J Korean Assoc Maxillofac Plast Reconstr Surg.  2014 Jan;36(1):7-12.

Treatment of Keratocystic Odontogenic Tumors in Nevoid Basal Cell Carcinoma Syndrome

• Ryu HH
• Shin DY
• Ryu SH
• Kim TW
• Lee JB
• Kim BJ
• Kim CH

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.

Keyword

Basal cell nevus syndrome; Multiple keratocystic odontogenic tumors; Multiple odontogenic keratocysts

MeSH Terms

Academic Medical Centers
Adolescent
Basal Cell Nevus Syndrome*
Carcinoma, Basal Cell
Cleft Lip
Cleft Palate
Diagnosis
Humans
Hypertelorism
Jaw
Macrocephaly
Male
Odontogenic Cysts*
Odontogenic Tumors*
Prognathism
Ribs