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KMID : 0358920140410010034
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Journal of the Korean Academy of Pedodontics
2014 Volume.41 No. 1 p.34 ~ p.39
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Nevoid Basal Cell Carcinoma Syndrome : A Case Report
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Lee Yoon-Jung
Park Jae-Hong
Choi Sung-Chul
Lee Soo-Eon
Kim Kwang-Chul
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Abstract
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Nevoid basal cell carcinoma syndrome(NBCCS) is a autosomal dominant disorder, and its major manifestations are multiple basal cell carcinoma, keratocystic odontogenic tumor, rib anomalies, palmer and plantar pits, calcification of the falx cerebri. Keratocystic odontogenic tumor(KCOT) is defined as intraosseous tumor of odontogenic origin with a characteristic lining of parakeratinized stratified squamous epithelium and potential aggressive behavior. We report a case of a 3-year-old patient with nevoid basal cell carcinoma syndrome who initially presented with unilocular keratocystic odontogenic tumor in maxillary canine region. Keratocystic odontogenic tumor was treated by enucleation, and periodic follow-up check will be required for early diagnosis of additional diseases related with this syndrome.
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KEYWORD
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Nevoid basal cell carcinoma syndrome, Keratocystic odontogenic tumor, Enucleation
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