Imaging modality correlations of an odontogenic keratocyst in the nevoid basal cell carcinoma syndrome: A family case report

Multiple maxillary and mandibular cysts are principle features of nevoid basal cell carcinoma syndrome (NBCCS; Gorlin-Goltz syndrome). We present a family case report of NBCCS with odontogenic keratocyst where the findings on plain films, CT, clinical, and histopathologic examinations are compared a...

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Published in:Oral surgery, oral medicine, oral pathology, oral radiology and endodontics oral medicine, oral pathology, oral radiology and endodontics, 2004-08, Vol.98 (2), p.232-236
Main Authors: Melo, Edielle Sant' Anna, Kawamura, Juliana Yuri, Alves, Carlos Augusto Ferreira, Nunes, Fabio Daumas, Jorge, Waldyr Antonio, Cavalcanti, Marcelo Gusmão Paraiso
Format: Article
Language:English
Subjects:
Adolescent
Basal Cell Nevus Syndrome - diagnostic imaging
Basal Cell Nevus Syndrome - genetics
Biological and medical sciences
Dentistry
Dermatology
Female
Humans
Male
Mandibular Diseases - diagnostic imaging
Mandibular Diseases - genetics
Maxillary Diseases - diagnostic imaging
Maxillary Diseases - genetics
Medical sciences
Middle Aged
Odontogenic Cysts - diagnostic imaging
Odontogenic Cysts - genetics
Otorhinolaryngology. Stomatology
Radiography, Panoramic
Tomography, X-Ray Computed
Tumors of the skin and soft tissue. Premalignant lesions
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Summary:Multiple maxillary and mandibular cysts are principle features of nevoid basal cell carcinoma syndrome (NBCCS; Gorlin-Goltz syndrome). We present a family case report of NBCCS with odontogenic keratocyst where the findings on plain films, CT, clinical, and histopathologic examinations are compared and analyzed. The systemic manifestations included frontal bossing, odontogenic keratocyst, ectopic calcification in 1 patient, and bifid rib in 1 patient. CT examination displayed aspects of bone morphology not visible on the plain films. Odontogenic keratocyst diagnosis was confirmed by histopathological examination. The features identified by these combined clinical, imaging, and histologic findings are helpful in identifying an NBCCS patient, distinguishing keratocyst from others cysts or neoplasic lesions, and can therefore influence surgical management. NBCCS is a rare autosomal dominant cancer predisposition syndrome, which is important to recognize when a patient has multiple odontogenic keratocysts, because lifelong monitoring is essential for patient management.
ISSN:1079-2104
1528-395X
DOI:10.1016/j.tripleo.2004.02.055