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