Gorlin syndrome : nevoid basal cell carcinoma

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Text of Gorlin syndrome : nevoid basal cell carcinoma

In the name of God

In the name of GodGorlin 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 bccLocation: Every where but mostly around eyelid, eye, nose and cheek

Shape: Different(noduloulcerative, pigmented, morphea, )

Metastasis has reported

Case reportGender: Female

Age: 25 years old

Complication: exofytic and pigmented lesions in neck and upper limb

sign of patientClinicalPearl plaque with specified borderPits of sole and palm

RadiogeraphyCervical and bifurcation ribOKC in mandible

SonographyHepatic hemangioma

HistopathologyAtrophic epiderm

Groups of basaloied cell with palisaiding form

Peripheral cleft in inflammatory struma

diagnosisMajor: 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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