Treatment of nevoid basal cell carcinoma syndrome: a case report
서동욱, 김수관, 오지수, 유재식,
소속 상세정보
서동욱 ( Seo Dong-Uk ) - Chosun University School of Dentistry Department of Oral and Maxillofacial Surgery
김수관 ( Kim Su-Gwan ) - Chosun University School of Dentistry Department of Oral and Maxillofacial Surgery
오지수 ( Oh Ji-Su ) - Chosun University School of Dentistry Department of Oral and Maxillofacial Surgery
유재식 ( You Jae-Seek ) - Chosun University School of Dentistry Department of Oral and Maxillofacial Surgery
KMID : 0355620160420050284
Abstract
Abstract (J Korean Assoc Oral Maxillofac Surg 2016;42:284-287)Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is characterized by various embryological deformities and carcinoma formation. It is caused by PTCHI gene mutations and is autosomal dominantly inherited. Some of the main symptoms of NBCCS are multiple basal cell carcinomas, multiple keratocystic odontogenic tumors (KCOTs) of the mandible, hyperkeratosis of the palmar and plantar, skeletal deformity, calcification of the falx cerebri, and facial defomity. Recurrent KCOT is the main symptom of NBCCS and is present in approximately 90% of patients. In NBCCS, KCOTs typically occur in multiples. KCOTs can be detected in patients under the age of 10, and new and recurring cysts develop until approximately the age of 30. The postoperation recurrence rate is approximately 60%. This case report presents a 14-year-old female patient with a chief complaint of a cyst found in the maxilla and mandible. The patient was diagnosed with NBCCS, and following treatment of marsupialization and enucleation, the clinical results were satisfactory.
키워드
Basal cell nevus syndrome; Jaw cysts
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