Keratocystic odontogenic tumor

Keratocystic odontogenic tumor (KCOT) is a benign neoplasm that originates from the dental lamina. It is a unique entity due to its aggressive behavior, high recurrence rate, and characteristic histopathological findings.

Etiology

The etiology of KCOT is not completely understood. However, it is believed to be associated with mutations in the PTCH1 gene. This gene is also associated with nevoid basal cell carcinoma syndrome (NBCCS), a condition that increases the risk of developing several types of cancer, including KCOT.

Clinical Features

KCOTs are often asymptomatic and are usually discovered during routine dental radiographic examinations. When symptoms do occur, they may include pain, swelling, and the presence of a cyst or lump in the mouth or jaw.

Diagnosis

The diagnosis of KCOT is based on a combination of clinical, radiographic, and histopathological findings. Radiographically, KCOTs often present as a unilocular or multilocular radiolucent lesion with well-defined borders. Histopathologically, KCOTs are characterized by a thin, uniform epithelial lining with a corrugated parakeratin surface and a prominent basal cell layer.

Treatment

The treatment of KCOTs is controversial due to their high recurrence rate. Treatment options include conservative methods such as enucleation and curettage, as well as more aggressive methods such as resection.

Prognosis

The prognosis of KCOTs is generally good, but the high recurrence rate can lead to significant morbidity. Regular follow-up is essential to monitor for recurrence.

See Also

• Odontogenic tumor
• Nevoid basal cell carcinoma syndrome
• PTCH1

References

Keratocystic odontogenic tumor Resources
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External Links

• Keratocystic odontogenic tumor: A 10-year retrospective study of 83 cases in an Iranian population (Journal of Oral and Maxillofacial Pathology)
• Keratocystic Odontogenic Tumor (GeneReviews)