Non-syndromic multiple keratocystic odontogenic tumors: An arduous challenge for oral and maxillofacial specialists

Gv Reddy,Garlapati Komali,K Sravan Kumar Reddy,M Haranadha Reddy,Pancha Venkat Bhagirath,Raj Kumar Badam,Rembers Anusha

doi:10.5348/ijcri-201664-cr-10652

Gv Reddy, Garlapati Komali + Show 5 more

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https://doi.org/10.5348/ijcri-201664-cr-10652

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Abstract

Introduction: Keratocystic odontogenic tumor (KOt) is a common developmental odontogenic cyst affecting the maxillofacial region. Multiple odontogenic keratocysts (OKcs) are usually seen in association with nevoid basal cell carcinoma syndrome but approximately only 5% of patients with keratocystic odontogenic tumor have multiple cysts without concomitant syndromic presentation. Only a few cases have been reported till date. case report: this report emphasizes a unique case of a young 14-year-old female suffering since an early age and the role of multidisciplinary approach in the diagnosis and management of a case of multiple keratocystic odontogenic tumors in a non-syndromic patient. conclusion: the non-syndromic KcOts are linked to the expression of a characteristic gene. they are associated with severe morbidity in the younger age group due to their multiple involvement of the jaws but their recurrence rate is less compared to that of syndromic type. the diagnosis and management of these tumors mandates multidisciplinary approach which can instill confidence and improve quality of life of the patients.

Highlights

Keratocystic odontogenic tumor (KOT) is a common developmental odontogenic cyst affecting the maxillofacial region
Received: 09 January 2016 Accepted: 02 March 2016 Published: 01 June 2016 seen in association with nevoid basal cell carcinoma syndrome but approximately only 5% of patients with keratocystic odontogenic tumor have multiple cysts without concomitant syndromic presentation
The KOT is a common developmental odontogenic cyst and its biologic behavior is similar to a benign neoplasm [5]

Summary

INTRODUCTION

Keratocystic odontogenic tumor (KCOT/KOT) is a developmental cyst derived from the enamel organ or dental lamina. A 14-year-old female patient reported with a chief complaint of swelling in the right side of face (Figure 1A) since four months with a history of slowly progressing swelling Her past history revealed that she was a diagnosed and surgically operated case of dentigerous cyst in relation to left deciduous maxillary teeth (C, D) at 6 years of age. At the age of 10 years, she was suspected for re-infection of the cyst with intra oral sinus opening and was advised an orthopantomogram (OPG) which revealed multiple radiolucencies involving maxilla and mandible (Figure 2A) Her personal history revealed mixed diet with no deleterious habits and family history was not contributory and there was no history of consanguineous marriage of her parents.

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