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Abstract

Keratocystic odontogenic tumor (KCOT), previously termed odontogenic keratocyst (OKC), is a unique pathological entity characterized by aggressive behavior, a high recurrence rate, and association with nevoid basal cell carcinoma syndrome (NBCCS). Once classified as a cyst, KCOT has gained tumor status due to its neoplastic potential, distinctive histopathological features, and complex molecular profile. This article provides a detailed overview of KCOT, covering its classification, etiology, clinical presentation, radiographic features, histology, differential diagnosis, treatment options, and recurrence potential. Keratocystic Odontogenic Tumor (KCOT), previously known as odontogenic keratocyst (OKC), is a unique and enigmatic developmental cyst of odontogenic origin that has attracted considerable attention due to its aggressive clinical behavior, high recurrence rate, and controversial classification. Initially described as a cyst, KCOT was reclassified as a tumor by the World Health Organization (WHO) in 2005 owing to its neoplastic characteristics, only to be reclassified again in 2017 as a cystic lesion based on updated molecular and clinical evidence. KCOT is typically found in the posterior mandible of young adults and is often associated with the nevoid basal cell carcinoma syndrome (NBCCS or Gorlin-Goltz syndrome). Its pathogenesis is linked to mutations in the PTCH1 gene, part of the Hedgehog signaling pathway, which underscores its neoplastic potential in syndromic and some sporadic cases. Radiographically, KCOT often presents as a unilocular or multilocular radiolucency with well-defined margins, and histologically, it features a parakeratinized stratified squamous epithelial lining with a palisaded basal layer. Clinically, the lesion may remain asymptomatic or present with swelling, pain, and cortical expansion. Due to its high propensity for recurrence, especially when treated with simple enucleation, various management strategies ranging from conservative (e.g., decompression, marsupialization) to aggressive (e.g., resection, adjunctive therapies like Carnoy’s solution or cryotherapy) have been employed. This comprehensive review aims to explore the historical evolution, pathogenesis, clinical and radiographic features, diagnostic criteria, histopathological profile, treatment modalities, recurrence patterns, and future directions in the understanding and management of KCOT. By integrating the latest evidence from the literature, we seek to provide a nuanced and up-to-date perspective on this complex odontogenic lesion, highlighting the challenges in classification, diagnosis, and treatment planning, and underscoring the importance of long-term follow-up in clinical practice.