AI Article Synopsis
- A case series of 10 individuals with nevoid basal cell carcinoma syndrome (NBCCS) is presented, detailing their symptoms and follow-up over 10 years.
- They exhibited specific features such as calcification of the falx cerebri, mild hypertelorism, and frontal bossing, with varying prevalence of associated tumors.
- The study emphasizes the significance of recognizing NBCCS for effective management and monitoring, particularly in patients with multiple odontogenic keratocyst tumors, due to its autosomal dominant inheritance and cancer risk.
Article Abstract
We present a family case series with 10 individuals having nevoid basal cell carcinoma syndrome (NBCCS) with a 10-year follow-up. All articles published in the literature between 1967 and 2011 on familial Gorlin-Goltz syndrome in any language were surveyed to determine the mapping of cases per country of occurrence of this disease. All patients in the present series were presented with calcification of the falx cerebri, mild hypertelorism, and frontal bossing. Odontogenic keratocystic tumors, palmar and plantar pits, and multiple basal cell carcinomas occurred in 90, 40, and 20%, respectively, of the patients. One of the patients died of skin cancer. Diagnosis of odontogenic keratocyst tumors was confirmed by histopathological examination. NBCCS is a rare autosomal dominant cancer predisposition syndrome; it is important to recognize it when a patient has multiple odontogenic keratocyst tumors because life-long monitoring is essential for patient management.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4755777 | PMC |
| http://dx.doi.org/10.1055/s-0035-1558454 | DOI Listing |
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