In the name of God
Gorlin Syndrome(Nevoid basal cell carcinoma)
EPIDEMIOLOGYDominant Autosomal
Familial Disorder
Mostly in Caucasian
Age:17-35 years old
Equal in Men and Women
SIGNFrontal Bossing in 54%Flat NoseHypertelorism in 42%Multiple jaw cyst(OKC) in 74% - Until 20 years oldMultiple pits in palm and sole in 87% - in second decade of
lifeMacrocephali in 50%Vertebra and ribs anomalis(bifurcation of ribs and
kyphoscoliosis)CNS disorder(falx cerebri calcification)
LESIONS OF BCC
Location: Every where but mostly around eyelid, eye, nose and cheek
Shape: Different(noduloulcerative, pigmented, morphea, …)
Metastasis has reported
CASE REPORT
Gender: Female
Age: 25 years old
Complication: exofytic and pigmented lesions in neck and upper limb
SIGN OF PATIENT
ClinicalPearl plaque with specified borderPits of sole and palm
RadiogeraphyCervical and bifurcation ribOKC in mandible
SonographyHepatic hemangioma
HISTOPATHOLOGY
Atrophic epiderm
Groups of basaloied cell with palisaiding form
Peripheral cleft in inflammatory struma
DIAGNOSIS
Major: multiple BCC until 30 years old – multiple OKC or polycystic cyst – 3 or more than it pits in sole and palm – Falx calcification and familial history of BCC syndrome
Minor: womb and heart fibroma – Medulobelastoma – lymphomesanteric cyst _ skeletal anomalis – congenital dis order like cleft lip and palate
Positive if : 2 major or 1 major with 2 minor
This patient had 3 major and 1 minor cereiteria
The end