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Case 122761

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Referral category

Disease / syndrome / tumor / condition

Region of interest

Mandible and mandible

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1 Files Info

Canaray 122761

Multiple odontogenic keratocysts in the mandible: possible Gorlin-Goltz syndrome

There are at least three separate cystic lesions or cystic tumours located in the left mandibular ramus, mandibular anterior/left premolar region, and right premolar area. These lesions are likely keratocystic odontogenic tumors associated with the nevoid basal cell carcinoma or Gorlin-Goltz syndrome. The radiographic features which support this diagnosis include the significant extension versus expansion of the cystic entities and scalloping borders of these lesions as well as displacement of the adjacent teeth. There is no evidence of cystic lesions in the maxilla. Further referral of the patient to a specialist for genetic test and follow up are warranted.

Axial views of the mandible

These axial cross-sectional images demonstrate the minimal expansion of the mandibular cortices compared to the extension of the cystic lesion within the alveolar bone as well as intact buccal and lingual cortices of the mandibular body.

Coronal views of the mandible

These coronal cross-sectional images demonstrate the intact mandibular cortical plates on both sides. Note the mild expansion of the cystic lesion in the mandibular body. The cystic lesion in the left mandibular ramus also causes mild cortical expansion. No cortical perforation is noted. The left mandibular nerve canal has been inferiority displaced at the posterior left mandible, but the borders of the canal appear intact.

Mesiodistal views of the left mandibular body and ramus

These mesiodistal cross-sectional images demonstrate the cystic lesion associated with teeth 38 and 37 as well as second cystic lesion in the left premolar area and anterior mandible. Note the inferior displacement of the mandibular nerve canal at the posterior left mandible by the cystic lesion associated with teeth 37 and 38.

Cystic lesion associated with tooth 44

A cystic lesion is present at lingual aspect of the crown of tooth 44. The tooth is distobuccally displaced. This is most likely a separate small keratocystic odontgenic tumour or an extension of a loculation of the larger cyst at the anterior mandible. The alveolar crest overlying the cyst is partially dehiscent mesial to tooth 43. No root resorption is evident on the adjacent teeth, but tooth 43 is rotated and the lamina dura of the distal surface of the root of the tooth has been lost.

Cystic lesion in the left mandbular ramus

Another cystic lesion is present at the left mandibular ramus. This cyst displaces the developing tooth 38 superiorly towards the coronoid notch. The border of the cystic lesion is continuous with the follicle of tooth 37. Mild expansion is present at the medial and lateral cortices of the ramus, but the cortices appear intact.

Cystic lesion in the mandibular body

A larger cystic lesion is present in the left mandibular body and the anterior mandible, which extends from the follicles of the unerupted teeth 34 and 35 to the distal area of the root apex of tooth 43. Mild buccolingual expansion is noted. The lesion scallops the internal surfaces of the buccal and lingual cortices and displaces the roots of teeth 31, 41, 42, and 43 in distobuccal directions. Tooth 33 is impacted and has been displaced inferiorly. Tooth 34 is displaced distally and buccally and is positioned buccal to the impacted tooth 35. No root resorption is noted on the involved teeth.

Missing or extracted tooth 25

Tooth 25 appears absent or extracted.

Normal remaining dentition in the maxilla

The remaining teeth in the maxilla demonstrate a normal appearance. No signs of cystic degeneration or intraosseous pathology is evident.

Axial views of the maxillary teeth

These axial cross-sectional images demonstrate the normal presence of the teeth and follicles in the maxilla. There is no evidence of cystic lesion in the maxillary bone.

Reconstructed lateral skull view

This lateral skull image reconstructed from CBCT data demonstrates a slight depression of the premaxilla. The remaining osseous and soft tissue structures in the image appear normal.

Reconstructed PA view

This PA cephalometric image reconstructed from CBCT data demonstrates relative symmetry in the mandible. The vertical positions of the condyles and mandibular angles are comparable. The remaining osseous and soft tissue structures in the image appear normal.

1. Abnormality of C1

The posterior arch of C1 exhibits a possible developmental cleft, which mimics a Jefferson's type of fracture, but the cortical borders of the separated parts of the posterior arch appear normal, which supports the diagnosis of a congenitally split atlas. This may be a part of skeletal abnormalities associated with the Gorlin-Goltz syndrome or an incidental finding with no association with the syndrome.

Otherwise normal cervical vetebrae

The cervical vertebrae that are visible in the field of view appear normal radiographically. No signs of osseous deformity are present. No abnormal curvatures are present.

2. Tentorium cerebelli calcifications

Linear calcification is present along the tentorium cerebelli. This may support the diagnosis of the Gorlin-Goltz syndrome. This type of calcification is not common in young individuals.

3. Physiologic intracranial calcifications

Midline and parasagittal physiologic calcifications are present intracranially. This is an incidental finding with no significance.