|Year : 2008 | Volume
| Issue : 1 | Page : 28-31
Clear cell calcifying epithelial odontogenic tumor: A rare case report
Jincy Thomas, Valsa Thomas, Nileena R Kumar, Sharafuddeen
Department of Oral Medicine and Radiology, Govt. Dental College, Calicut-673 008, India
Department of Oral Medicine and Radiology, Govt. Dental College, Calicut-673 008
Source of Support: None, Conflict of Interest: None
| Abstract|| |
The calcifying epithelial odontogenic tumor (CEOT) is a benign epithelial odontogenic lesion that accounts for less than 1% of all odontogenic tumors. Clear cell variant of CEOT is extremely rare with only fifteen cases documented till date. The occurrences of clear cells may prove to be a sign of increased tumor aggressiveness, indicating the need for a more radical surgical approach.
Keywords: Calcifying epithelial odontogenic tumor and clear cell
|How to cite this article:|
Thomas J, Thomas V, Kumar NR, Sharafuddeen. Clear cell calcifying epithelial odontogenic tumor: A rare case report. J Indian Acad Oral Med Radiol 2008;20:28-31
|How to cite this URL:|
Thomas J, Thomas V, Kumar NR, Sharafuddeen. Clear cell calcifying epithelial odontogenic tumor: A rare case report. J Indian Acad Oral Med Radiol [serial online] 2008 [cited 2021 Jan 22];20:28-31. Available from: https://www.jiaomr.in/text.asp?2008/20/1/28/44358
| Introduction|| |
Calcifying epithelial odontogenic tumor (CEOT) is a very rare neoplasm with only fewer than 200 cases reported till date. CEOT accounts for less than 1% of all odontogenic tumors. Fifteen cases of documented examples of clear cell calcifying epithelial odontogenic tumor (CCCEOT) have been published so far.  This benign and locally aggressive tumor was first reported by Pindborg in 1955  and he called it CEOT. They arise from the reduced enamel of embedded tooth or from stratum intermedium of enamel organ.  The case reported here is a 55 year old female with clear cell variant of CEOT.
| Case Report|| |
A 55 year old female was referred to the Department of Oral Medicine and Radiology, Goverment Dental College, Calicut with the chief complaint of pain in the left side lower jaw since 2 weeks. Her history revealed that she had extracted a left lower posterior tooth which was grossly decayed from a private dentist with the same complaint. Also took antibiotics and analgesics. Had mild symptomatic relief and later after 3 days the pain started again and was referred to our college.
On general examination, she was well-nourished and moderately built. A midline neck swelling was noticed, which moved with deglutination, and later proved to be multi nodular goiter by FNAC. She also had left sided sub mandibular lymphadenopathy.
Extraorally there was no facial asymmetry or any sinus opening [Figure 1].
Intraorally there was missing teeth in relation to 16, 26, and 47 for which history of extraction was present. There was a root stump in relation to 36.
On palpation, there was tenderness in the alveolar ridge and retromolar area distal to 37 and slight buccal cortical expansion of uniform bony hard consistency in the retromolar region compared to the opposite side [Figure 2].
The patient was subjected to radiographic examination. The panoramic view demonstrated a multilocular radiolucency 4x3 cm in angle ramus region of left side region. It extends from the distal aspect of 37, extending posteriorly till about 1 cm from the mandibular notch, it has a scalloped border, and the lesion is expansile with slight expansion of anterior border of ramus of mandible mesially about 0.3 cm. In the ramus region, it extends approximately about the full width of ramus [Figure 3].
Bone biopsy was done and histopathological report came as clear cell variant of CEOT [Figure 4].
Subsequently a computed tomography (CT) scan was advised. CT demonstrated lytic lesion involving angle and ramus of mandible on left side. Level I lymphnode of maximum short axis diameter 6 mm on left side. Level II lymphnode of maximum axis diameter 9 mm on left side [Figure 5],[Figure 6],[Figure 7].
Resection of the lesion was done and there was no recurrence till date.
| Discussion|| |
CEOT accounts for less than 1% of all odontogenic tumors  the relative tumor frequency ranges between 4% and 3% of which extra osseous variant constitutes about 6% and intraosseous variant 93.6% of total CEOT. The age range of all CEOT cases varies between 8 and 92 years and the mean being 38.9 years. For extra osseous type the age range is between 12 and 64 years and mean 34.4 years. Extra osseous CEOT are diagnosed at an earlier age due its presentation as a gingival mass, but intra-osseous CEOT are diagnosed only by 3 rd, 4 th or 5 th decade of life. 
The overall age range of clear cell variant of CEOT ranges from 14 to 68 years with a mean of 41.5 years. Mean age for the intra osseous CCCEOT is however considerably higher (46.3 years) than for the extra osseous variant (34.3 years). The male-female ratio for the intra osseous located CCCEOT is 1 : 2 compared to 1 : 1 for extra osseous variant.
The site of CEOT studied in 181 cases is within the jaw bone. The intra-osseous tumors were found primarily in the mandible, and in 82% of cases the tumor was located in the premolar and the molar region. 
Philipsen and Reichart, most recently reported 50 cases of intra-osseous CEOT associated with an unerupted tooth, of which 52% of the teeth were identified as mandibular molars.
Recent studies conducted by Chaudhry and associates, emphasizes that the tumor cells exhibit morphologic characteristics of squamous epithelium derived from reduced enamel epithelium of related unerupted tooth, in the peripheral variant the tumor arises from rests of dental lamina or basal cells of oral epithelium. The epithelial remnants of dental lamina complex is conceptualized as unified source of origin for the diverse locations of CEOT. 
Intraosseous CEOT is by far the most common, usually shows local invasiveness. They present as a painless mass with slow growth where as in this case the tumor started as a painful jaw swelling. When CEOT is located in maxilla the patient may complain of nasal congestion, epitasis and headache. 
Radiograph taken early in the development of this tumor reveal a radiolucent area around the crown of a mature, unerupted tooth. Periphery of the lesion may be a well-defined cyst like cortex or in some cases ill-defined. The internal structure may be unilocular or multilocular (honeycomb) with numerous scattered radio opaque foci (driven snow appearance) of varying size and density. The most characteristic and diagnostic finding is the appearances of radiopacities close to the crown of embedded tooth, in addition small thin opaque trabeculae may cross the radiolucency in many direction.
CEOT can displace developing tooth or prevent its eruption, there can be an associated expansion of the jaw with the maintenance of a cortical boundary.  The diagnostic difficulty can be caused by dentigerous cyst, ameloblastoma, adenomatoid odontogenic tumor, ameloblastic fibro-odontoma and calcifying odontogenic cyst.
Extra osseous CEOT - this is a variant of CEOT which appears most commonly as a painless firm gingival mass the mucosa may show ulcerations due to trauma. It can be confused with fibrous hyperplasia, peripheral giant cell granuloma and epulis. 
Histologically, CEOT is a benign but locally invasive neoplasm originating from odontogenic epithelium and characterized by sheets and islands of uniform, vacuolated and clear cells. CCOT can be distinguished from clear cell variant of CEOT because it lacks the characteristic calcifications and amyloid-like deposition.  Yamaguchi et al . believed that clear tumor represent a feature of cytodifferentiation rather than a simple degenerative phenomenon.  It should be remembered that not only clear cell CEOT but also several other odontogenic tumors such as solid multicystic ameloblastoma,  calcifying ghost cell odontogenic tumors  and occasionally adenomatoid odontogenic tumors  may show clear cell differentiation. Primary tumors of putative odontogenic origin, composed principally of clear cells, have recently been described under the diagnosis of clear cell odontogenic tumor (CCOT).  CCOT can be distinguished from clear cell variant of CEOT because it lacks the characteristic calcifications and amyloid like deposition. 
Methods of treatment have ranged from simple enucleation or curettage to hemimandibulectomy or hemimaxillectomy for CEOT. Eleven cases of clear cell CEOT with limited follow-up information do not allow conclusions to be drawn regarding the biologic behavior, treatment and prognosis of this variant.  Recurrence rates was stated to be 14% which is mostly attributable to inadequate treatment.  However, as reported  the occurrences of clear cells may prove to be a sign of increased tumor aggressiveness, indicating the need for a more radical surgical approach. Five years should be the absolute minimum follow-up necessary to assess the cure for CEOT. 
CEOT is an extremely rare benign locally invasive neoplasm located either intraosseously or extraosseously. This article highlights about the clear cell variant of CEOT, its manifestations, radiological features and management, emphazing the aggressive nature of the neoplasm and the need for a radical surgical approach.
| References|| |
|1.||Philipsen HP, Reichart PA. Calcifying epithelial tumor: Biological profile based on 181 cases from the literature. Oral Oncol 2000;36:17-26. [PUBMED] [FULLTEXT]|
|2.||Pindborg JJ. Calcifying epithelial odontogenic tumors. Acta Pathol Microbiol Scant 1995;111:71. |
|3.||Ghom AG. Textbook of Oral Medicine. Jaypee Pub; p. 229-15. |
|4.||Neville BW, Damm, Allen CM, Bouqout JE, editors. Oral and maxillofacial pathology. WB Saunders; p. 623-15. |
|5.||Chaudhry AP, Holte NO, Vickers RA. Calcifying epithelial odontogenic tumor: Report of a case. Oral Surg Oral Med Oral Pathol 1962;15:43-8. |
|6.||Philipsen HP, Reichart PA. Odontogenic tumors and allied lesion. Quintessence Publishing Co Ltd; p. 93-10. |
|7.||White and Pharoah. Oral radiology-principles and Interpretations. 5 th ed. Elsevier; p. 423. |
|8.||Chomette G, Auriol M, Guilbert F. Histoenzymological and ultra structural study of a bifocal calcifying odontogenic tumor: Characteristics of epithelial cells and histogenesis of amyloid like material. Virchows Arch A Pathol Anat Histopathol 1984;403:67-76. |
|9.||Yamaguchi A, Kokubu JM, Takagi M, Ishikawa G. calcifying epithelial odontogenic tumor: Histochemical and electron microscopic observations on a case. Bull Tokyo Med Dent Univ 1980;27:129-35. |
|10.||Muller H, Slootweg P. Clear cell differentiation in an ameloblastoma. J Maxillofac Surg 1986;14:158-60. |
|11.||Ng KH, Siar CH. Clear cell change in a calcifying odontogenic cyst. Oral Surg Oral Med Oral Pathol 1985;60:417-9. |
|12.||Philipsen HP, Reichart PA, Nekai H. The adenomatoid odontogenic tumor (AOT): An Update. Oral Med Pathol 1998;2:55-60. |
|13.||Koppang HS, Bang G, Hansen SL, Gilhuus Moe O, Aksdal E. Hellzelliger odontogener tumor: Kasuistischer Beitrag. Dtsch Z Mund kiefer Geischtschir 1988;12:356-60. |
|14.||Hansen LS, Eversole LR, Green TL, Powell NB. Clear cell odontogenic tumor: A new histologic variant with aggressive potential. Head Neck Surg 1985;8:115-23. |
|15.||Franklin CD, Pindborg JJ. The calcifying epithelial odontogenic tumor: A review and analysis of 113 cases. Oral Surg Oral Med Oral Pathol 1976;42:753-65. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]