Journal of Oral and Maxillofacial Pathology

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


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