| CASE REPORT |
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| Year : 2017 | Volume
: 1
| Issue : 1 | Page : 26-30 |
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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
Correspondence Address:
Aravinda Konidena
Department of Oral Medicine and Radiology, Swami Devi Dyal Hospital and Dental College, Golpura, Barwala, Panchkula, Haryana
India
 Source of Support: None, Conflict of Interest: None  | Check |
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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