Treatment of nevoid basal cell carcinoma syndrome: a case report.
10.5125/jkaoms.2016.42.5.284
- Author:
Dong Uk SEO
1
;
Su Gwan KIM
;
Ji Su OH
;
Jae Seek YOU
Author Information
1. Department of Oral and Maxillofacial Surgery, School of Dentistry, Chosun University, Gwangju, Korea. sgckim@chosun.ac.kr
- Publication Type:Case Report
- Keywords:
Basal cell nevus syndrome;
Jaw cysts
- MeSH:
Adolescent;
Basal Cell Nevus Syndrome*;
Carcinoma, Basal Cell;
Congenital Abnormalities;
Female;
Humans;
Jaw Cysts;
Mandible;
Maxilla;
Odontogenic Cysts;
Odontogenic Tumors;
Recurrence;
Spinal Cord
- From:Journal of the Korean Association of Oral and Maxillofacial Surgeons
2016;42(5):284-287
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
