCG-like component

148
Q

ddx of central odontogenic fibroma if more collagenized

A

desmoplastic fibroma

149
Q

site of peripheral odontogenic fibroma

A

posterior mandibular facial gingiva

150
Q

site of granular cell odontogenic tumor

A

posterior mandible

151
Q

lysosomal granules NKC13+ are characteristic of

A

granular cell odontogenic tumor

152
Q

characteristic organelle of granular cell odontogenic tumor

A

lysosomal granules NKC13+

153
Q

recurrence of myxoma

A

25%

154
Q

what differentiates fibromyxoma or myxofibroma from regular myxoma

A

more collagen

155
Q

ddx of myxoma vs chondromyxoid fibroma

A

chondromyxoid fibroma has cartilage

156
Q

ddx of myxoma vs myxoid neurofibroma

A

myxoid neurofibroma is s100+

157
Q

what’s the ddx for myxoma

A

chondromyxoid fibroma has cartilage; myxoid neurofibroma is s100+

158
Q

malignant myxoma and its histo features

A

myxosarcoma: marked cellularity and cellular atypia

159
Q

myxoma with marked cellularity and cellular atypia

A

myxosarcoma, malignant

160
Q

odontogenic entity with BRAF

A

ameloblastoma

161
Q

odontogenic entity with PTCH

A

OKC

162
Q

odontogenic cells with Wnt/beta catenin

A

ghost cells

163
Q

mucoep mutation

A

t(11;19) CRTC-MAML2 – Notch pathway

164
Q

Notch pathway mutation in

A

mucoep

165
Q

t(11;19) mutations

A

mucoep

166
Q

CRTC-MAML2 mutation

A

mucoep

167
Q

clear cell odontogenic carcinoma mutation

A

EWSR1-ATF1

168
Q

EWSR1-ATF1 mutation

A

clear cell odontogenic carcinoma

169
Q

mets of malignant ameloblastoma

A

lungs and cervical LNs

170
Q

cysts associated with mandibular PM/M

A

LPC and gingival cyst

171
Q

PTCH is which pathway

A

SHH

172
Q

odontogenic tumors constitute ____% of specimens in oral path labs

A

<1%

173
Q

prevalence of NBCCS

A

1:60,000

174
Q

T or F, buccal bifurcation cyst is developmental

A

F, inflammatory

175
Q

odontogenic cyst associated with proliferative periostitis

A

buccal bifurcation

176
Q

T or F, OKCs enlarge 2/2 osmotic pressure

A

F, factors unknown

177
Q

T or F, OKCs comprise 20% of all odontogenic cysts

A

F, 3-11%

178
Q

Lateral periodontal cysts account for ____% of all epithelial lined jaw cysts

A

<2%

179
Q

gender predilection of OOCs

A

2:1 M:F

180
Q

oncoproteins in syndromic OKCs

A

p53 and cyclin D1 (bcl-1)

181
Q

how many unicystic ameloblastomas are in posterior mandible

A

90%

182
Q

least common ameloblastoma

A

basal cell variant

183
Q

recommended surgical margins of ameloblastoma resection

A

1-1.5 cm beyond radiographic

184
Q

most dangerous location for ameloblastoma

A

posterior maxilla

185
Q

treatment recommendation for ameloblastic fibroma

A

initial conservative, aggressive for recurrence

186
Q

average age and gender for myxoma

A

25-30yo, no gender predilection

187
Q

average age of ameloblastic fibro-odontoma

A

10yo

188
Q

Ameloblastic fibro-odontoma gender predilection

A

M:F 3:2

189
Q

odontogenic fibromas gender predilection

A

F>M, 40 yo mean

OP - Yield (19 decks)

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