Nevoid basal cell carcinoma syndrome: A case report with literature review

• Main Authors: Manjula Hebbale, Pushkar Gawande, Anisha Maria, Rajsekhar Halli, Versha R Giroh
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2018-01-01
• Series: Journal of Indian Academy of Oral Medicine and Radiology
• Subjects: Basal cell carcinoma; basal cell nevi; Gorlin–Goltz syndrome; multiple jaw cysts
• Online Access: http://www.jiaomr.in/article.asp?issn=0972-1363;year=2018;volume=30;issue=2;spage=177;epage=180;aulast=Hebbale

Nevoid basal cell carcinoma or Gorlin–Goltz syndrome is an autosomal dominant inherited disorder that results in numerous basal cell carcinomas (BCC) as well as a number of other facial and skeletal findings. The estimated prevalence varies from 1/57000 to 1/256000 with a male-to-female ratio of 1:1. Main clinical manifestations include multiple BCCs, odontogenic keratocysts of jaws, intracranial ectopic calcifications, and skeletal abnormalities affecting the ribs, vertebra, and skull. Management requires a multidisciplinary approach, with surgical removal of odontogenic keratocysts; surgery for BCCs should be indicated when the number of such lesions is limited. We report a case that was managed surgically, with a follow-up of more than 2 years having no signs of recurrence and relevant literature review.