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21 yo, M

Painless mild swelling

Radiolucent lesion of the anterior maxilla


Gross Findings

The biopsy consisted of multiple strips of cystic epithelium with calcified luminal accretions. Entirely submitted.


Microscopic Findings

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Final Diagnosis:


  • Rare, accounting for less than 1% of odontogenic cysts
  • No gender predilection
  • Peak incidence in the second and third decades
  • Almost equally in the maxilla and mandible.
  • In the maxilla there is a strong predilection for the anterior side
  • Approximately 10% of cases are extraosseous with a predilection for the anterior mandibular gingiva
  • COC has mutations in CTNNB1, which encodes β catenin



  • Treated by conservative surgical removal, enucleation and/or curettage.
  • 8% recurrence rate


Take-Home Messages

  • It exhibits histologic features similar to pilomatrixoma, the epithelium transitions into ghost cell-type keratinization
  • Though characteristic, ghost cells form in other odontogenic tumours and do not alone justify a diagnosis of this cyst
  • The basal cells resemble ameloblastoma, but there is no microcystic change/cytoplasmic vacuoles between the nuclei and the basement membrane, and the cells are variable cuboidal to columnar, as opposed to tall columnar in ameloblastoma.
  • The differential diagnosis includes dentinogenic ghost cell tumor {27669959} and ghost cell odontogenic carcinoma.