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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 29  |  Issue : 4  |  Page : 333-336

Maxillary Calcifying Epithelial Odontogenic Tumor Presenting at a Postextraction Site: A Case Report and Brief Review of Literature


1 Department of Oral Medicine and Radiology, Meghna Institute of Dental Sciences, Nizamabad, Telangana, India
2 Department of Oral and Maxillofacial Pathology, Meghna Institute of Dental Sciences, Nizamabad, Telangana, India
3 Department of Oral and Maxillofacial Surgery, Venkateswara Dental College and Hospital, Chennai, Tamil Nadu, India

Date of Submission28-Mar-2017
Date of Acceptance28-Jan-2018
Date of Web Publication15-Feb-2018

Correspondence Address:
Dr. Talla Harshavardhan
Department of Oral Medicine and Radiology, Meghna Institute of Dental Sciences, Nizamabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_35_17

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   Abstract 


Calcifying epithelial odontogenic tumor (CEOT) was described as an entity by Danish pathologist Jens J. Pindborg in 1955. It is a rare benign odontogenic neoplasm forming 0.4–3.0% of all intraosseous tumors. Also known as Pindborg tumor, it is traditionally described as an uncommon, benign neoplasm of exclusively epithelial tissue of origin. Much of controversy exists in the nature of origin of this tumor; there is no consensus on the originating cells of CEOT. Some pathologists suggest that it is derived from stratum intermedium layer of enamel organ in the tooth development stage; others favor a different hypothesis in which this tumor may arise from the remnants of primitive dental lamina in the initial stages of odontogenesis. Most of the cases reported involve the posterior mandible than the maxilla in the ratio of 3:1. The mean age is 30–40 years with no gender predilection. The present case throws highlight on the unusual site of the lesion, i.e. presenting in the maxillary molar region as an ulcerated area in the area of extraction socket. The study also attempts to briefly emphasize the concepts of CEOT and also revives the review of literature.

Keywords: Calcifying epithelial odontogenic tumour, dental lamina, odontogenesis


How to cite this article:
Harshavardhan T, Naag S, Umamagesh DP, Priyadarshini E. Maxillary Calcifying Epithelial Odontogenic Tumor Presenting at a Postextraction Site: A Case Report and Brief Review of Literature. J Indian Acad Oral Med Radiol 2017;29:333-6

How to cite this URL:
Harshavardhan T, Naag S, Umamagesh DP, Priyadarshini E. Maxillary Calcifying Epithelial Odontogenic Tumor Presenting at a Postextraction Site: A Case Report and Brief Review of Literature. J Indian Acad Oral Med Radiol [serial online] 2017 [cited 2019 Aug 21];29:333-6. Available from: http://www.jiaomr.in/text.asp?2017/29/4/333/225555




   Introduction Top


The calcifying epithelial odontogenic tumor (CEOT) was first introduced into scientific literature almost 50 years ago by late Dr. Jens J. Pindborg. As a mark of honor to this pathologist, the maxillofacial pathologists use the term Pindborg tumor as a synonym to this entity. It is defined as a benign, uncommon, slow-growing odontogenic tumor that is exclusively of epithelial origin.[1],[2] It has a wide age incidence from 35 to 60 years with mean of 38 years. It usually presents as a hard, painless mass, generally affecting the mandible.[2] Few sporadic cases affecting the maxilla have also been reported. Although its biological behavior is indolent, the maxillary tumors tend to grow rapidly and are locally invasive.[3] Histopathology shows sheets and islands of epithelial cells with marked intercellular bridges. The nuclei are hyperchromatic and bizarre. Pale eosinophilic masses such as amyloid and Liesegang's rings are pathognomonic of this lesion.[1] Treatment is surgical resection with clear margins. A 14% recurrence rate is seen on longstanding cases. The prognosis is considered to be very favorable in most of the cases.[3]


   Case Report Top


A 30-year-old male patient reported to our ward with a complaint of painless swelling in the right posterior maxilla since 2 months. Patient gave a history of extraction of his upper right first and second molars due to pain and mobility. Postextraction, after 2 months duration, patient noticed a swelling that progressively increased to the present size. Examination revealed a swelling in relation to right posterior maxilla of 5 × 5 cm in size extending buccopalatally, with red, indurated, ulcerative margins at the site of extraction. There was no dry socket or any other postextraction complication seen. Due to large size of the lesion, indentations of mandibular teeth were discernable on the lesional area [Figure 1]. Orthopantomograph revealed a well-defined mixed radiopaque-radiolucent lesion associated with an impacted maxillary third molar at the infraorbital margin [Figure 2]. Provisional diagnosis of fibro osseous lesion and a differential diagnosis of dentigerous cyst and CEOT was made. Incisional biopsy showed features consistent with CEOT.
Figure 1: Clinical presentation with mild ulcerated margins and intraoral swelling in the upper right molar region; indentations also observed on the swelling

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Figure 2: Orthopantomograph revealing an impacted third molar at the infraorbital region, healed extraction sockets in the molar region, and radiopaque-lucent areas in the region of third molar

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Excision of the lesion was done under local anesthesia. Buccal pad of fat was harvested into the defect and sutured to the palatal periosteum, after the tumor was successfully excised [Figure 3]. Histopathology showed discrete sheets of polyhedral cells, eosinophilic and hyalinized materials with concentric rings (Liesegang's rings). All the findings confirmed the diagnosis of CEOT [Figure 4] and [Figure 5]. One year follow-up of the patient showed no recurrences, and healing of the biopsied site was uneventful. Due to financial constraints of the patient, no advanced diagnostic aids were advised.
Figure 3: Excision of the lesion completely under local anesthesia

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Figure 4: Low power view of the lesion showing fibrous stroma with island of epithelial cells and periphery showing calcified area and eosinophilic amyloid-like areas

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Figure 5: Epithelial island showing cells with hyperchromatic nuclei, prominent intercellular bridges, and polyhedral cell outlines

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   Discussion Top


CEOT, most commonly known as Pindborg tumor, is a benign but rare odontogenic tumor making up <1% of all odontogenic neoplasms. The CEOT is most commonly reported in the fourth to sixth decade of life with a mean of 37–43 years of age.[2] There does not appear to be a gender predilection for this lesion. Like ameloblastoma, the CEOT presents most often as an intrabony mandibular lesion, usually involving the ascending ramus region.[2] Franklin and Pindborg reviewed 113 cases in a review and found that tumor favors the mandible than maxilla in a ratio of 3:1.[2] There have been very few reported cases in the maxilla till date and posterior maxilla being the rarest site. To the best of our knowledge, on literature search we could not find any reported cases showing the occurrence in the posterior maxilla. This case adds to a small number of Pindborg tumor cases located in the maxilla. Three distinctive features of the present case are: a rare localization of the lesion, presentation as an ulcerated mass, and a comparatively young age of patient. Nascimento et al.[3] reported a case in the maxilla with significant amyloid-like deposit and sparse Liesegang rings. Both these components were significantly observed in the present case. Bridle et al.[4] reported an unusual case in the maxilla with abnormal eye sign, but in the present case there was no eye involvement. Anisha Maria et al.[5] reported a case in the maxilla of a 25-year-old male who underwent extraction of the molar tooth assuming the tooth to be the cause of swelling. Although CEOT is a benign tumour, it has variable biologic behavior ranging from mild to moderate invasiveness. The tumor grows by infiltration and produces cortical expansion and root resorption. Very rarely do intraosseous CEOT present as ulcerated masses except for a case reported by Hiroyuki Nakamo et al.[6]

The peripheral CEOT is smaller than 2 cm and arises from gingival or edentulous alveolar mucosa, generally in the anterior region, and painless and firm mass. The peripheral CEOT shows a definitive sex predilection of male in 2:1 ratio. They are less aggressive clinically than central ones.[1] The present case is one of its kind as the lesion appears in the posterior-most region of maxilla, presenting as an ulcerated surface mucosa in a young male patient who has undergone extraction of his molars due to pain and mobility. The lesion was large measuring 5 × 5 cm buccopalatally. No sinus involvement or nerve paresthesia was observed. The histopathology of the present case showed discrete epithelial sheets of cells with prominent intercellular bridges, pale eosinophilic bodies which are amyloid-like in nature. The stroma also showed lamellar concentric bodies of calcification called the Liesegang's rings. There was no bizarre mitotic figures except for hyperchromatic nuclei.

Radiographic presentation

An early tumor may be completely radiolucent. As the tumor matures and becomes larger, it becomes radiolucent-opaque, although they can show only radiolucency. The spectrum can range from unilocular radiolucency to multilocular “soap bubble” appearance.[1],[2],[4] Most are associated with impacted tooth. Characteristically, multiple radiopacities of varying size within radiolucent areas and extensive areas of calcifications causing them to become radiopaque are also seen. This form of presentation is described by Pindborg as “wind-driven snow” pattern.[2],[5] Peripheral tumors are typically radiolucent with bone showing superficial erosive pattern. The computed tomography and magnetic resonance imaging (MRI) are not of primary diagnostic importance.[1],[2] However, to be able to see the internal structures of the lesion and its involvement with neighboring structures gives useful information. Plain films are unreliable in detecting the degree of cortical bone destruction. MRI shows hypotense lesion with T1-weighted images and hypertense images with T2 images.[1],[2],[4]

Histopathology

According to Pindborg, the characteristic histologic criteria for diagnosis of CEOT are sheets of large polygonal epithelial cells that have well-defined borders and show prominent intercellular bridges. Nuclear pleomorphism varied with size, shape, and number. Cellular abnormalities and mitotic figures are rarely seen. Cytoplasm is abundant and eosinophilic. Varying amounts of extracellular amyloid-like material that stains Congo red positive in polarized light. Concentric calcified masses of Liesegang's rings calcification pattern are pathognomonic for this tumor.[1],[2],[3],[4],[5] Ai-Ru et al. proposed a subclassification into four patterns. Two or more patterns can coexist in the same tumor. The patterns are as follows: [5]

Pattern I: Polyhedral epithelial cells arrangements with deeply eosinophilic cytoplasm and prominent nuclei, distinct cell outlines and intercellular bridges, frequent cell abnormalities, no mitotic figures and calcifications are seen in the fibrous stroma.

Pattern II: Cribriform appearance of the cell arrangements, intercellular bridges might be discrete, few cell abnormalities, and calcification of the eosinophilic material filling cribriform spaces with Liesegang calcification pattern.

Pattern III: Scattered or dense arrangements of the epithelial cells with varying size and multinucleated giant cells are frequent; mucoid material is present in the stroma.

Pattern IV: Nests and cords of epithelial cells containing an abundant eosinophilic cytoplasm or a clear vacuolated cytoplasm, variable amounts of stroma with eosinophilic material and calcifications.

As mentioned, the amyloid-like substances are round, pale eosinophilic masses within the sheets of tumor cells, and because of its affinity with mineral salts it can undergo calcification, often having the concentric rings called Liesegang's rings.[1] Calcium salts are often deposited within these areas. The eosinophilic material shows apple-green birefringence under polarized light with Congo red. It also stains positive for crystal violet and Thioflavine.[1],[2]

There is some controversy as to whether the homogenous substance is a degeneration product or is actively secreted and whether it is extracellular or intracellular in origin.[1] The amyloid in Pindborg tumor is probably derived from degradation of lamina densa material secreted by tumor epithelial cells. There is controversy as to whether the homogenous substance is a degeneration product or is actively secreted, and whether it is extracellular or intracellular in origin. The amyloid in Pindborg tumor is probably derived from degeneration of lamina densa material, secreted by the tumor epithelial cells.[1],[2] An ultrastructural study of eosinophilic masses has showed two types of structures that are related. The first type appears as sheets of fine filaments measuring 10–12 nm in diameter.[1] The second type is in the form of aggregates of lamina densa fragments, probably secreted by tumor epithelium. These fragments appear to undergo some loss of electron density and become degraded into fine filaments, having similar thickness and electron density to those forming the filamentous masses. It is concluded that the fine filamentous material is a form of amyloid which results from degeneration of lamina densa material.[1],[2]

Treatment and prognosis

The CEOT has variable biologic behavior ranging from very mild to moderate invasiveness. The literature has varied regarding its treatment because it is an uncommon and slow-growing tumor and follow-up is often lost or only minimal. The CEOT is managed surgically by removal of the tumor with a border of clinically and radiographically normal bone. A margin of about 1 cm is adequate.[1],[3],[4],[5]

An overall recurrence rate of 14% is noted. Those treated with resection approaches have few recurrences. Therefore, CEOT is best treated with a resection using 1–1.5 cm clearance. A minimum of 5 years to maximum of 10 years may be needed for recurrence of this tumor.[1],[4],[5] In the present case, there was surgical resection of the tumor margin by an intraoral approach, 1 cm healthy bone was achieved. The healing was uneventful and there was no recurrence seen after 9 months of follow-up.


   Conclusion Top


Since the first review in 1966, the number of publications of CEOT has increased from 26 to the present little over 200 cases. This case report is presented with a view of rare involvement of maxilla by the tumor. In our case the tooth was embedded close to the infraorbital margin, which on initial appearance gave a dentigerous cyst appearance. The lesion was noticed due to a nonhealing socket of 2 months duration with a history of extraction. This study emphasizes the need to correlate the clinical, radiological, and histopathological areas to arrive at appropriate diagnosis and treatment planning.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Patino B, Fernandez J, Garcia-Rozado A, Martin R, Luis Lopez-Cedrun J, Sanroman B. Calcifying epithelial odontogenic (Pindborg) tumour: A series of 4 distinctive cases and review of the literature. J Oral Maxillofac Surg 2005;63:1361-8.  Back to cited text no. 1
    
2.
Goode RK. Calcifying epithelial odontogenic tumor. Oral Maxillofac Surg Clin North Am 2004;16:323-31.  Back to cited text no. 2
[PUBMED]    
3.
Nascimento GJF, Pereira KMA, Nonaka CFW, Medeiros AMC, Galvao HC. Calcifying epithelial odontogenic tumor of the maxilla. Braz J Otorhinilaryngol 2009;75:468.  Back to cited text no. 3
    
4.
Bridle C, Visram K, Piper K, Ali N. Maxillary calcifying epithelial odontogenic (Pindborg) tumor presenting with abnormal eye signs: Case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;102:e12-5.  Back to cited text no. 4
[PUBMED]    
5.
Maria A, Sharma Y, Malik M. Calcifying epithelial odontogenic tumour: A case report. J Maxillofac Oral Surg 2010;9:302-6.  Back to cited text no. 5
[PUBMED]    
6.
Nakano H, Ota Y, Yura Y. Calcifying epithelial odontogenic tumor of the maxilla with ulcerative stomatitis: A case report. Br J Oral Maxillofac Surg 2009;47:222-4.  Back to cited text no. 6
[PUBMED]    


    Figures

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



 

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