Multiple keratocystic odontogenic tumors: Insight to a rare syndrome?

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

Multiple keratocystic odontogenic tumors: Insight to a rare syndrome?

Authors

Aravinda Konidena Department of Oral Medicine and Radiology, Swami Devi Dyal Hospital and Dental College, Panchkula, Haryana, India
Samridhi Shekhar Department of Oral Medicine and Radiology, Swami Devi Dyal Hospital and Dental College, Panchkula, Haryana, India
Avani Dixit Department of Oral Medicine and Radiology, Swami Devi Dyal Hospital and Dental College, Panchkula, Haryana, India
Deepa Jatti Patil Department of Oral Medicine and Radiology, Swami Devi Dyal Hospital and Dental College, Panchkula, Haryana, India

Keywords:

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

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.

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Published

2022-08-18

How to Cite

Aravinda Konidena, Samridhi Shekhar, Avani Dixit, & Deepa Jatti Patil. (2022). Multiple keratocystic odontogenic tumors: Insight to a rare syndrome?. International Journal of Clinicopathological Correlation, 1(1), 26–30. Retrieved from https://editorialmanager.in/index.php/ijcpc/article/view/383