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

Multiple keratocystic odontogenic tumors: Insight to a rare syndrome?


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

Correspondence Address:
Aravinda Konidena
Department of Oral Medicine and Radiology, Swami Devi Dyal Hospital and Dental College, Golpura, Barwala, Panchkula, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijcpc.ijcpc_4_17

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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.


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