Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome): A Rare Late-Onset Case Report

Nevoid basal cell carcinoma syndrome (NBCCS), or Gorlin syndrome, is a hereditary disorder characterized by multiple basal cell carcinomas (BCCs) and systemic abnormalities. We report a 63-year-old male with a history of multifocal BCCs beginning approximately 20 years ago, who underwent staged wide excisions and reconstructions. Computed tomography revealed lamellar calcification of the falx cerebri and tentorium cerebelli, fulfilling a major diagnostic criterion. Despite the absence of palmar pits and skeletal anomalies, a clinical diagnosis of NBCCS was established based on two fulfilled major criteria: multiple BCCs and falx calcification. Molecular genetic testing for PTCH1 or SUFU variants was not performed; this absence of genotypic confirmation is acknowledged as a limitation. This case illustrates that NBCCS may remain undiagnosed until late adulthood when phenotypic expression is incomplete, and that incidental intracranial calcification on routine imaging can serve as the critical diagnostic bridge. Systematic application of the Kimonis criteria and long-term multidisciplinary surveillance are essential in such patients.