Odontogenic Cysts - part II Flashcards

(67 cards)

1
Q

nevoid basal cell carcinoma syndrome (NBCCS)

A

complex hamartoneoplastic/malformation syndrome exhibiting over 100 different signs and symps related to skin, CNS and skeletal system

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2
Q

what occurs to ppl with NBCCS?

A

develop multiple odontogenic keratocysts at an early age

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3
Q

NBCCS is also known as what?

A
  1. Gorlin syndrome

2. Gorlin-Goltz syndrome

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4
Q

T/F: NBCCS is autosomal recessive

A

false, dominant

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5
Q

which chromosome does NBCCS affect?

A

9

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6
Q

T/F: NBCCS has complete penetrance and extremely variable expressivity

A

true

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7
Q

40% of NBCCS cases are new mutations related to which gene?

A

PTCH

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8
Q

what develops in at least 75% of affected NBCCS pts?

A

OKCs of the jaws

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9
Q

when does OKCs of the jaw develop for NBCCS pts?

A

begin in 1st decade, after age 7

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10
Q

craniofacial features of NBCCS

A
  1. enlarged occipitofrontal circumference
  2. heavy brow ridges - frontal bossing
  3. broad nasal root
  4. mild ocular hypertelorism (wide-set)
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11
Q

skin features of NBCCS

A
  1. milia and multiple epidermal cysts
  2. 1-2 mm shallow pits of palms and/or soles
  3. basal cell carcinomas
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12
Q

what is different about basal cell carcinomas that develop on the skin of NBCCS pts?

A
  1. are multiple
  2. occur in unexposed, as well as exposed, skin
  3. develop at an earlier age (puberty-35 yrs)
  4. often show melanin pigmentation
  5. are usually quiescent, with a few being aggressive
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13
Q

radiographic features of NBCCS

A
  1. lamellar calcification of falx cerebri
  2. bifid, fused or hypoplastic ribs
  3. multiple OKCs maxilla, mandible or both
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14
Q

tx for NBCCS

A
  1. genetic counseling
  2. remove OKCs as needed
  3. remove BCCs as needed
  4. sunscreen and reduce sun exposure to decrease risk of BCC
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15
Q

px of NBCCS

A

guarded

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16
Q

T/F: BCC in NBCCS pts are small and not very aggressive, but this is variable

A

true

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17
Q

gingival cyst of newborn

A

“dental lamina cyst”

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18
Q

gingival cyst of newborn represents what?

A

an enlarged version of the normal microcystic structures that arise from dental lamina rests (rests of Serres)

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19
Q

what is gingival cyst of newborn similar to?

A
  1. Epstein’s pearls

2. Bohn’s nodules

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20
Q

Epstein’s pearls

A

cysts found on palatal raphe

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21
Q

Bohn’s nodules

A

cysts laterally along hard/soft palate

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22
Q

clinical features of gingival cyst of newborn

A
  1. 1-2 mm yellow-white papules
  2. often multiple
  3. on alveolar ridge of newborn
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23
Q

where does gingival cyst of newborn occur?

A

usually maxillary

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24
Q

histopathologic features of gingival cyst of newborn

A
  1. thin, uniform stratified squamous epithelial lining

2. keratin debris in cyst lining

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25
tx of gingival cyst of newborn
none
26
T/F: gingival cyst of newborn self-marsupialize or involute/degenerate spontaneously
true
27
px of gingival cyst of newborn
excellent, rarely found after 3 months
28
what is the gingival cyst of adult related to?
lateral periodontal cyst
29
where does lateral periodontal cyst arise from?
rests of dental lamina (Serres) found in the gingival CT
30
cystic degeneration of the rests in lateral periodontal cyst gives rise to what?
gingival cyst
31
T/F: gingival cyst of adult has a female predilection
false, NO gender predilection
32
where does gingival cyst of adult occur?
anterior segments of jaws
33
gingival cyst of adult occur above or below what?
MG jxn
34
T/F: gingival cyst of adult is usually lingual, sometimes facial
false, facial, sometimes lingual
35
clinical features of gingival cyst of adult
1. smooth-surfaced, dome shape 2. <1 cm 3. feels tense to palpation 4. asymptomatic 5. no color change with early lesions
36
what color are larger gingival cyst of adult lesions?
bluish or translucent
37
radiographic features of gingival cyst of adult
1. minimal, if any, radiolucent change | 2. cyst within CT
38
histopathologic features of gingival cyst of adult
1. cyst lined by thin, uniform layer of cuboidal or attenuated (thinned) stratified squamous epithelium with CT 2. may see localized nodular thickening
39
tx of gingival cyst of adult
conservative excision
40
px of gingival cyst of adult
excellent
41
lateral periodontal cyst
non-keratinized developmental cyst occurring adjacent or lateral to the root of a tooth, probably arising from rests of Serres
42
T/F: lateral periodontal cyst are usually ofund incidentally on radiographs
true
43
T/F: lateral periodontal cyst is symptomatic
false, asymptomatic
44
what should always be done when dx lateral periodontal cyst?
always vitality test
45
radiographic features of lateral periodontal cyst
1. radiolucent, usually unilocular but can appear multiolocular 2. usually less than 1 cm in diameter
46
where is the most common location radiographically for lateral periodontal cyst?
mand premolar-canine-lateral incisor area
47
histopathologic features of lateral periodontal cyst
cyst lined by thin, uniform stratified squamous epithelium within fibrous CT
48
lateral periodontal cyst is identical to what histopathologically?
gingival cyst of adult
49
tx of lateral periodontal cyst
curettage
50
prognosis of lateral periodontal cyst
excellent
51
T/F: lateral periodontal cyst commonly recurs
false, rarely recurs
52
what should be done after tx'ing lateral periodontal cyst?
periodic radiographic follow-up
53
where does calcifying odontogenic cyst (Gorlin cyst) arise from?
from rests of Serres
54
T/F: maxilla is more affected by Gorlin cyst
false, equal distribution b/w maxilla and mandible
55
who does Gorlin cyst affect?
any age, but peak in 2nd-4th decade
56
which area does Gorlin cyst appear majority of the time?
incisor-canine region
57
clinical features of Gorlin cyst
1. usually intrabony | 2. may expand alveolar bone
58
Gorlin cyst in gingival soft tissue is called what?
peripheral calcifying odontogenic cyst
59
radiographic features of Gorlin cyst
1. well-defined unilocular radiolucency 2. resorption and divergence of adjacent roots often seen 3. scattered radiopacities
60
what is Gorlin cyst often associated with?
1. impacted tooth | 2. odontoma
61
histopathologic features of Gorlin cyst
1. peripheral palisading cells and stellate reticulum-like areas 2. some lesional cells undergo "ghost cell" change then may calcify
62
what is Gorlin cyst similar to histopathologically?
odontogenic tumor ameloblastoma
63
ghost cells
pale eosinophilic, with swollen cystoplasm, loss of the nucleus that exhibits a faint "ghostly" remnant of a nuclear membrane outline
64
tx of Gorlin cyst
enucleation w curettage
65
px of Gorlin cyst
good
66
T/F: recurrence of Gorlin cyst is not common but occurs
true
67
what is needed after tx'ing Gorlin cyst?
periodic radiographic follow-up