Odontogenic Cysts - part II Flashcards

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
Q

tx of gingival cyst of newborn

A

none

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26
Q

T/F: gingival cyst of newborn self-marsupialize or involute/degenerate spontaneously

A

true

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27
Q

px of gingival cyst of newborn

A

excellent, rarely found after 3 months

28
Q

what is the gingival cyst of adult related to?

A

lateral periodontal cyst

29
Q

where does lateral periodontal cyst arise from?

A

rests of dental lamina (Serres) found in the gingival CT

30
Q

cystic degeneration of the rests in lateral periodontal cyst gives rise to what?

A

gingival cyst

31
Q

T/F: gingival cyst of adult has a female predilection

A

false, NO gender predilection

32
Q

where does gingival cyst of adult occur?

A

anterior segments of jaws

33
Q

gingival cyst of adult occur above or below what?

A

MG jxn

34
Q

T/F: gingival cyst of adult is usually lingual, sometimes facial

A

false, facial, sometimes lingual

35
Q

clinical features of gingival cyst of adult

A
  1. smooth-surfaced, dome shape
  2. <1 cm
  3. feels tense to palpation
  4. asymptomatic
  5. no color change with early lesions
36
Q

what color are larger gingival cyst of adult lesions?

A

bluish or translucent

37
Q

radiographic features of gingival cyst of adult

A
  1. minimal, if any, radiolucent change

2. cyst within CT

38
Q

histopathologic features of gingival cyst of adult

A
  1. cyst lined by thin, uniform layer of cuboidal or attenuated (thinned) stratified squamous epithelium with CT
  2. may see localized nodular thickening
39
Q

tx of gingival cyst of adult

A

conservative excision

40
Q

px of gingival cyst of adult

A

excellent

41
Q

lateral periodontal cyst

A

non-keratinized developmental cyst occurring adjacent or lateral to the root of a tooth, probably arising from rests of Serres

42
Q

T/F: lateral periodontal cyst are usually ofund incidentally on radiographs

A

true

43
Q

T/F: lateral periodontal cyst is symptomatic

A

false, asymptomatic

44
Q

what should always be done when dx lateral periodontal cyst?

A

always vitality test

45
Q

radiographic features of lateral periodontal cyst

A
  1. radiolucent, usually unilocular but can appear multiolocular
  2. usually less than 1 cm in diameter
46
Q

where is the most common location radiographically for lateral periodontal cyst?

A

mand premolar-canine-lateral incisor area

47
Q

histopathologic features of lateral periodontal cyst

A

cyst lined by thin, uniform stratified squamous epithelium within fibrous CT

48
Q

lateral periodontal cyst is identical to what histopathologically?

A

gingival cyst of adult

49
Q

tx of lateral periodontal cyst

A

curettage

50
Q

prognosis of lateral periodontal cyst

A

excellent

51
Q

T/F: lateral periodontal cyst commonly recurs

A

false, rarely recurs

52
Q

what should be done after tx’ing lateral periodontal cyst?

A

periodic radiographic follow-up

53
Q

where does calcifying odontogenic cyst (Gorlin cyst) arise from?

A

from rests of Serres

54
Q

T/F: maxilla is more affected by Gorlin cyst

A

false, equal distribution b/w maxilla and mandible

55
Q

who does Gorlin cyst affect?

A

any age, but peak in 2nd-4th decade

56
Q

which area does Gorlin cyst appear majority of the time?

A

incisor-canine region

57
Q

clinical features of Gorlin cyst

A
  1. usually intrabony

2. may expand alveolar bone

58
Q

Gorlin cyst in gingival soft tissue is called what?

A

peripheral calcifying odontogenic cyst

59
Q

radiographic features of Gorlin cyst

A
  1. well-defined unilocular radiolucency
  2. resorption and divergence of adjacent roots often seen
  3. scattered radiopacities
60
Q

what is Gorlin cyst often associated with?

A
  1. impacted tooth

2. odontoma

61
Q

histopathologic features of Gorlin cyst

A
  1. peripheral palisading cells and stellate reticulum-like areas
  2. some lesional cells undergo “ghost cell” change then may calcify
62
Q

what is Gorlin cyst similar to histopathologically?

A

odontogenic tumor ameloblastoma

63
Q

ghost cells

A

pale eosinophilic, with swollen cystoplasm, loss of the nucleus that exhibits a faint “ghostly” remnant of a nuclear membrane outline

64
Q

tx of Gorlin cyst

A

enucleation w curettage

65
Q

px of Gorlin cyst

A

good

66
Q

T/F: recurrence of Gorlin cyst is not common but occurs

A

true

67
Q

what is needed after tx’ing Gorlin cyst?

A

periodic radiographic follow-up