| Abstract|| |
Calcifying epithelial odontogenic tumor (CEOT) is a rare, benign odontogenic tumor. It constitutes 0.4-3% of all odontogenic tumors. There is only a single case, which has been reported in the Indian literature that too in cytology. Microscopically, these are composed of large sheets of epithelial cells, amorphous amyloid-like material and calcification. Although these lesions are benign, they can be locally aggressive, but malignant transformation and metastasis is rare. Considering its locally aggressive nature, appropriate management and long-term follow-up is recommended. We describe two cases of CEOT involving the hard palate and leading to facial alterations.
Keywords: Benign odontogenic tumor, calcifying epithelial odontogenic tumor, maxilla, Pindborg tumor
|How to cite this article:|
Mandal S, Varma K, Khurana N, Mandal A K. Calcifying epithelial odontogenic tumor: Report of two cases. Indian J Pathol Microbiol 2008;51:397-8
| Introduction|| |
Calcifying epithelial odontogenic tumor (CEOT) is a rare, benign odontogenic neoplasm, forming only 0.4-3% of all intraosseous odontogenic tumors. ,,,,, Only a single case of CEOT has been reported in the Indian literature. 
We describe two cases of CEOT in a young female presenting as a growth in the hard palate.
| Case Histories|| |
A 17-year-old female presented with a lump on the right side of hard palate. The growth was initially small in size and increased gradually over a period of 2 years. On examination, there was a proliferative growth in the right side of hard palate measuring 6 × 5 × 4 cm, causing deformity of face. CT PNS showed a lytic destructive, expansile lesion of right alveolar process of maxilla with extension into right buccal space and hard palate with features of calcification and new bone formation. Biopsy from the hard palate showed a low-grade epithelial tumor composed of polyhedral cells with prominent interdigitation, focal moderate anisonucleosis and areas of calcification features on which the diagnosis of CEOT was based. Surgery was performed, which confirmed our diagnosis.
Gross examination showed a part of hard palate along with a growth, which measured 6 × 5 × 4 cm. The normal alignment of the teeth was distorted by the growth. The cut surface was gray white, firm with involvement of surrounding alveolar process of maxilla [Figure 1a-c]. Microscopically, the tumor tissue was covered with keratinized hyperplastic squamous epithelium [Figure 2a]. The subepithelial tissue showed a cellular tumor composed of sheets of polygonal cells, which at places were showing prominent interdigitations. The nuclei were normochromatic with only focal mild pleomorphism. The sheets were separated by dense fibrous tissue at places appearing as acellular amyloid-like, with focal areas showing scanty to moderate calcification of this acellular material [Figure 2b]. Mitosis was insignificant. Immunohistochemistry was positive for pan-cytokeratin (CK) and epithelial membrane antigen (EMA) [Figure 2c]. Final diagnosis of CEOT was made. One-year follow-up of the patient was uneventful.
A 22-year-old female presented with a progressively increasing swelling in the upper lip for the past 6 months. On examination, there was firm, whitish, multilobulated soft tissue swelling in the upper labial sulcus and alveolus that measured 5 × 4 × 4 cm. The patient also gave a history of being operated for a cyst in the same region 1 year back. X-ray PNS showed an ill-defined mixed radiolucency in place of missing upper two incisor teeth, involving the alveolus and encroaching upon nasal cavity and hard palate. Complete enucleation of the lesion was done.
Grossly, we received multiple irregular gray white, firm, soft tissue measuring 6 × 4 × 3 cm. Cut surface was gray white with firm areas. Microscopically, the tumor was composed of sheets of polyhedral cells with prominent intercellular bridges. There were few areas of amorphous, eosinophilic material and the center of some of these areas was calcified, some of which were concentric-like psammoma bodies [Figure 2d,e]. Final diagnosis of CEOT was made. The patient is still under follow-up for the past 2 years and no recurrence has occurred.
| Discussion|| |
Calcifying epithelial odontogenic tumor (Pindborg) tumor was first described by Pindborg in 1955.  The term 'Pindborg' was introduced by Shafer et al. , in 1963.  Only a single case has been reported in the Indian literature till now. 
The tumor is thought to arise from the epithelial elements of the enamel, but the exact part from which it originates is not known. However, it has been postulated that it may arise from either the external epithelium, stratum intermedium of the enamel organ or from the cellular remnants of the basal sheet or basal stratum of the gingival epithelium. 
The age range is wide 8-92 years, although it is most commonly seen in the fourth and fifth decade of life. , Ninety-four percent of the lesions are central and intraosseous and 6% are extraosseous. ,,, Intraosseous CEOT shows a maxilla-to-mandible site ratio of 1:2 and is mainly located in the premolar/molar region. In this case, both the lesions were located in the maxilla. Half of the cases are associated with an impacted tooth.  Clinically, it presents as a slowly growing expansile jaw lesion, which may be associated with pain, nasal obstruction, epistaxis and headache.  Maxillary, tumors may present with facial alteration. In the present report, one of the cases (case 1) presented with mass in the hard palate with facial alteration.
In the initial stage, it is totally radiolucent, simulating a dentigerous because of its relation with impacted tooth. Small intratumoral calcification starts appearing in the second phase, which is characteristic but not diagnostic. The final stages are associated with osseous destruction and the tumoral calcification giving it a honeycomb appearance. Intraosseous lesions that are large in size are generally multilobulated and can simulate an ameloblastoma radiologically. 
Although these lesions are benign, they can be locally aggressive, but malignant transformation and metastasis is rare. The local recurrence rate is 10-15%.  The treatment is generally surgical, varying from simple enucleation to partial resection of the affected bone. 
Calcifying epithelial odontogenic tumor is a rare, benign and destructive lesion, which is often confused clinically and radiologically with dentigerous cyst and other osseous tumors. Considering its locally aggressive nature, appropriate management and long-term follow-up is recommended.
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Department of Pathology, Maulana Azad Medical College, New Delhi - 110 002
Source of Support: None, Conflict of Interest: None
[Figure 1a-c], [Figure 2a], [Figure 2b], [Figure 2c], [Figure 2d,e]