Gorlin syndrome : nevoid basal cell carcinoma
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Transcript of Gorlin syndrome : nevoid basal cell carcinoma
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In the name of God
Gorlin Syndrome(Nevoid basal cell carcinoma)
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EPIDEMIOLOGYDominant Autosomal
Familial Disorder
Mostly in Caucasian
Age:17-35 years old
Equal in Men and Women
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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)
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LESIONS OF BCC
Location: Every where but mostly around eyelid, eye, nose and cheek
Shape: Different(noduloulcerative, pigmented, morphea, …)
Metastasis has reported
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CASE REPORT
Gender: Female
Age: 25 years old
Complication: exofytic and pigmented lesions in neck and upper limb
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SIGN OF PATIENT
ClinicalPearl plaque with specified borderPits of sole and palm
RadiogeraphyCervical and bifurcation ribOKC in mandible
SonographyHepatic hemangioma
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HISTOPATHOLOGY
Atrophic epiderm
Groups of basaloied cell with palisaiding form
Peripheral cleft in inflammatory struma
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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
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The end