Imaging Sci Dent.  2011 Dec;41(4):171-175. 10.5624/isd.2011.41.4.171.

Imaging findings in a case of Gorlin-Goltz syndrome: a survey using advanced modalities

• Bronoosh P
• Shakibafar AR
• Houshyar M
• Nafarzade

• KMID: 2167403
• DOI: http://doi.org/10.5624/isd.2011.41.4.171

Abstract

Gorlin-Goltz syndrome is an infrequent multi-systemic disease which is characterized by multiple keratocysts in the jaws, calcification of falx cerebri, and basal cell carcinomas. We report a case of Gorlin-Goltz syndrome in a 23-year-old man with emphasis on image findings of keratocyctic odontogenic tumors (KCOTs) on panoramic radiograph, computed tomography, magnetic resonance (MR) imaging, and Ultrasonography (US). In this case, pericoronal lesions were mostly orthokeratinized odontogenic cyst (OOC) concerning the MR and US study, which tended to recur less. The aim of this report was to clarify the characteristic imaging features of the syndrome-related keratocysts that can be used to differentiate KCOT from OOC. Also, our findings suggested that the recurrence rate of KCOTs might be predicted based on their association to teeth.

Keyword

Gorlin-Goltz syndrome; Tomography, X-Ray Computed; Magnetic Resonance Imaging; Ultrasonography

MeSH Terms

Basal Cell Nevus Syndrome
Carcinoma, Basal Cell
Humans
Jaw
Magnetic Resonance Imaging
Magnetic Resonance Spectroscopy
Odontogenic Cysts
Odontogenic Tumors
Recurrence
Tomography, X-Ray Computed
Tooth
Young Adult

Figure

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  Fig. 1 Panoramic radiograph shows multiple cystic odontogenic lesions in both jaws.

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  Fig. 2 Skull PA radiograph shows falx cerebri calcification.

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  Fig. 3 A. Coronal CT scan shows a cystic lesion associated with an impact tooth causing expansion and displacement of the floor of the right antrum and a mucous retention cyst in the left sinus. B. Axial CT scan shows a lytic lesion in the region of right maxillary canine as well as in ramus both sides. C. Axial CT scan shows soft tissue density in the right maxillary sinus with mucoperiosteal thickening and thinning of medial wall. D. Axial CT scan of the mandible reveals a well defined expansile lytic lesion in the right side crossing midline. E. Coronal CT scan shows a hypodense expansile lesion causing thinning of medial wall of the left mandibular ramus.

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  Fig. 4 Ultrasonograph shows a well demarcated hypoechoic lesions in the right side of mandible.

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  Fig. 5 A. Axial T1WI of mandible shows intermediate signal intensity (SI). B. Coronal T1W MRI of patient shows an expansion of inferior wall of the right maxillary sinus and anterior wall of nasal cavity due to low-intermediate SI lesion associated with an impacted tooth (signal void). C. Coronal post contrast T1W MRI reveals moderate enhancement of cystic wall in the right maxillary sinus. D. Axial T2WI of the mandible shows a homogenous high SI lesion. E. Coronal T2W MRI reveals a well defined expansile hyperintense lesion inferior to the right antrum with heterogeneous signal intensity. F. Axial T2WI of the maxilla shows a hyper intense heterogeneous lesion and a retention pseudocyst.

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  Fig. 6 Histopathologic view of KCOT (H&E stain, ×10). The epithelial lining is 6 to 8 cells thick, with a hyperchromatic and palisaded basal cell layer. Note the corrugated parakeratotic surface.

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