What is nevoid basal cell carcinoma syndrome?

Updated: Mar 02, 2020
  • Author: Robert S Bader, MD; Chief Editor: William D James, MD  more...
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Answer

In addition to basal cell carcinoma, this autosomal dominant disorder can result in the early formation of multiple odontogenic keratocysts, palmoplantar pitting, intracranial calcification, and rib anomalies. Various tumors such as medulloblastomas, meningioma, fetal rhabdomyoma, and ameloblastoma also can occur. [36]

Odontogenic keratocysts, palmoplantar pitting, intracranial calcification, and rib anomalies may be seen. Mutations in the hedgehog signaling pathway, particularly the patched gene, are causative. [37]

Go to Nevoid Basal Cell Carcinoma Syndrome to see more complete information on this topic.


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