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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
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Year : 2013  |  Volume : 17  |  Issue : 3  |  Page : 479
 

Clear cell variant of calcifying epithelial odontogenic tumor of maxilla: Report of a rare case


1 Department of Oral and Maxillofacial Surgery, Al Jabal Al Ghardi University, Al Zawia, Libya
2 Department of Oral and Maxillofacial Surgery, Rajarajeshwari Dental College and Hospital, Bangalore, India
3 Department of Oral and Maxillofacial Surgery, Karnataka Lingayat Education Society Dental College and Hospital, Bangalore, India

Date of Web Publication17-Jan-2014

Correspondence Address:
Shruthi Rangaswamy
Rajarajeshwari Dental College and Hospital, Mysore Road, Bangalore - 560 005, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-029X.125228

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   Abstract 

The calcifying epithelial odontogenic tumor (CEOT) is a rare benign tumor of the jaws. Pindborg's tumor having clear cells is extremely rare. Twelve central lesions have been reported of which only three cases have occurred in maxilla. Clear cell variant is a distinct entity, has more aggressive biological behavior and higher chances of recurrence. Hence it is important that presence of clear cells be included in histopathological diagnosis. Here we present a rare case of clear cell CEOT having aggressive behavior.


Keywords: Calcifying epithelial odontogenic tumor, clear cell variant, Pindborg′s tumor


How to cite this article:
Badrashetty D, Rangaswamy S, Belgode N. Clear cell variant of calcifying epithelial odontogenic tumor of maxilla: Report of a rare case. J Oral Maxillofac Pathol 2013;17:479

How to cite this URL:
Badrashetty D, Rangaswamy S, Belgode N. Clear cell variant of calcifying epithelial odontogenic tumor of maxilla: Report of a rare case. J Oral Maxillofac Pathol [serial online] 2013 [cited 2021 Jul 24];17:479. Available from: https://www.jomfp.in/text.asp?2013/17/3/479/125228



   Introduction Top


The calcifying epithelial odontogenic tumor (CEOT) is a rare benign tumor of the jaws first described by Pindborg [1] in 1955 and also referred as Pindborg tumor. [2] The clear cell variant of CEOT was first reported by Abrams and Howell [3] in 1967. Nineteen cases [3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17] of clear cell CEOT (CCEOT) have been described in literature. Topographically two entities have been distinguished: intraosseous (central) and extraosseous (peripheral). Twelve central lesions [3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13] have been reported of which only three cases have been reported in maxilla. [9],[11],[13]

Typically, CEOT has been described as slow growing, benign and locally aggressive tumor. Hicks et al., [12] suggested that clear cell variant may show more aggressive behavior with higher recurrence (22%).

Clear cells are present as cellular components of the epithelial lining of lateral periodontal and gingival cysts in adults, or they may be found as clear-cell rests of the dental lamina within the connective tissue wall of these cysts. Rarely clear cells may occur in certain epithelial odontogenic tumors. [18] Philipsen and Reichart [19] reviewed 181 cases of Pindborg tumor, 15 of which are clear cell containing lesions. Some authors have classified CCEOT as separate clinical entity.

In this paper, a rare case of CCEOT of posterior maxilla is reported and clinical, radiographic histological features of the same are discussed.


   Case Report Top


A 36-year-old Indian woman reported to our department with painless, slow growing swelling of right upper jaw and loss of upper teeth due to mobility. Intra oral examination revealed solitary swelling in right posterior maxillary region with missing posteriors. The swelling was about 3 × 2 cm with normal color, intact mucosa and indentations of opposing tooth [Figure 1]. The mass was firm on palpation with slightly more expansion towards the palatal side. OPG showed 3 × 3.5 cm mixed radiolucent-radiopaque lesion in the posterior maxilla with impacted teeth. Radiographically "driven snow" appearance was appreciable [Figure 2]. Computed tomography (CT) revealed radiolucent-radiopaque mass in the right maxilla invading the cortex [Figure 3]. The lesion extended to posterior maxilla and obliterated the right nasal cavity.
Figure 1: Intra oral photograph showing solitary swelling in right posterior maxillary region

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An incisional biopsy was taken from the lesion. The specimen was subjected to histopathological examination. Hematoxylin and Eosin stained sections showed fibrous connective tissue stroma with islands of polyhedral epithelial cells containing nuclei of varying form and size. Extracellular eosinophilic amyloid like material was found containing concentric calcifications in the form of Liesegang rings [Figure 4]. Few scattered cells were found with clear vacuolated cytoplasm (clear cells). No cellular atypia or mitotic figures seen. The features were suggestive of CCEOT.
Figure 2: Snow-driven, radiolucent-radioopaque lesion seen in right maxilla in orthopantomogram

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Figure 3: Computed tomography scan showing radiolucent-radiopaque mass in the right maxilla invading the buccal cortex

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Figure 4: Initial histology sections showing Liesegang rings (H&E stain, 200)

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Considering the age of the patient, esthetic concern and benign nature of CEOT, curettage of the lesion along with the associated tooth was performed under general anesthesia (GA).

Patient reported back after 10 months complaining of nasal obstruction and tearing. CT scan confirmed recurrence in the posterior maxilla [Figure 5]. Intranasal biopsy was performed, revealed clear cells in the stroma of CEOT confirming the diagnosis of clear cell CEOT [Figure 6]. Subtotal maxillectomy with Weber Fergusson approach was done. Six weeks postoperatively, an obturator was given. No recurrence was found on 2 years follow-up.
Figure 5: Recurrence evident in CT

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Figure 6: Photomicrograph of intranasal biopsy showing Liesegang rings and clear cells (H&E stain, 100)

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   Discussion Top


Clear cell variant of CEOT is a rare entity and central CCEOT occurring in maxilla is rarest entity. An extensive review of literature on clinical and radiologic features of CCEOT has been done by Anavi et al. [20] that records 19 cases so far comprising of 12 central and seven peripheral lesions. Equal distribution in males and females, age range of 14-68 years; and of the 12 central lesions, nine involving the mandible and three in maxilla (75:25) have been reported.

The typical clinical presentation of CCEOT is painless swelling of the jaw causing cortical expansion. Radiological features of CEOT is unilocular or multilocular lesion with mixed radiolucent and radiopacity of various density. Classically driven snow or wind driven appearance has been described. Mostly associated with impacted tooth. [21] Clinical features and radiological features of the case presented are consistent with that of the other CCEOT reported. The three maxillary lesions [9],[11],[13] described were radiolucent in OPG, in the presented case lesion was typically of driven snow appearance.

Histological sections of CEOT containing islands of polyhedral epithelial cells, having nuclei of varying form and size with extracellular eosinophilic material-amyloid and concentric calcifications within the connective tissue matrix in the form of Liesegang rings are typical. [22] Clear cell variant consists of sheets or cords of clear cells with foamy cytoplasm in the matrix. The clear epithelial cells contain glycogen, demonstrated by PAS reaction. [19] These features were detected in the case presented confirming the diagnosis of clear cell variant. Other variants of CEOT described include pigmented, [23] Langerhan cells containing [9] and non-calcifying [11] variants. Asano et al., in a CCEOT has demonstrated Birbeck granules.

The clear cell variant tends to be more aggressive and shows higher recurrence rates than conventional CEOT. Treatment options range from simple enucleation or curettage to radical and extensive resections. The surgical management modalities in 19 reported cases were complete or partial resection in seven patients (37%), excision in seven (37%), enucleation in four (21%) and curettage in one case (5%). Recurrence in two cases has been reported (17%). [20]

Waldron et al., [24] and Hicks et al., [12] have reported that occurrence of the clear cells may prove to be a sign of increased tumor aggressiveness indicating a more radical approach. Recurrence of tumor following curettage in our case strongly supports the data and enbloc resection is recommended.


   Conclusion Top


Evidence supports that clear cell variant is a distinct entity, has more aggressive biological behavior and higher chances of recurrence. Hence, it is important that presence of clear cells be included in histopathological diagnosis. The identification of clear cell directs the surgeon towards more definitive surgical excision of the lesion. Maxillary lesions should be treated more aggressively because they grow faster and possess close proximity to important structures.

 
   References Top

1.Pindborg JJ. Calcifying epithelial odontogenic tumours. Acta Pathol Microbiol Scand Suppl 1955;105:71-6.  Back to cited text no. 1
    
2.Shafer WG, Hine MK, Levy BM. A Textbook of Oral Pathology, 2 nd ed. Philadelphia: W. B. Saunders; 1963. p. 217.  Back to cited text no. 2
    
3.Abrams AM, Howell FV. Calcifying epithelial odontogenic tumors: report of four cases. J Am Dent Assoc 1967;74:1231-40.  Back to cited text no. 3
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4.Anderson HC, Kim B, Minkowitz S. Calcifying epithelial odontogenic tumor of pindborg. An electron microscopic study. Cancer 1969;24:585-96.  Back to cited text no. 4
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5.Greer RO Jr, Richardson JF. Clear-cell calcifying odontogenic tumor viewed relative to the Pindborg tumor. Oral Surg Oral Med Oral Pathol 1976;42:775-9.  Back to cited text no. 5
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6.Wallace J, MacDonald GD. Calcifying epithelial odontogenic tumour (Pindborg Tumour): a case report. Br J Plast Surg 1974;27:28-30.  Back to cited text no. 6
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7.Oikarinen VJ, Calonius PE, Meretoja J. Calcifying epithelial odontogenic tumor (Pindborg tumor) case report. Int J Oral Surg 1976;5:187-91.  Back to cited text no. 7
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8.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.  Back to cited text no. 8
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9.Asano M, Takahashi T, Kusama K, Iwase T, Hori M, Yamanoi H, et al. A variant of calcifying epithelial odontogenic tumor with Langerhans cells. J Oral Pathol Med 1990;19:430-4.  Back to cited text no. 9
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10.Schmidt-Westhausen A, Philipsen HP, Reichart PA. Clear cell calcifying epithelial odontogenic tumor. A case report. Int J Oral Maxillofac Surg 1992;21:47-9.  Back to cited text no. 10
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11.Takata T, Ogawa I, Miyauchi M, Ijuhin N, Nikai H, Fujita M. Non-calcifying Pindborg tumor with Langerhans cells. J Oral Pathol Med 1993;22:378-83.  Back to cited text no. 11
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12.Hicks MJ, Flaitz CM, Wong ME, McDaniel RK, Cagle PT. Clear cell variant of calcifying epithelial odontogenic tumor: case report and review of the literature. Head Neck 1994;16:272-7.  Back to cited text no. 12
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13.Kumamoto H, Sato I, Tateno H, Yokoyama J, Takahashi T, Ooya K. Clear cell variant of calcifying epithelial odontogenic tumor (CEOT) in the maxilla: report of a case with immunohistochemical and ultrastructural investigations. J Oral Pathol Med 1999;28:187-91.  Back to cited text no. 13
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14.Wertheimer FW, Zielinski RJ, Wesley RK. Extraosseous calcifying epithelial odontogenic tumor (Pindborg tumor). Int J Oral Surg 1977;6:266-9.  Back to cited text no. 14
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15.Ai-Ru L, Zhen L, Jian S. Calcifying epithelial odontogenic tumors: A clinicopathologic study of nine cases. J Oral Pathol 1982;11:399-406.  Back to cited text no. 15
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16.Houston GD, Fowler CB. Extraosseous calcifying epithelial odontogenic tumor: Report of two cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;83:577-83.  Back to cited text no. 16
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17.Orsini G, Favia G, Piattelli A. Peripheral clear cell calcifying epithelial odontogenic tumor. Report of a case. J Periodontol 2000;71:1177-80.  Back to cited text no. 17
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18.Rasmussen LG, Magnusson BC, Borrman H. The lateral periodontal cyst. A histopathological and radiographic study of 32 cases. Br J Oral Maxillofac Surg 1991;29:54-7.  Back to cited text no. 18
    
19.Philipsen HP, Reichart PA. Calcifying epithelial odontogenic tumour: Biological profile based on 181 cases from the literature. Oral Oncol 2000;36:17-26.  Back to cited text no. 19
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20.Anavi Y, Kaplan I, Citir M, Calderon S. Clear-cell variant of calcifying epithelial odontogenic tumor: Clinical and radiographic characteristics. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:332-9.  Back to cited text no. 20
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21.Kaplan I, Buchner A, Calderon S, Kaffe I. Radiological and clinical features of calcifying epithelial odontogenic tumour. Dentomaxillofac Radiol 2001;30:22-8.  Back to cited text no. 21
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22.Kramer IR, Pindborg JJ, Shear M. WHO histological typing of odontogenic tumours. Berlin: Springer-Verlag; 1992. p. 15-26.  Back to cited text no. 22
    
23.Richardson JF, Balogh K, Merk F, Booth D. Pigmented odontogenic tumor of jawbone. A previously undescribed expression of neoplastic potential. Cancer 1974;34:1244-51.  Back to cited text no. 23
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24.Waldron CA, Small IA, Silverman H. Clear cell ameloblastoma--an odontogenic carcinoma. J Oral Maxillofac Surg 1985;43:707-17.  Back to cited text no. 24
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


This article has been cited by
1 Clear cell variant of calcifying epithelial odontogenic tumor of maxilla: Report of a rare case
Badrashetty, D., Rangaswamy, S., Belgode, N.
Journal of Oral and Maxillofacial Pathology. 2013; 17(3): 479
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