Open AccessMultiple keratocystic odontogenic tumors in nevoid basal cell carcinoma syndrome
Author(s) -
Treville Pereira,
Avinash Tamgadge,
Swati Sapdhare,
Ashwini Pujar
Publication year - 2015
Publication title -
annals of maxillofacial surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.365
H-Index - 6
ISSN - 2231-0746
DOI - 10.4103/2231-0746.175770
Subject(s) - nevoid basal cell carcinoma syndrome , keratocystic odontogenic tumor , medicine , basal cell nevus syndrome , keratocyst , odontogenic , pathology , dermatology , basal cell carcinoma , basal (medicine) , basal cell , insulin
Keratocystic odontogenic tumor (KCOT) is of particular interest because its recurrence rate is high and its behavior is aggressive. Nevoid basal cell carcinoma syndrome (NBCCS), which is also known as Gorlin syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and with a predisposition to neoplasms. These multiple KCOTs have warranted an aggressive treatment at the earliest because of the damage and possible complications. Recurrence of these lesions is a characteristic feature that has to be considered while explaining the prognosis to the patient. Here, we report a case of a 14-year-old boy with clinical features of basal cell nevus syndrome and multiple KCOTs. In addition to the other common features, congenitally missing third molars in all the four quadrants is a feature which has not been previously reported in association with NBCCS in Indian patients.