Non-Calcifying/Langerhans Cell-Rich Calcifying Epithelial Odontogenic Tumour: A Critical Review of the Rare and Distinctive Entity.

BACKGROUND

The study aims to analyse the non-calcifying/Langerhans cell rich (NCLC) subtype of calcifying epithelial odontogenic tumour (CEOT).  METHOD: The features of cases of the NCLC subtype of CEOT noted in the English literature by PubMed as well as 3 new cases were reviewed.

RESULTS

Overall, twenty-one cases were noted. Many were women in the fourth to sixth decades (male-to-female ratio =1 to 2). Radiologically, the lesion is often unilocular with resorption of the affected teeth. Nineteen of the 21 cases occurred in the maxilla, especially the anterior portion. On pathological examination, epithelial cells are noted in non-calcifying amyloid-rich fibrous stroma. The main differential diagnosis is the amyloid subtype of central odontogenic fibroma. Immunohistochemical studies revealed the tumour epithelial cells were positive for cytokeratins and p63 and contained CD1a, S-100, and langerin-positive Langerhans cells. On a median follow-up of 2 years, one patient had a recurrence one year after curettage.

CONCLUSION

The NCLC subtype of CEOT is unique as it contains significant numbers of Langerhans cells and has clinicopathological features distinctive from classic CEOT.