Premalignant changes in odontogenic keratocyst of a 14-year-old patient

Introduction

Odontogenic keratocyst (OKC) is known for its characteristic behavior, aggressive growth pattern, and a high tendency to recur. Dysplastic change in an OKC is an infrequent phenomenon.

Case Report

We report an unusual case of an OKC, exhibiting premalignant changes, in the anterior mandible of a 14-year-old female. The lesion was an incidental finding during a routine dental examination. The patient was asymptomatic, and no signs of swelling or cortical expansion were noted. Radiographically, it presented as a unilocular, well-defined, expansile radiolucency with partially corticated margins. There was a marked divergence of mandibular incisors and canine. The patient exhibited palmar pitting, as seen in Gorlin-Goltz syndrome. Histologic evaluation of the incisional biopsy showed moderate to severe dysplastic changes in the lining epithelium. The cyst was enucleated 2 months later and the biopsy revealed changes consistent with severe dysplasia and focal carcinoma in situ. Atypical changes such as nuclear hyperchromatism, increased nuclear/cytoplasmic ratio, loss of cellular polarity, dyskeratosis, and atypical mitoses were observed. Occasional teardrop-shaped budding of the lining epithelium was present. The underlying connective tissue displayed an intense inflammatory infiltrate and scant germinal centers. The patient will be periodically re-evaluated and followed up closely.

Conclusion

Epithelial dysplasia in OKC is not a common occurrence. Whether OKCs with epithelial dysplasia have a higher recurrence and/or should be treated differently than OKCs without dysplasia, especially in syndromic patients, remains uncertain. Should dysplasia be considered a predictive factor for the recurrence of an OKC? Since many OKCs may not recur until 10 years or more after the initial treatment, a long-term follow-up, including periodic radiographic imaging, is recommended.