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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Format: | Article |
Language: | English |
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Wolters Kluwer Medknow Publications
2012-01-01
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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. |
first_indexed | 2024-12-11T01:20:25Z |
format | Article |
id | doaj.art-0b41c0135340438387a136c1957ecd82 |
institution | Directory Open Access Journal |
issn | 0976-237X 0976-2361 |
language | English |
last_indexed | 2024-12-11T01:20:25Z |
publishDate | 2012-01-01 |
publisher | Wolters Kluwer Medknow Publications |
record_format | Article |
series | Contemporary Clinical Dentistry |
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 |
work_keys_str_mv | AT nkkiran nevoidbasalcellcarcinomasyndromegorlingoltzsyndrome AT tntilakraj nevoidbasalcellcarcinomasyndromegorlingoltzsyndrome AT ksmukunda nevoidbasalcellcarcinomasyndromegorlingoltzsyndrome AT vrajashekarreddy nevoidbasalcellcarcinomasyndromegorlingoltzsyndrome |