Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)

The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple...

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Main Authors: N K Kiran, T N Tilak Raj, K S Mukunda, V Rajashekar Reddy
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2012-01-01
Series:Contemporary Clinical Dentistry
Subjects:
Online Access:http://www.contempclindent.org/article.asp?issn=0976-237X;year=2012;volume=3;issue=4;spage=514;epage=518;aulast=Kiran
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author N K Kiran
T N Tilak Raj
K S Mukunda
V Rajashekar Reddy
author_facet N K Kiran
T N Tilak Raj
K S Mukunda
V Rajashekar Reddy
author_sort N K Kiran
collection DOAJ
description The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.
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spelling doaj.art-0b41c0135340438387a136c1957ecd822022-12-22T01:25:43ZengWolters Kluwer Medknow PublicationsContemporary Clinical Dentistry0976-237X0976-23612012-01-013451451810.4103/0976-237X.107459Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)N K KiranT N Tilak RajK S MukundaV Rajashekar ReddyThe Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.http://www.contempclindent.org/article.asp?issn=0976-237X;year=2012;volume=3;issue=4;spage=514;epage=518;aulast=KiranBifid ribscalcification of falx cerebrinevoid basal cell carcinoma syndromeodontogenic keratocystspalmer and planter pits
spellingShingle N K Kiran
T N Tilak Raj
K S Mukunda
V Rajashekar Reddy
Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)
Contemporary Clinical Dentistry
Bifid ribs
calcification of falx cerebri
nevoid basal cell carcinoma syndrome
odontogenic keratocysts
palmer and planter pits
title Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)
title_full Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)
title_fullStr Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)
title_full_unstemmed Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)
title_short Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)
title_sort nevoid basal cell carcinoma syndrome gorlin goltz syndrome
topic Bifid ribs
calcification of falx cerebri
nevoid basal cell carcinoma syndrome
odontogenic keratocysts
palmer and planter pits
url http://www.contempclindent.org/article.asp?issn=0976-237X;year=2012;volume=3;issue=4;spage=514;epage=518;aulast=Kiran
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AT tntilakraj nevoidbasalcellcarcinomasyndromegorlingoltzsyndrome
AT ksmukunda nevoidbasalcellcarcinomasyndromegorlingoltzsyndrome
AT vrajashekarreddy nevoidbasalcellcarcinomasyndromegorlingoltzsyndrome