Non-syndromic Multiple Keratocystic Odontogenic Tumor: A Case Report - Non-syndromic Multiple Keratocystic Odontogenic Tumor: A Case Report - Open Access Pub

A Keratocystic Odontogenic Tumor (KCOT) is a benign odontogenic lesion that is well-known for its histopathological features and invasive clinical behaviors. KCOTs may be non-syndromic or syndromic, and the syndromic type presents a higher risk of recurrence and invasive behaviors. Non-syndromic KCOTs are uncommon and account for only 5% of the cases. The purpose of the present case report is to conduct clinical, radiographic and pathologic evaluations and treat a 37-year-old man with non-syndromic nevoid basal cell carcinoma. Syndromic and non-syndromic keratocysts present more invasive behaviors than the solitary type. The initial symptoms of syndromic keratocysts appear as multiple keratocysts of the mandible and maxilla that are diagnosed by a dentist; as a result, the diagnosis and treatment of the disease is very crucial. DOI10.14302/issn.2379-8572.joa-17-1668 Odontogenic keratocyst is a developmental odontogenic cyst that requires special considerations due to its histopathological features and invasive clinical behaviors. The WHO has recommended the term KCOT to be used as it better reflects the molecular genetic changes in the cyst, which resemble the changes in certain neoplasms 1. Multiple odontogenic keratocysts are normally associated with concomitant cutaneous, skeletal, ocular and neurological anomalies as part of nevoid basal cell carcinoma syndrome or the Gorlin-Goltz syndrome, 2 although they may also be associated with the Simpson-Golabi-Behmel syndrome, the orofacial digital syndrome, the Noonan syndrome and the Ehler-Danlos syndrome 2, 3, 4, 5. Non-syndromic multiple odontogenic keratocysts are uncommon and only about 5% of the patients report this type 2. KCOTs originate from the remnant of the dental lamina and normally affect a wide age range but affect men very little 1. The most common sites involved in odontogenic keratocysts include the posterior mandible body and the ramus areas 1. Its syndromic type usually occurs in the first decades of life and presents a higher risk of recurrence as well as more invasive behaviors compared to the non-invasive type. Odontogenic keratocysts may turn into invasive neoplasms such as ameloblastoma and squamous cell carcinoma. The syndromic and non-syndromic types of keratocysts are more invasive than the solitary types 6. Given that multiple KCOTs are very rare and may be the first symptom of the syndromic condition and since the non-syndromic type is less common, the present study was conducted to perform clinical, radiographic and pathologic evaluations and treatment non-syndromic multiple