Cargando…
Basal cell nevus syndrome with excessive basal cell carcinomas
Basal cell nevus syndrome (BCNS), also known as basal cell carcinoma nevus syndrome, Gorlin syndrome, Gorlin-Goltz syndrome, and nevoid basal cell carcinoma, is a rare autosomal dominant disorder with a prevalence of approximately 1/60,000. A lower prevalence rate of 1/13,939,393 has also been repor...
Autores principales: | , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Korean Cleft Palate-Craniofacial Association
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8107455/ https://www.ncbi.nlm.nih.gov/pubmed/33957740 http://dx.doi.org/10.7181/acfs.2021.00136 |
_version_ | 1783689954730180608 |
---|---|
author | Kim, Choon Soo Na, Young Cheon |
author_facet | Kim, Choon Soo Na, Young Cheon |
author_sort | Kim, Choon Soo |
collection | PubMed |
description | Basal cell nevus syndrome (BCNS), also known as basal cell carcinoma nevus syndrome, Gorlin syndrome, Gorlin-Goltz syndrome, and nevoid basal cell carcinoma, is a rare autosomal dominant disorder with a prevalence of approximately 1/60,000. A lower prevalence rate of 1/13,939,393 has also been reported in Korea. We report the case of a 40-year-old male patient with multiple black pigmented macules on the face that first appeared when he was a teenager. His clinical features of jaw cysts, bifid ribs, and calcification of the falx cerebri were fitting within the criteria for the diagnosis of BCNS. We excised all suspected macules and sent permanent biopsy. Most of the histological examinations of the biopsy samples taken during surgical excision of the face masses showed basal cell carcinomas. Ten months after the surgery, the patient has remained free from symptoms and is undergoing follow-up observation. |
format | Online Article Text |
id | pubmed-8107455 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Korean Cleft Palate-Craniofacial Association |
record_format | MEDLINE/PubMed |
spelling | pubmed-81074552021-05-19 Basal cell nevus syndrome with excessive basal cell carcinomas Kim, Choon Soo Na, Young Cheon Arch Craniofac Surg Case Report Basal cell nevus syndrome (BCNS), also known as basal cell carcinoma nevus syndrome, Gorlin syndrome, Gorlin-Goltz syndrome, and nevoid basal cell carcinoma, is a rare autosomal dominant disorder with a prevalence of approximately 1/60,000. A lower prevalence rate of 1/13,939,393 has also been reported in Korea. We report the case of a 40-year-old male patient with multiple black pigmented macules on the face that first appeared when he was a teenager. His clinical features of jaw cysts, bifid ribs, and calcification of the falx cerebri were fitting within the criteria for the diagnosis of BCNS. We excised all suspected macules and sent permanent biopsy. Most of the histological examinations of the biopsy samples taken during surgical excision of the face masses showed basal cell carcinomas. Ten months after the surgery, the patient has remained free from symptoms and is undergoing follow-up observation. Korean Cleft Palate-Craniofacial Association 2021-04 2021-04-20 /pmc/articles/PMC8107455/ /pubmed/33957740 http://dx.doi.org/10.7181/acfs.2021.00136 Text en Copyright © 2021 The Korean Cleft Palate-Craniofacial Association https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Kim, Choon Soo Na, Young Cheon Basal cell nevus syndrome with excessive basal cell carcinomas |
title | Basal cell nevus syndrome with excessive basal cell carcinomas |
title_full | Basal cell nevus syndrome with excessive basal cell carcinomas |
title_fullStr | Basal cell nevus syndrome with excessive basal cell carcinomas |
title_full_unstemmed | Basal cell nevus syndrome with excessive basal cell carcinomas |
title_short | Basal cell nevus syndrome with excessive basal cell carcinomas |
title_sort | basal cell nevus syndrome with excessive basal cell carcinomas |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8107455/ https://www.ncbi.nlm.nih.gov/pubmed/33957740 http://dx.doi.org/10.7181/acfs.2021.00136 |
work_keys_str_mv | AT kimchoonsoo basalcellnevussyndromewithexcessivebasalcellcarcinomas AT nayoungcheon basalcellnevussyndromewithexcessivebasalcellcarcinomas |