From: Genetics of Skin Cancer (PDQ®)
PDQ Cancer Information Summaries [Internet].
Bethesda (MD): National Cancer Institute (US); 2002-.
NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.
Table 2. Frequency of Nonmalignant Findings in Basal Cell Nevus Syndrome (BCNS)
| Finding | Frequency (%) | Median Age of Onset |
|---|---|---|
| Palmar/plantar pits | 87 | Usually by age 10 y |
| Keratogenic jaw cysts | 74 | Usually by age 20 y |
| Bridged sella | 68 | Congenital |
| Calcification of falx cerebri | 65 | Usually by age 40 y |
| Macrocephaly | 50 | Congenital |
| Hypertelorism | 42 | Congenital |
| Osseous lucencies in the hands | 30 | Congenital |
| Frontal bossing | 27 | Congenital |
| Bifid ribs | 26 | Congenital |
| Calcification of tentorium cerebelli | 20 | Not reported |
| Ovarian fibromas | 17 | 30 y |
| Hemivertebra | 15 | Congenital |
| Pectus deformity | 11 | Congenital |
| Fusion of vertebral bodies | 10 | Congenital |
| Cleft lip/palate | 3 | Congenital |
Adapted from a report by Kimonis et al. [55] about 105 individuals with BCNS seen at the National Institutes of Health between 1985 and 1997.