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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
CASE REPORT Table of Contents   
Year : 2020  |  Volume : 24  |  Issue : 2  |  Page : 315-321
Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series


Department of Oral Pathology and Microbiology, RUHS College of Dental Sciences (Government Dental College and Hospital), Jaipur, Rajasthan, India

Correspondence Address:
Shikha Saxena
Department of Oral Pathology and Microbiology, RUHS College of Dental Sciences (Government Dental College and Hospital), Jaipur - 302 015, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jomfp.JOMFP_358_19

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Nevoid basal-cell carcinoma syndrome (NBCCS) or Gorlin–Goltz syndrome is an autosomal dominant-inherited condition that exhibits high penetrance and variable expressivity; however, this disorder can arise spontaneously. In 1960, Gorlin and Goltz described the syndrome as a condition, comprising the principle triad of multiple basal cell carcinoma, odontogenic keratocysts, and skeletal anomalies. The diagnostic findings of NBCCS in four patients were studied and compared with other reports in the Indian population. Early diagnosis of this syndrome is important for counseling of patients to prevent the life-long complications of this syndrome which includes the malignancy, oro-maxillofacial deformation, and destruction; this may be reduced if the diagnosis and treatment are made feasible at the earliest.


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Journal of Oral and Maxillofacial Pathology | Published by Wolters Kluwer - Medknow
Online since 15th Aug, 2007