| • | AKA Gorlin-Goltz syndrome |
| • | numerous BCC’s (rarely invasive) |
| • | jaw cysts (odontogenic keratocysts) |
| • | epithelium lined; the presenting complaint in ~50% of patients |
| • | the syndrome was discovered by a dentist, RJ Gorlin |
| • | appear during the first decade of life and displace the child’s teeth; cause pain, drainage, and jaw swelling |
| • | calcification of the falx cerebri (85-90%) |
| • | general appearance – large head, broad nasal ridge, hypertelorism, frontal bossing |
| • | etiology – mutation in the PATCHED (PTC) gene = tumor suppressor gene |
|