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CASE REPORT
Year : 2018  |  Volume : 30  |  Issue : 2  |  Page : 177-180

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


1 Department of Oral Medicine and Radiology, Bharati Vidyapeeth Deemed University Dental College and Hospital, Pune, Maharashtra, India
2 Department of Oral and Maxillofacial Surgery, Sinhagad Dental College, Pune, Maharashtra, India
3 Department of Oral and Maxillofacial Surgery, RKDF Dental College, Bhopal, Madhya Pradesh, India
4 Department of Oral and Maxillofacial Surgery, Bharati Vidyapeeth Deemed University Dental College and Hospital, Pune, Maharashtra, India

Correspondence Address:
Dr. Manjula Hebbale
Department of Oral Medicine and Radiology, Bharati Vidyapeeth Deemed University Dental College and Hospital, Pune - 411 043, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_51_18

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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.


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