Gorlin syndrome : nevoid basal cell carcinoma

pathology

1. In the name of God Gorlin Syndrome (Nevoid basal cell carcinoma)

2. Epidemiology Dominant Autosomal Familial Disorder Mostly in Caucasian Age:17-35 years old Equal in Men and Women

3. sign Frontal Bossing in 54% Flat Nose Hypertelorism in 42% Multiple jaw cyst(OKC) in 74% - Until 20 years old Multiple pits in palm and sole in 87% - in second decade of life Macrocephali in 50% Vertebra and ribs anomalis (bifurcation of ribs and kyphoscoliosis ) CNS disorder( falx cerebri calcification)

4. Lesions of bcc Location: Every where but mostly around eyelid, eye, nose and cheek Shape: Different( noduloulcerative , pigmented, morphea , …) Metastasis has reported

5. Case report Gender: Female Age: 25 years old Complication: exofytic and pigmented lesions in neck and upper limb

6. sign of patient Clinical Pearl plaque with specified border Pits of sole and palm Radiogeraphy Cervical and bifurcation rib OKC in mandible Sonography Hepatic hemangioma

7. Histopathology Atrophic epiderm Groups of basaloied cell with palisaiding form Peripheral cleft in inflammatory struma

8. 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

9. The end