ONLINE ONLY ARTICLES - CASE REPORT
Year : 2020 | Volume
: 24 | Issue : 3 | Page : 591--592
Gorlin syndrome: A rare case report
Sandhya Shrivastava1, Sushruth Nayak2, Prachi Nayak2, Sourabh Sahu3
1 Department of Oral Pathology and Microbiology, New Horizon Dental College and Research Institute, Bilaspur, Chhattisgarh, India
2 Department of Oral Pathology and Microbiology, M.M. College of Dental Sciences and Research, M.M (Deemed to be University), Mullana (Ambala), Haryana, India
3 Department of Oral Surgery, Government Dental College, Raipur, Chhattisgarh, India
Correspondence Address:
Sushruth Nayak
Department of Oral Pathology and Microbiology, M.M. College of Dental Sciences and Research, M.M (Deemed to be University), Mullana (Ambala) - 133 207, Haryana
India
Gorlin syndrome is an autosomal dominant inherited condition that exhibits high penetrance and variable expressivity. The syndrome is caused by mutations in PTCH, a tumor suppressor gene that has been mapped to chromosome 9q22.3-q31. It is observed that most aggressive forms of keratocystic odontogenic tumor are the first signs of this syndrome which show a high recurrence rate due to its varied histopathological features. The other components are multiple basal cell carcinomas of the skin, intracranial calcifications and rib and vertebral anomalies. In addition, >100 minor criteria have been described with two major and one minor criteria or one major and three minor criteria necessary for confirmatory diagnosis. It is a rare syndrome having an estimated incidence of 1 in 50,000–150,000 in the general population with a 3:1 male/female gender predilection. Here, we report the case of a 14-year-old female patient diagnosed with Gorlin syndrome.
How to cite this article:
Shrivastava S, Nayak S, Nayak P, Sahu S. Gorlin syndrome: A rare case report.J Oral Maxillofac Pathol 2020;24:591-592
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How to cite this URL:
Shrivastava S, Nayak S, Nayak P, Sahu S. Gorlin syndrome: A rare case report. J Oral Maxillofac Pathol [serial online] 2020 [cited 2021 Feb 28 ];24:591-592
Available from: https://www.jomfp.in/article.asp?issn=0973-029X;year=2020;volume=24;issue=3;spage=591;epage=592;aulast=Shrivastava;type=0 |
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