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Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series
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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer - Medknow
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7802829/ https://www.ncbi.nlm.nih.gov/pubmed/33456241 http://dx.doi.org/10.4103/jomfp.JOMFP_358_19 |
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author | Saxena, Shikha Sundaragiri, Krishna Sireesha Bhargava, Akshay Sankhla, Bharat |
author_facet | Saxena, Shikha Sundaragiri, Krishna Sireesha Bhargava, Akshay Sankhla, Bharat |
author_sort | Saxena, Shikha |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-7802829 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Wolters Kluwer - Medknow |
record_format | MEDLINE/PubMed |
spelling | pubmed-78028292021-01-15 Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series Saxena, Shikha Sundaragiri, Krishna Sireesha Bhargava, Akshay Sankhla, Bharat J Oral Maxillofac Pathol Case Report 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. Wolters Kluwer - Medknow 2020 2020-09-09 /pmc/articles/PMC7802829/ /pubmed/33456241 http://dx.doi.org/10.4103/jomfp.JOMFP_358_19 Text en Copyright: © 2020 Journal of Oral and Maxillofacial Pathology http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Saxena, Shikha Sundaragiri, Krishna Sireesha Bhargava, Akshay Sankhla, Bharat Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series |
title | Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series |
title_full | Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series |
title_fullStr | Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series |
title_full_unstemmed | Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series |
title_short | Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series |
title_sort | studying the multiple faces of nevoid basal-cell carcinoma syndrome: a case series |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7802829/ https://www.ncbi.nlm.nih.gov/pubmed/33456241 http://dx.doi.org/10.4103/jomfp.JOMFP_358_19 |
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