Transcript
Page 1: Gorlin syndrome : nevoid basal cell carcinoma

In the name of God

Gorlin Syndrome(Nevoid basal cell carcinoma)

Page 2: Gorlin syndrome : nevoid basal cell carcinoma

EPIDEMIOLOGYDominant Autosomal

Familial Disorder

Mostly in Caucasian

Age:17-35 years old

Equal in Men and Women

Page 3: Gorlin syndrome : nevoid basal cell carcinoma

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)

Page 4: Gorlin syndrome : nevoid basal cell carcinoma

LESIONS OF BCC

Location: Every where but mostly around eyelid, eye, nose and cheek

Shape: Different(noduloulcerative, pigmented, morphea, …)

Metastasis has reported

Page 5: Gorlin syndrome : nevoid basal cell carcinoma

CASE REPORT

Gender: Female

Age: 25 years old

Complication: exofytic and pigmented lesions in neck and upper limb

Page 6: Gorlin syndrome : nevoid basal cell carcinoma

SIGN OF PATIENT

ClinicalPearl plaque with specified borderPits of sole and palm

RadiogeraphyCervical and bifurcation ribOKC in mandible

SonographyHepatic hemangioma

Page 7: Gorlin syndrome : nevoid basal cell carcinoma

HISTOPATHOLOGY

Atrophic epiderm

Groups of basaloied cell with palisaiding form

Peripheral cleft in inflammatory struma

Page 8: Gorlin syndrome : nevoid basal cell carcinoma

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

Page 9: Gorlin syndrome : nevoid basal cell carcinoma

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