Case Report

Year : 2017 | Volume : 1 | Issue : 1 | Page : 26 - 30

Multiple keratocystic odontogenic tumors: Insight to a rare syndrome?

Aravinda Konidena, Samridhi Shekhar, Avani Dixit, Deepa Jatti Patil

Department of Oral Medicine and Radiology, Swami Devi Dyal Hospital and Dental College, Panchkula, Haryana, India

Address for correspondence:

Dr. Aravinda Konidena, Department of Oral Medicine and Radiology, Swami Devi Dyal Hospital and Dental College, Golpura,

Barwala, Panchkula, Haryana, India.

E‐mail: karavinda1@gmail.com

Abstract

Nevoid basal cell carcinoma (BCC) syndrome is a rare syndrome caused by mutations in PTCH1 gene and characterized by several developmental anomalies and neoplasms affecting multiple organ systems. Conventionally, the syndrome was described as a triad of multiple BCCs, keratocystic odontogenic tumors(KCOTs), and bifid ribs. However, recently, we encountered a 25‐year‐old male patient who presented incidentally with multiple KCOTs, bifid ribs, and other characteristic findings, but not BCCs. This case along with appropriate literature review is being presented herewith.

Keywords: Falx cerebri calcification, Gorlin‐Goltz syndrome, keratocystic odontogenic tumor

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

How to cite this article:

Konidena A, Shekhar S, Dixit A, Patil DJ. Multiple keratocystic odontogenic tumors: Insight to a rare syndrome? Int J Clinicopathol Correl 2017;1:26-30.