Orthokeratinized odontogenic cyst (OOC): Clinicopathological and radiological features of a series of 10 cases

Nasir Uddin, Maha Zubair, Jamshid Abdul-Ghafar, Zia Ullah Khan, Zubair Ahmad

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17 Citations (Scopus)


Background: Orthokeratinized Odontogenic Cyst (OOC) is a rare, developmental odontogenic cyst which was considered in the past to be a variant of Odontogenic keratocyst (OKC) later renamed as keratocystic odontogenic tumor (KCOT). The treatment of OOC is by enucleation and the prognosis, following enucleation is excellent with a recurrence rate of less than 2%. On the other hand, OKC has a recurrence rate between 8 and 25% after enucleation. Thus it is important to differentiate between the two entities. Methods: All cases reported in our section as OOC during the period 2013 to 2018 were retrieved from the surgical pathology files and slides were reviewed by the authors. All cases which met the histological criteria for OOC were included. Results: A total of 10 cases were included. 70% patients were males, ages ranged from 23 to 60 years, with mean age of 38.9 years. 70% of cases were located in the mandible and 90% patients presented with swelling. Radiologically, 90% cases were unilocular, all were radiolucent lesions. Mean size was 4.0 cm. Histologically, all cases demonstrated the classic microscopic features. Follow-up was available in 8 patients. All were treated by enucleation. All 8 were alive with no recurrences over a follow-up period ranging from 7 to 62 months. Conclusions: OOC has a better prognosis than OKC and needs to be differentiated from OKC due to differences in treatment and prognosis. Large majority of our cases presented with swelling and occurred in the mandibles of young males. All were radiolucent and most were unilocular. All were treated by enucleation and no recurrences occurred over follow up period ranging up to 62 months. Our findings were similar to those described in other published series.

Original languageEnglish
Article number28
JournalDiagnostic Pathology
Issue number1
Publication statusPublished - 4 Apr 2019


  • Keratocystic odontogenic tumor
  • Odontogenic cyst
  • Odontogenic keratocyst
  • Orthokeratinized odontogenic cyst


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