Early Diagnosis and Intervention in Familial Nevoid Basal Cell Carcinoma Syndrome: A Case Report of Generational Surveillance

Introduction Familial Nevoid Basal Cell Carcinoma Syndrome (FNBCC), also known as Gorlin syndrome. The syndrome, delineated by Gorlin and Goltz in 1960 was first reported in 1894(1), is a rare autosomal dominant genetic disorder characterized by a heterogeneous array of clinical manifestations affecting multiple organ systems. Gorlin and Goltz defined the condition as a syndrome comprising the principal triad of multiple basal cell nevi, jaw keratocystic odontogenic tumors, and skeletal anomalies (2)

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Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2025-02-28
DOI
https://doi.org/10.5281/zenodo.15209387
Primary Topic
Hedgehog Signaling Pathway Studies
Type
article
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article

Early Diagnosis and Intervention in Familial Nevoid Basal Cell Carcinoma Syndrome: A Case Report of Generational Surveillance

Zenodo (CERN European Organization for Nuclear Research)
Hedgehog Signaling Pathway Studies
article

Early Diagnosis and Intervention in Familial Nevoid Basal Cell Carcinoma Syndrome: A Case Report of Generational Surveillance

article en

Abstract

Introduction Familial Nevoid Basal Cell Carcinoma Syndrome (FNBCC), also known as Gorlin syndrome. The syndrome, delineated by Gorlin and Goltz in 1960 was first reported in 1894(1), is a rare autosomal dominant genetic disorder characterized by a heterogeneous array of clinical manifestations affecting multiple organ systems. Gorlin and Goltz defined the condition as a syndrome comprising the principal triad of multiple basal cell nevi, jaw keratocystic odontogenic tumors, and skeletal anomalies (2)

Zenodo (CERN European Organization for Nuclear Research)
International Association for Dental Research (US)
Good health and well-being
Openalex Percentile: Top 60%
Hedgehog Signaling Pathway Studies
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Early Diagnosis and Intervention in Familial Nevoid Basal Cell Carcinoma Syndrome: A Case Report of Generational Surveillance · Zenodo (CERN European Organization for Nuclear Research) (2025) | TGRS Research Map | TGRS