Treatment of Keratocystic Odontogenic Tumors in Nevoid Basal Cell Carcinoma Syndrome
류현호, 신동윤, 류석환, Kim Taek-Woo, 이종범, 김복주, 김철훈,
소속 상세정보
류현호 ( Ryu Hyun-Ho ) - Dong-A University Medical Center Department of Oral and Maxillofacial Surgery
신동윤 ( Shin Dong-Yoon ) - Dong-A University Medical Center Department of Oral and Maxillofacial Surgery
류석환 ( Ryu Seok-Hwan ) - Dong-A University Medical Center Department of Oral and Maxillofacial Surgery
( Kim Taek-Woo ) - Dong-A University Medical Center Department of Oral and Maxillofacial Surgery
이종범 ( Lee Jong-Bum ) - Dong-A University Medical Center Department of Oral and Maxillofacial Surgery
김복주 ( Kim Bok-Joo ) - Dong-A University School of Medicine Department of Oral and Maxillofacial Surgery
김철훈 ( Kim Chul-Hoon ) - Dong-A University Medical Center Department of Oral and Maxillofacial Surgery
KMID : 1036320140360010007
Abstract
Nevoid basal cell carcinoma syndrome (NBCCS) is inherited as an autosomal dominant trait with variable conditions, including multiple basal cell carcinoma, numerous keratocystic odontogenic tumors (KOTs) in the jaws, ectopic calcification of the falx cerebri, bifid ribs, macrocephaly, kyphoscoliosis, cleft palate, frontal and temporal bossing, mild ocular hypertelorism, mild mandibular prognathism, vertebral fusion, and so on. A 16-year-old boy visited the Dong-A University Medical Center, requiring diagnosis and treatment of multiple cystic lesions. He presented with many conditions related to NBCCS, including multiple KOTs, bifid rib, cleft lip, frontal bossing, mild ocular hypertelorism, and mild mandibular prognathism. No characteristic cutaneous manifestations (nevoid basal cell carcinoma) were observed in this patient. We report on a case of multiple KOTs associated with NBCCS with a review of the literature.
키워드
Basal cell nevus syndrome; Multiple keratocystic odontogenic tumors; Multiple odontogenic keratocysts
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