Nevoid basal cell carcinoma syndrome: report of an aggressive case with ethmoid sinus invasion.
Nevoid basal cell carcinoma syndrome, also referred to as Gorlin-Goltz syndrome, is a rare autosomal dominant disorder characterized by multiple basal cell carcinomas, jaw cysts, palmar or plantar pits, ectopic calcification of the falx cerebri, and various skeletal developmental abnormalities. A minority of basal cell carcinomas demonstrate aggressive behavior and involve the craniofacial bones in nevoid basal cell carcinoma syndrome. A non-familial case of nevoid basal cell carcinoma syndrome with a basal cell carcinoma of the eyelid invading to the ethmoid sinus is reported.
Tabuchi
Keiji
K
Department of Otolaryngology, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennodai, 305-8575, Tsukuba, Japan.
Okubo
Hideki
H
Takahashi
Kazuhiko
K
Uemaetomari
Isao
I
Shimoyamada
Hiroaki
H
Nakayama
Yoshio
Y
Hara
Akira
A
D000368:Aged; D001478:Basal Cell Nevus Syndrome / Q000235:genetics / Q000473:pathology*; D005005:Ethmoid Sinus / Q000473:pathology*; D005142:Eyelid Neoplasms / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009361:Neoplasm Invasiveness; D009918:Orbital Neoplasms / Q000235:genetics / Q000473:pathology; D010255:Paranasal Sinus Neoplasms / Q000235:genetics / Q000473:pathology*; D010375:Pedigree; D014057:Tomography, X-Ray Computed