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Calcifying epithelial odontogenic tumor

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CALCIFYING EPITHELIAL ODONTOGENIC TUMOR: CLINICAL & RADIOGRAPHIC FEATURES, HISTOPATHOLOGY AND TREATMENT

Author: Sanketh DS, MDS

INTRODUCTION & PATHOGENESIS

Calcifying epithelial odontogenic tumor (CEOT) is a rare benign lesion, that comprises 0.4 – 3% of all odontogenic tumors. It has been eponymously called Pindborg tumor after JJ Pindborg first described it in 1955. 

The odontogenic origin of CEOT has been a subject of debate, as some pathologists believe CEOT to arise from the epithelial remnants of disintegrated dental lamina whereas some others argue that the epithelial cells of the tumor resemble the stratum intermedium layer of the enamel organ. However, the exact etiology of this lesion still remains unknown.

CLINICAL FEATURES

CEOT occurs in patients across a wide age range, ranging from 8-92 years with the mean age range being between the 3rd and 5th decades. CEOT could be intra-osseous or extra-osseous with the former comprising 94% of all CEOT lesions. It occurs equally among males and females and 2/3rds of the reported CEOTs occur in the mandible with a predilection for the posterior molar regions.

The lesion usually presents as an asymptomatic swelling and 50% of the cases have been reported to be associated with an unerupted tooth.

RADIOGRAPHIC FEATURES

The lesion shows different radiographic variations and most commonly manifests as a mixed radiolucent-radiopaque lesion with a unilocular or a multilocular radiolucency filled with numerous radiopaque scattered flecs of calcification of varying sizes. This has been termed as “driven snow appearance”.

Besides, CEOT could also manifest as a unilocular or multilocular radiolucency with the unilocular radiolucency being more common in the maxilla.  Lesions associated with an unerupted tooth, may show a peri-coronal radiolucency and may mimic a dentigerous cyst.

HISTOPATHOLOGY

CEOT shows small islands or sheets of polygonal epithelial cells in a fibrous stroma. The epithelial cells in most cases appear uniform and monomorphic, although there are cases that show pleomorphism of cells, with nuclei also showing considerable pleomorphism and increase in size. However, pleomorphism in CEOT does not indicate malignancy. The cells show distinct borders and intercellular bridges are prominent under high power.

A characteristic feature of CEOT is the presence of homogenous, eosinophilic and hyalinised amyloid like material scattered in focal areas. Also, calcifications may develop within the amyloid like material to form concentric rings called “liesegang rings”. The amyloid-like material stains positively with Congo red and exhibits an apple-green birefringence in polarized light.

TREATMENT & PROGNOSIS

Though CEOT was thought to be locally aggressive it is presently believed not to be very aggressive and is rather thought to be an expansile lesion. It has a recurrence rate of 15% but this is attributed to either inadequate treatment or treatment by curettage. CEOT does not require aggressive approaches to treatment and usually warrants a local resection with inclusion of a narrow rim of surrounding bone. Maxillary lesions however, tend to be more rapidly growing than the mandibular lesions and may require a more aggressive treatment. The overall prognosis for CEOT is good and 5 years should be a minimum follow-up period to assess the status of treatment.

REFERENCES

Odontogenic tumors and allied lesions. Reichart and Philipsen.

Shafer’s Textbook of Oral Pathology. Rajendran and Sivapathasundaram.6th Edition.

Oral and Maxillofacial Pathology.Neville,Damm,Allen,Chi. South Asian Edition.

Angadi PV and Rekha K. Calcifying Epithelial Odontogenic Tumor (Pindborg Tumor). Head Neck Pathol. 2011 Jun; 5(2): 137–139.

Kaushal S, Mathur SR, Vijay M, and RustagiA. Calcifying epithelial odontogenic tumor (Pindborg tumor) without calcification: A rare entity. J Oral Maxillofac Pathol. 2012; 16(1): 110–112.

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