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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 16  |  Issue : 2  |  Page : 105-108

Calcifying epithelial odontogenic tumor of the jaw: A report of two rare cases


1 Department of Oral Pathology and Microbiology, Institute of Dental Science Siksha O Anusandhan Deemed to be University, Bhubaneswar, Odisha, India
2 Department of Oral Medicine and Radiology, Institute of Dental Science Siksha O Anusandhan Deemed to be University, Bhubaneswar, Odisha, India
3 Medical Research Laboratory, IMS and SUM Hospital, Siksha O Anusandhan Deemed to be University, Bhubaneswar, Odisha, India
4 Nepalgunj Medical College, Nepalgunj, Nepal

Date of Submission30-Dec-2018
Date of Acceptance03-Apr-2019
Date of Web Publication19-Jun-2019

Correspondence Address:
Mahesh Chandra Sahu
Medical Research Laboratory, IMS and SUM Hospital, Siksha O Anusandhan Deemed to be University, Bhubaneswar, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/am.am_99_18

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  Abstract 


Calcifying epithelial odontogenic tumor (CEOT), also recognized as a Pindborg tumor, is a rare benign odontogenic neoplasm of epithelial derivation and is aggressive in the vicinity with a tendency to invade bone and neighboring soft tissues. It accounts for 1% of all the odontogenic tumors. Intraosseous CEOT is more common in comparison to extraosseous variant and is commonly situated at the posterior aspect of the mandible. Here, we report two cases of CEOT situated in the posterior aspect of mandible and both the cases were seen in female young adult age group. Partial mandibulectomy was performed in both the cases, and they were on regular follow-up. Both the cases were without any recurrence of tumor in 6 months of follow-up. Due to rarity of the case, we deliberate the radiologic features, histologic features, clinical presentation, and management of these two cases.

Keywords: Calcifying epithelial odontogenic tumor, mandible, partial mandibulectomy


How to cite this article:
Bhuyan SK, Bhuyan R, Sahu MC, Bhuyan S. Calcifying epithelial odontogenic tumor of the jaw: A report of two rare cases. Apollo Med 2019;16:105-8

How to cite this URL:
Bhuyan SK, Bhuyan R, Sahu MC, Bhuyan S. Calcifying epithelial odontogenic tumor of the jaw: A report of two rare cases. Apollo Med [serial online] 2019 [cited 2019 Dec 8];16:105-8. Available from: http://www.apollomedicine.org/text.asp?2019/16/2/105/260696




  Introduction Top


Odontogenic tumors are originated from epithelial, mesenchymal, or ectomesenchymal elements that have been a part of the tooth-forming apparatus. Calcifying epithelial odontogenic tumor (CEOT) also well known as Pindborg tumor is an uncommon benign epithelial neoplasm of odontogenic origin, characterized by deposition of amyloid-like material which later gets calcified.[1],[2] Thoma and Goldman reported the first case in 1946 as an “adenoid adamantoblastoma,” but the term CEOT was first described by Dr. Jens Jorgen Pindborg in 1955 and was generally accepted and adopted by the WHO in 1971.[2] Over half of the global population is concentrated in Asian countries, especially China and India, but the number of published series from those two countries is only limited in the recent past.[3],[4],[5],[6] Nearly, two hundred cases of CEOT have been reported in the literature.[7]

It is a neoplasm that is exclusively derived from the epithelial tissue, but there is no harmony on the originating cells. Different pathologists suggest different opinion regarding origin of CEOT. It may be derived from the stratum intermedium layer of the enamel organ in the tooth development stage. Furthermore, tumor may arise from remnants of the embryonic dental lamina found in the initial stage of odontogenesis.[8]

The tumor is commonly presented in the second-to-sixth decade of life with no gender predilection. The tumor is similar in behavior to ameloblastoma but has a lesser recurrence rate after surgery. Although sometimes locally invasive, it is a slow-growing neoplasm with recurrence rate of 10%–15% and rare malignant transformation. Timely diagnosis is essential to avoid maxillofacial distortion and destruction of vital structures.[9]

Here, we report two cases of CEOT of the mandibular posterior region in young females, and the characteristic clinical, radiological, and histopathological features are highlighted for both the cases.


  Case Reports Top


Case 1

A 29-year-old female patient reported to the Department of Oral Medicine and Radiology with the chief complaint of swelling in the left side of the face for 7 months. The swelling gradually increased in size to attain its present size and was not associated with pain or discharge from the swelling. The patient had undergone an extraction of decayed 36 few months back. Personal history revealed patient with no tobacco habits and had a good oral hygiene. The family and medical history was noncontributory. On extraoral examination, there was marked facial asymmetry on the left side of the face due to swelling measuring 3 cm × 2 cm × 3 cm in the mandibular region. On palpation, swelling was hard in consistency nontender, noncompressible, nonfluctuant, with loose skin over the swelling. There was no involvement of the lymph nodes. Intraorally, a diffuse swelling was present in 34, 35, 36 region, about 3 cm × 2 cm in diameter obliterating the gingivobuccal sulcus with buccal cortical plate expansion from 34,35 and lingual cortical expansion till 36 with elevated floor of the mouth. Overlying mucosa was normal in color and consistency. Mouth opening was slightly restricted. The swelling was mildly tender on palpation [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d.
Figure 1: (a) Clinical presentation of swelling at the right side of the mandible, (b) orthopantomogram showing radiolucent area with impacted tooth, (c) occlusal X-ray showing flecks of calcification, and (d) three-dimensional computed tomography scan showing tumor demarcations

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Radiological examination with intraoral periapical radiograph revealed 34, 35, 36 region with mixed radiopaque radiolucent lesions with loss of lamina dura and altered bony trabeculare. Mandibular occlusal radiograph of the left side depicted localized mixed radiolucency and radiopacity in 34, 35, 36 region, missing 36 with irregular bony trabeculae traversing in the radiolucency and with prominent buccal and lingual cortical plate expansion. Panoramic radiograph depicted a localized well-defined mixed radiolucency and radiopacity surrounded by corticated border in the left body of the mandible in 34, 35, 36, 37 regions. Radiopacity more prominent coronal to 36, reflective of driven snow appearance with displacement of 35, along with root resorption in 34, 35 and apical displacement of inferior alveolar nerve canal, the bony trabeculae being indistinct within the lesion [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d. Incisional biopsy was performed from the growth and histopathological examination revealed sheets of polyhedral epitheloid cells in the background of scanty collagenous computed tomography (CT) stroma. Few epitheloid cells show pleomorphism and hyperchromatism. Cells showed prominent intercellular bridges. Areas of dystrophic calcification and Liesegang rings were present [Figure 2]a and [Figure 2]b. With the above features, a diagnosis of CEOT was concluded. The patient was managed by partial hemimandibulectomy on the left side with fibular reconstruction and was on under regular follow-up with no recurrence reported for the last 6 months.
Figure 2: Photomicrograph (H and E stain) showing (a) uniform distribution of polyhedral cells with areas of calcifications (×10) and (b) hyperchromatic and pleomorphic nuclei (×40)

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Case 2

A 24-year-old female patient reported to the Department of Oral Medicine and Radiology with the chief complaint of swelling in the right lower jaw for 5 months. The swelling gradually increased in size and was not associated with pain or any discharges. On extraoral examination, a diffused ovoid swelling was present on the right side of the face measuring 4 cm × 4 cm in size. On palpation, the swelling was hard in consistency and nontender. Intraorally, diffuse swelling present extending from 43 to 46 regions, ovoid in shape, and involving the alveolobuccal sulcus measuring 5 cm × 4 cm in size, with no secondary changes. On palpation, the swelling was hard in consistency, noncompressible, nonreducible, and nontender. Expansion of the buccal cortical plates, the lingual plates being intact. There was over retained deciduous teeth 85 and 45 was missing [Figure 3]a,[Figure 3]b,[Figure 3]c.
Figure 3: (a) Intraoral presentation of the tumor on the left side of the mandible, (b) the cortical plate expansion with areas of calcifications on occlusal X-ray, and (c) well-defined radiolucency with radio-opaque border with impacted tooth on orthopantomogram

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Radiological examination with intraoral periapical radiograph revealed a well-defined mixed radiopaque-radiolucent lesion, involving the periapical region in relation to 43, 44, 85, 46, measuring 5 cm × 5 cm in size, having well-defined radiopaque corticated borders, and streaks of radiopacity present within the radiolucency. Marked root resorption was observed with 85 and 46. Orthopantomograph revealed a well-circumscribed mixed radiopaque-radiolucent lesion, involving the right mandibular ramus in relation to 44, 85, 46, measuring 5 cm × 5 cm in size, having well-defined radiopaque corticated borders, with the presence of radiopaque mass in the center of the lesion, and radiopaque streaks extending to periphery, suggestive of driven-snow appearance. There is apical displacement of teeth in relation to 45 with the involvement of inferior border of mandible and root resorption in 85, 46. Mandibular occlusal radiograph revealed expansion of the buccal cortical plates in relation to 43, 44, 85, 46, with mild lingual plate expansion in relation with 46. CT image revealed an oval expansile lytic lesion involving right mandibular ramus having internal coarse clumped calcifications and isoattenuating soft-tissue compartment measuring 5 cm × 5.7 cm. Outer scalloped well-corticated rim maintained except a small defect anteriorly (4 mm), suggestive of a lytic expansile mandibular lesion with smooth corticated border [Figure 4]a and [Figure 4]b. Incisional biopsy was performed for histopathological analysis which showed sheets of polyhedral epitheloid cells in background of scanty collagenous CT stroma. Few epitheloid cells showed pleomorphism and hyperchromatism. Areas of dystrophic calcification and Leisgang rings were present. All the above pathological features concluded a diagnosis of CEOT [Figure 2]a. Right side partial hemimandibulectomy with fibular reconstruction was performed and was on regular follow-up, with no recurrence reported for the last 12 months.
Figure 4: (a) Intraoperative tumor finding and (b) uniform distribution of polyhedral cells with amyloid rings on photomicrograph (H and E stain: ×10)

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  Discussion Top


CEOT is a rare, benign, but locally aggressive odontogenic tumor and accounts for 1% of all odontogenic tumors.[1],[7] The epithelial cells of CEOT are found to be reminiscent of the sequestered cells from the stratum intermedium layer of the enamel organ. This impression has been achieved as per the morphological similarity of the tumor cells to the normal cells of stratum intermedium and also there is a high activity of alkaline phosphatase and adenosine triphosphate observed.[1],[5] Amyloid deposition within CEOT is the result of immunologic reaction to these stratum intermedium cells. Others claim that it ascends from remnants of the primitive dental lamina found in the initial stage of odontogenesis, and these epithelial rests are probably true progenitor cell.[1],[10]

CEOT usually appears during the mean age of 40 years, and there is no gender predilection, but both of our cases are females.[1] It is categorized as intraosseous or central CEOT and extraosseous or peripheral CEOT; intraosseous lesion being the most common accounting for 94% of cases and 6% cases are extraosseous variant. Intraosseous CEOT is commonly located in the posterior mandible, that is, in the premolar/molar region, whereas extraosseous lesions are commonly occurs on the anterior gingival.[11] An association of impacted tooth is found in most of the intraosseous CEOT which is reported in both of our cases.[1] An asymptomatic slowly enlarging mass of the affected jaw is the typical clinical presentation of CEOT. The lesion may exhibit mild to moderate invasiveness resulting bony swelling with cortical expansion, tooth movement, and root resorption, and finally perforation.[7],[11] Both of our cases are intraosseous tumor located in the posterior mandible and are asymptomatic, but locally aggressive, showing cotical bone resoption without perforation of bone.

Depending on the different stage of development of the lesion, radiographic appearance of CEOT may vary from a well-defined radiolucency to mixed radiolucent-radiopaque and completely radiopaque mass.[11] In the initial stage, it may appear as dentigerous cyst because of its relation with impacted tooth and is completely radiolucent.[10] The mixed radiolucent-radiopaque appearance of the lesion may be present having many small irregular trabeculae passing through the radiolucent area and “driven-snow” appearance results due to scattered flecks of intratumoral calcification within the radiolucency which is the characteristic of CEOT but not diagnostic. The margins are usually well defined or sclerotic as contemporaneous in our cases.[6],[11] The radiographic findings in both of our cases also showed classical “driven-snow” appearance.

The diagnosis of CEOT is based on histological examination and revealed the presence of various amounts of proliferated polyhedral cells arranged in masses, sheets, and islands surrounded by a scanty connective tissue stroma. These cells consist of abundant eosinophilic, finely granular cytoplasm with nuclear pleomorphism, anisonucleosis, and hyperchromatism along with prominent intercellular junctions. Eosinophilic “amyloid-like” materials are deposited extracellularly and gradually become concentric calcified deposits, known as “Leisegang rings,” which is the pathognomonic for CEOT. Such amyloid materials are positive for Congo red stain and reveal an apple green birefringence under a polarized microscope.[2],[5],[7] Our cases also have classic “Leisegang ring” in one case and amyloid materials in another case.

Well-defined, small, intrabony mandibular lesions are usually treated by simple enucleation or curettage with judicious removal of a thin layer of bone adjacent to the tumor. Whereas, large tumors require aggressive approaches such as segmental resection, hemimandibulectomy, and hemimaxillectomy, followed by reconstruction with grafting or distraction osteogenesis.[6] However, the lesions treated with conservative approach show a high recurrence rate ranging from 15% to 30%. Therefore, a resection using 1.0–1.5 cm margins in bone should be done in CEOT.[11] Both of the present cases are managed by partial hemimandibulectomy, followed by full reconstruction with fibula and they are under regular follow-up with no recurrence reported till date. Malignant transformation of CEOT is extremely rare with only few cases have been reported.[2],[4] The frequency of malignant tumors is more in Eastern Asia (6.3%) than elsewhere, including one Tamil Nadu, Indian study (range 0.5%–2.48%).[3],[4],[5],[12],[13],[14],[15] It was found to occur more in female patients and in the posterior mandible. The female predilection is in contrast to other reports.[3],[4],[12],[16]


  Conclusions Top


Pindborg tumor is rare a benign infiltrative odontogenic tumor with local expansion. Vital resection of the entire mass with tumor-free surgical margins is the ideal treatment as tumor will recur if not completely removed and long term follow-up is recommended.

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.



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



 

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