Introduction
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History
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:
CALCIFYING ODONTOGENIC CYST
- 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
Treatment
- 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.