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 mi...

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Bibliographic Details
Published in:Auris, nasus, larynx nasus, larynx, 2003-12, Vol.30 (4), p.421-423
Main Authors: Tabuchi, Keiji, Okubo, Hideki, Takahashi, Kazuhiko, Uemaetomari, Isao, Shimoyamada, Hiroaki, Nakayama, Yoshio, Hara, Akira
Format: Article
Language:English
Subjects:
Aged
Basal cell carcinoma
Basal Cell Nevus Syndrome - genetics
Basal Cell Nevus Syndrome - pathology
Ethmoid sinus
Ethmoid Sinus - pathology
Eyelid Neoplasms - genetics
Eyelid Neoplasms - pathology
Female
Humans
Magnetic Resonance Imaging
Neoplasm Invasiveness
Nevoid basal cell carcinoma (Gorlin–Goltz) syndrome
Orbital Neoplasms - genetics
Orbital Neoplasms - pathology
Paranasal Sinus Neoplasms - genetics
Paranasal Sinus Neoplasms - pathology
Pedigree
Tomography, X-Ray Computed
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Summary: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.
ISSN:0385-8146
1879-1476
DOI:10.1016/S0385-8146(03)00092-0