Rare diseases · Sign or symptom
Odontogenic keratocysts of the jaw
HP:0010603
What it means
A benign uni- or multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium and potential for aggressive, infiltrative behavior.
Keratocystic odontogenic tumor (KCOT) most commonly affects the mandible, but may also be localized in the maxilla.
Rare diseases that can present with this3
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Keratocystic odontogenic tumor · Keratocystic odontogenic tumour · Keratocysts of the jaw
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.