Satellite, or daughter, cysts, showing budding off the main cyst (figure 3), are usually seen in patients with NBCCS
, and their detection should prompt a clinical evaluation for NBCCS
NBCCS is also known as Gorlin-Goltz syndrome, Gorlin's syndrome, and basal cell nevus syndrome.
Patients with NBCCS often have frontal bossing and a broad nasal bridge.
8,9) Other neoplasms found less commonly in NBCCS include medulloblastoma, meningioma, cardiac fibroma, and ovarian fibroma.
A review of the literature found five reported cases of FR and NBCCS.
Our patient with NBCCS is the first reported to have an FR of the tongue.
The manifestations of NBCCS include multiple basal cell carcinomas, odontogenic keratocysts, palmar and plantar pitting, dural calcifications, craniofacial anomalies, medulloblastomas and other intracranial tumors, ovarian fibromas, and various degrees of mental retardation.
In this article, we describe the imaging findings in a case of NBCCS.
The clinical suspicion of NBCCS was high because of the documented presence of this syndrome in his family; his mother, grandmother, and three uncles all had a history of NBCCS.
A plain-film skeletal survey--with emphasis on the scapulae, ribs, hands, and lumbar spine--was performed to search for the bony lesions characteristic of NBCCS.
6-8) The findings in out patient were in accord with previous radiographic studies of NBCCS.
The imaging findings in the mandible and maxilla were consistent with odontogenic keratocysts, which are the characteristic lesions of NBCCS.