Lin 3/9/15 Flashcards
Syndromes we are learning:
multiple hamartomas
skin and mucosa
bone
non-genetic
hamartomas
- not a true tumor
- originate from original tissue
Gorlin syndrome is aka
nevoid basal cell carcinoma syndrome
PTCH is a _____ ____ gene
patched is hereditary
tumor suppressor
true
what are the clinical manifestations of Gorlin syndrome?
- complete penetrance
- variable expressivity
multiple basal cell carcinomas:
begins to appear _____
appears on _____ and ____ skin
puberty
sun exposed skin or
not - exposed skin
typical BCC
older
fair skin [white]
TF
at puberty like 19 and appears multiple Basal cell carcinoma
think nevoid basal cell carcinoma
true
multiple odontogenic keratocyst
average age starts age ___
and is not a good differential factor
19
true
OKC
odontogenic keratocyst multiple cysts are common
true
nevoid basal cell carcinoma syndrome have 4 clinical features
- palmar and plantar pits
- skeletal abnormalities:
- calcified falx cerebri
- rib and vertebral anomalies - enlarged head circumference [frontal bossing]
- hypertelomerism - various tumors
hypertolerism
increased distance bt the eyes
TF
OKC you can surgically remove them
they do reoccur
true
true
high grade childhood tumor
happens in small % in gorlin syndrome
highly malignant
Medulloblastoma
TF
Medulloblastoma: closely monitor by MRI before ____ yo
7 yo
true
