Odontogenic Cysts - part II Flashcards
nevoid basal cell carcinoma syndrome (NBCCS)
complex hamartoneoplastic/malformation syndrome exhibiting over 100 different signs and symps related to skin, CNS and skeletal system
what occurs to ppl with NBCCS?
develop multiple odontogenic keratocysts at an early age
NBCCS is also known as what?
- Gorlin syndrome
2. Gorlin-Goltz syndrome
T/F: NBCCS is autosomal recessive
false, dominant
which chromosome does NBCCS affect?
9
T/F: NBCCS has complete penetrance and extremely variable expressivity
true
40% of NBCCS cases are new mutations related to which gene?
PTCH
what develops in at least 75% of affected NBCCS pts?
OKCs of the jaws
when does OKCs of the jaw develop for NBCCS pts?
begin in 1st decade, after age 7
craniofacial features of NBCCS
- enlarged occipitofrontal circumference
- heavy brow ridges - frontal bossing
- broad nasal root
- mild ocular hypertelorism (wide-set)
skin features of NBCCS
- milia and multiple epidermal cysts
- 1-2 mm shallow pits of palms and/or soles
- basal cell carcinomas
what is different about basal cell carcinomas that develop on the skin of NBCCS pts?
- are multiple
- occur in unexposed, as well as exposed, skin
- develop at an earlier age (puberty-35 yrs)
- often show melanin pigmentation
- are usually quiescent, with a few being aggressive
radiographic features of NBCCS
- lamellar calcification of falx cerebri
- bifid, fused or hypoplastic ribs
- multiple OKCs maxilla, mandible or both
tx for NBCCS
- genetic counseling
- remove OKCs as needed
- remove BCCs as needed
- sunscreen and reduce sun exposure to decrease risk of BCC
px of NBCCS
guarded
T/F: BCC in NBCCS pts are small and not very aggressive, but this is variable
true
gingival cyst of newborn
“dental lamina cyst”
gingival cyst of newborn represents what?
an enlarged version of the normal microcystic structures that arise from dental lamina rests (rests of Serres)
what is gingival cyst of newborn similar to?
- Epstein’s pearls
2. Bohn’s nodules
Epstein’s pearls
cysts found on palatal raphe
Bohn’s nodules
cysts laterally along hard/soft palate
clinical features of gingival cyst of newborn
- 1-2 mm yellow-white papules
- often multiple
- on alveolar ridge of newborn
where does gingival cyst of newborn occur?
usually maxillary
histopathologic features of gingival cyst of newborn
- thin, uniform stratified squamous epithelial lining
2. keratin debris in cyst lining
tx of gingival cyst of newborn
none
T/F: gingival cyst of newborn self-marsupialize or involute/degenerate spontaneously
true
px of gingival cyst of newborn
excellent, rarely found after 3 months
what is the gingival cyst of adult related to?
lateral periodontal cyst
where does lateral periodontal cyst arise from?
rests of dental lamina (Serres) found in the gingival CT
cystic degeneration of the rests in lateral periodontal cyst gives rise to what?
gingival cyst
T/F: gingival cyst of adult has a female predilection
false, NO gender predilection
where does gingival cyst of adult occur?
anterior segments of jaws
gingival cyst of adult occur above or below what?
MG jxn
T/F: gingival cyst of adult is usually lingual, sometimes facial
false, facial, sometimes lingual
clinical features of gingival cyst of adult
- smooth-surfaced, dome shape
- <1 cm
- feels tense to palpation
- asymptomatic
- no color change with early lesions
what color are larger gingival cyst of adult lesions?
bluish or translucent
radiographic features of gingival cyst of adult
- minimal, if any, radiolucent change
2. cyst within CT
histopathologic features of gingival cyst of adult
- cyst lined by thin, uniform layer of cuboidal or attenuated (thinned) stratified squamous epithelium with CT
- may see localized nodular thickening
tx of gingival cyst of adult
conservative excision
px of gingival cyst of adult
excellent
lateral periodontal cyst
non-keratinized developmental cyst occurring adjacent or lateral to the root of a tooth, probably arising from rests of Serres
T/F: lateral periodontal cyst are usually ofund incidentally on radiographs
true
T/F: lateral periodontal cyst is symptomatic
false, asymptomatic
what should always be done when dx lateral periodontal cyst?
always vitality test
radiographic features of lateral periodontal cyst
- radiolucent, usually unilocular but can appear multiolocular
- usually less than 1 cm in diameter
where is the most common location radiographically for lateral periodontal cyst?
mand premolar-canine-lateral incisor area
histopathologic features of lateral periodontal cyst
cyst lined by thin, uniform stratified squamous epithelium within fibrous CT
lateral periodontal cyst is identical to what histopathologically?
gingival cyst of adult
tx of lateral periodontal cyst
curettage
prognosis of lateral periodontal cyst
excellent
T/F: lateral periodontal cyst commonly recurs
false, rarely recurs
what should be done after tx’ing lateral periodontal cyst?
periodic radiographic follow-up
where does calcifying odontogenic cyst (Gorlin cyst) arise from?
from rests of Serres
T/F: maxilla is more affected by Gorlin cyst
false, equal distribution b/w maxilla and mandible
who does Gorlin cyst affect?
any age, but peak in 2nd-4th decade
which area does Gorlin cyst appear majority of the time?
incisor-canine region
clinical features of Gorlin cyst
- usually intrabony
2. may expand alveolar bone
Gorlin cyst in gingival soft tissue is called what?
peripheral calcifying odontogenic cyst
radiographic features of Gorlin cyst
- well-defined unilocular radiolucency
- resorption and divergence of adjacent roots often seen
- scattered radiopacities
what is Gorlin cyst often associated with?
- impacted tooth
2. odontoma
histopathologic features of Gorlin cyst
- peripheral palisading cells and stellate reticulum-like areas
- some lesional cells undergo “ghost cell” change then may calcify
what is Gorlin cyst similar to histopathologically?
odontogenic tumor ameloblastoma
ghost cells
pale eosinophilic, with swollen cystoplasm, loss of the nucleus that exhibits a faint “ghostly” remnant of a nuclear membrane outline
tx of Gorlin cyst
enucleation w curettage
px of Gorlin cyst
good
T/F: recurrence of Gorlin cyst is not common but occurs
true
what is needed after tx’ing Gorlin cyst?
periodic radiographic follow-up