Odontogenic keratocysts (OKCs) associated with NBCCS have occasionally been reported to

transform into aggressive neoplasms such as ameloblastomas and squamous cell carcinoma.[10]
The cyst lining seen in the NBCSS-related odontogenic keratocyst (OKC) is classically
parakeratinized and does not appear to be associated with the orthokeratinized variant of the
OKC.
Many odontogenic tumors have been described to have extraosseous equivalents, and although
odontogenic keratocysts (OKCs) are regarded primarily as intraosseous lesions, they can also
occur in a peripheral location[11] ; 16 such cases have been reported in the literature with a mean
patient age of 57.8 years and a female predilection.[12] Peripheral odontogenic keratocysts can be
seen in association with NBCCS.[12]
The cystic nature of odontogenic keratocyst (OKC) has long been debated, with some
investigators classifying the OKC as a benign tumor.[13] In recent years, the WHO has
recommended that the term "keratocystic odontogenic tumor" (KCOT) replace the term
"odontogenic keratocyst" (OKC), as it better reflects the neoplastic nature of the lesion.[14, 15]
Several factors, such as the cyst's aggressive behavior, high mitotic activity histologically, and
evidence of associated genetic and chromosomal abnormalities (eg, mutation of the PTCH gene)
often seen in neoplasia, serve as the basis for this new classification.[6, 15, 16]