Gorlin-Goltz syndrome

Case contributed by Dr Dalia Ibrahim

Presentation

The patient had a history of multiple maxillary and mandibular cysts. He has undergone a biopsy from the left maxillary cyst.

Patient Data

Gender: Male
Modality: CT

Multiple unilocular cysts of variable sizes seen at the mandibular symphysis, body and rami as well as both maxilla, the left maxillary cyst shows internal gas lucency, likely sequel after biopsy.

Calcification of falx cerebri, tentorium and petroclinoid ligaments.

Bilateral maxillary, ethmoidal and frontal sinusitis.

Case Discussion

Biopsy proven odontogenic keratocyte. No evidence of malignancy.

Major criteria of Gorlin-Goltz syndrome

  • basal cell cancers : > 2 or 1 under the age 20
  • odontogenic keratocysts 
  • bilamellar calcification of the falx cerebri
  • palmar pits : 3 or more
  • rib anomalies : bifid rib, fused, splayed
  • first degree relative with Gorlin syndrome
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Case Information

rID: 28511
Case created: 27th Mar 2014
Last edited: 6th Nov 2015
System: Head & Neck
Inclusion in quiz mode: Included

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