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Basal Cell Nevus Syndrome
Aka: Basal Cell Nevus Syndrome, Gorlin's Syndrome
- See Also
- Nonmelanoma Skin Cancer
- Basal Cell Carcinoma
- Epidemiology
- Prevalence: 1 per 60,000
- Autosomal Dominant inheritance
- Gene localized to Chromosome 9q22.1-q31
- Signs: Major features
- Multiple Nevoid Basal Cell Cancers
- Odontogenic keratocysts (jaw cysts)
- First sign of syndrome (occurs in early childhood)
- Cysts lined with keratinized epithelium
- Originate in dental lamina
- Locally erode to teeth if not completely excised
- Calcified falx cerebri
- Pitting of plantar or palmar surface
- Congenital skeletal abnormalities
- Hypertelorism
- High Scapulae (Sprengel's Deformity)
- Frontal bossing (may also involve parietal area)
- Synostosis
- Bifid ribs
- Associated conditions
- Cleft Lip and Palate
- Neoplasms
- Medulloblastoma
- Meningioma
- Myocardial fibroma
- Lyomesenteric cyst
- Ovarian fibroma
- Rhabdomyoma
- Eye disorders
- Coloboma
- Cataracts
- Glaucoma
- Radiology
- Skull XRay: Calcified falx cerebri
- Chest XRay: Rib asymmetry or bifid ribs
- Panoramic films (Panorex): Dental cysts
- Obtain films annually
- Pelvic Ultrasound: Ovarian Cysts or fibromas
- Brain MRI: Meningiomas or Medulloblastoma
- Prevention: Modifiable risk factor reduction
- See Sun Exposure (lists general preventive measures)
- See Sunscreen
- References
- Bitar (2002) Am Fam Physician 65(12):2501-4 [PubMed]
- Stulberg (2004) Am Fam Physician 70:1481-8 [PubMed]