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CORRESPONDENCE
Year : 2014  |  Volume : 10  |  Issue : 1  |  Page : 194-196

Calcifying epithelial odontogenic tumor: A clinico-radio-pathological dilemma


1 Department of Head and Neck Surgical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
2 Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India
3 Department of Radiology, Tata Memorial Hospital, Mumbai, Maharashtra, India

Date of Web Publication23-Apr-2014

Correspondence Address:
Prathamesh S Pai
Department of Head and Neck Surgical Oncology, Tata Memorial Hospital, Ernest Borges Road, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.131411

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 > Abstract 

The calcifying epithelial odontogenic tumor (CEOT) is a rare benign neoplasm of mandible in adults. The presentation of this entity is varied and often confused with a variety of mucosal and jaw lesions and clinical, radiological, and pathological feature of CEOT often-mimic malignancy. The objective of this report is to highlight the clinical features and radiological findings which should arouse suspicion of a benign lesion and importance of providing adequate clinical information to the pathologist to attain accurate diagnosis.We discussed two cases with tumors located in the maxilla. Both presented as expansile lesions with one biopsy proven squamous cell carcinoma. Both were pursued with clinico-radiological suspicion of benign lesions and confirmed with pathological correlation of histology and immunohistochemistry as CEOT. Therefore a High index of suspicion and clinico-radiological information are the key feature for diagnosis of this rare tumor.

 > Abstract in Chinese 

牙源性钙化上皮瘤:临床放射病理学的困境
钙化牙源性上皮性肿瘤(CEOT)是一种罕见的成人下颌骨良性肿瘤。该实体的表现是多种多样的,经常与各种黏膜和颌骨病变混淆,其临床,放射学和病理学特征经常模仿恶性肿瘤。本报告的目的是为了强调其临床特点和放射学发现,根据这些特点和发现应该怀疑良性病变,以及为病理学家的准确诊断提供足够的临床信息。我们讨论了位于上颌骨的肿瘤病例2例。均表现为膨胀性病变,活检证实鳞状细胞癌。两人在临床影像学上都怀疑是良性病变,但病理相关组织学和免疫组化证实为牙源性钙化上皮瘤。因此高度怀疑和临床放射学信息是这种罕见的肿瘤诊断的关键。
关键词:牙源性钙化上皮瘤,颌骨瘤,牙源性肿瘤,Pindborg瘤


Keywords: Calcifying epithelial odontogenic tumor, jaw tumor, odontogenic tumors, pindborg tumor


How to cite this article:
Hada M S, Sable M, Kane S V, Pai PS, Juvekar S L. Calcifying epithelial odontogenic tumor: A clinico-radio-pathological dilemma. J Can Res Ther 2014;10:194-6

How to cite this URL:
Hada M S, Sable M, Kane S V, Pai PS, Juvekar S L. Calcifying epithelial odontogenic tumor: A clinico-radio-pathological dilemma. J Can Res Ther [serial online] 2014 [cited 2019 Sep 17];10:194-6. Available from: http://www.cancerjournal.net/text.asp?2014/10/1/194/131411


 > Introduction Top


The calcifying epithelial odontogenic tumor (CEOT) is rare (1%), first described by Pindborg in 1955. [1] CEOT occurs as single, painless, gingival mass resembling oral hyperplastic mucosal lesion. [2] The peripheral location within mandible suggests histogenesis from dental lamina or basal cells of oral epithelium. [1],[3] CEOT's are radiographically characterized as uni or multilocular radiolucent areas with patchy radio-opacity and are histologically composed of polyhedral odontogenic epithelial cell cords, interspersed with extracellular amyloid. [1],[2],[4]

CEOT have varied presentation and are often confused with a variety of lesions including malignancy. Two patients presented to our tertiary cancer care center as maxillary cancers and were eventually diagnosed to have CEOT. The objective of this report is to highlight clinical and radiological features of CEOT and importance of clinical information in aiding pathological diagnosis.

Case 1

A 35-year-old male presented with ulcero-proliferative upper alveolar 5 cm × 4 cm mass with free overlying skin, since 7 months [Figure 1]. Computed tomography (CT) Scan revealed a heterogeneously enhancing hypodense, well circumscribed, expansile soft tissue mass surrounded by a thin rim of expanded and focally eroded bone with scattered flecks of calcification [Figure 2]. Radiological differential diagnosis was between ossifying fibroma, ameloblastoma, and giant cell tumor. Clinical information (tumor location, radiological features, and clinical suspicion) were given to the pathologist. In view of tumor location, morphology, and immunohistochemistry (IHC) (expressed cytokeratin (CK), p63, and focally calponin), a diagnosis was given in favor of CEOT with differential diagnosis of myoepithelial neoplasm of salivary gland. Patient underwent orbital plate preserving maxillectomy. Patient is doing well on regular follow up of over 18 months.
Figure 1: Clinical picture showing submucosal lesion left upper alveolus

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Figure 2: CT scan axial and coronal section showing expansile lesion bulging into maxillary floor, scattered flecks of calcification through radiolucent area of calcifications

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Case 2

A 67-year-old male, tobacco user presented with 4 cm × 3 cm submucosal painless upper alveolar swelling since 8 months with biopsy reported as squamous cell carcinoma. CT Scan showed an expansile destructive intraosseous lesion with epicentre in the maxillary alveolus. In view of long standing history, slow progression this lesion was clinically benign but a possibility of metastatic lesion was also considered. Review of the biopsy was CEOT. These highlighted the importance of having a high clinical suspicion against malignancy when dealing with large expansile lesions in relation to molars, minimal bone destruction or with bone remodeling and long standing history.

Pathological features

Case 1: Under low power alternate areas of hyper and hypocellularity were seen. The tumor cells were arranged in nests, strands, and acini in hypercellular areas and were basal like showing hyperchromatin nuclei and scanty cytoplasm. Cells in the hypercellular areas appeared stellate-shaped showing loose streaming pattern with abundant rounded amyloid-like deposits. Cellular pleomorphism and multinucleation were lacking. Also noted were focal tubular pattern lined by cuboidal cells and Liesegang's rings. Basal like cells were positive for p63 [Figure 3].
Figure 3: (a) Tumor cells arranged in nests, acini and strands (H and E, ×4). (b) Tubular pattern (H and E, ×20). (c) Liesegang's rings (H and E, ×20). (d) p63 positivity in tumor cells (IHC, ×20)

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Case 2: Tumor was composed of islands of closely packed large polyhedral epithelial cells in a fibrous stroma. The tumor cells showed a well-defined border with intracellular bridges with central hyperchromatic and pleomorphic nuclei. Sudden nucleomegaly, multinucleation, and smudged chromatin were noted. The cytoplasm was abundant granular and eosinophilic. On closer inspection, the stroma showed amyloid-like material. Keratin pearls were absent. Mitotic activity was lacking [Figure 4]. Though tumor did have superficial resemblance to Squamous-cell carcinoma (SCC), mitotic activity, nuclear pleomorphism, and multinucleation was not matching and the presence of amyloid along with clinico-radiological correlation clinched the diagnosis.
Figure 4: (a) Tumor composed of islands of large polyhedral epithelial cells in a fibrous stroma (H and E, ×4). (b and c) stroma of tumor shows extracellular amyloid-like material. (d) Congo red stain does not demonstrate amyloid

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 > Discussion Top


Jaw tumors are common in India with rampant abuse of tobacco and areca nut. Oral cancers accounting for 30% of cancer burden in India, it is not surprising to consider a patient presenting with a jaw lesion as malignancy. Submucosal lesions and indolent growth should arouse suspicion of benign diseases.

Pindborg [5] considered CEOT to be odontogenic in origin. CEOT are either intraosseous or extraosseous, with intraosseous involving the mandible twice as commonly as compared to maxilla and predominantly in premolar and molar region. [4] Both our patients had intraosseous lesions involving the upper alveolus in molar region. Extraosseous frequently occur in the anterior gingival. [6] Pathologists have high index of suspicion for odontogenic tumor when they occur in mandible, while minor salivary gland tumors are commonly suspected in the maxilla.

Radiologically, intraosseous lesions are well circumscribed, surrounded by thin rim of expanded bone, unerupted teeth, and scattered flecks of calcification. Similar appearing lesions are odontomas, which have an amorphus appearance with multiple discrete foci of calcification, ossifying fibromas, which have more calcification, while fibrous dysplasias are mainly homogenous and expanded lesions.

Histomorphologically, CEOT is composed of two distinct cell types, large polyhedral and small basal-like. The large cells derived from intermediate squamous cells show degenerative changes like smudged chromatin, multinucleation, and sudden nucleomegaly. In contrast, basal cells are smaller, have scanty cytoplasm, and can proliferate to form various pattern like nests, cords, or acini.

When large polyhedral cells predominate, they give squamoid appearance with amyloid-like stroma, and calcification similar to keratin arousing suspicion of squamous carcinoma (Case 2). When basal cells predominate, the differential diagnosis includes minor salivary gland tumor (Case 1). Case 1 also mimicked myoepithelial cell tumor with the amyloid-like stroma being mistaken for hyaline stroma. Characteristic Liesegang rings in and around epithelial cells, however, offered diagnostic clue. IHC can play important role in diagnosis as in Case 2, despite a diagnosis of malignancy, it is advisable to review with IHC to know the exact nature of the lesion which will go a long way towards patient counseling and treatment plan.

When CEOT is predominantly composed of stellate cells and basal cells, the differentials includes ameloblastoma and minor salivary gland tumor. Ameloblastomas by and large show strong and diffuse positivity for CK19 and Calretinin; whereas majority of carcinoma of salivary gland origin show strong positivity for CK7 and carcinoembryonic antigen (CEA). Pure myoepithelial tumor are CK7 negative and express p63, high molecular weight cytokeratin (HMW CK), Calponin, and CD10. CEOT can be distinguished on IHC from above mentioned tumor as it lacks expression for calretinin and CK7.

When CEOT shows polyhedral epithelial cells with abundant eosinophilic cytoplasm and significant pleomorphism, it closely resembles squamous carcinoma, however, strong and diffuse positivity for CK5/6 and p63on IHC, as seen in squamous carcinoma, facilitates the distinction from CEOT.

Surgical management of mandibular lesions ranges from simple enucleation [6] to hemimandibulectomy. However, treatment of maxillary lesions is usually maxillectomy, [4] since they grow rapidly, occur in vicinity of important structures (orbit, skull base) and do not remain confined. Recurrence (14%) occurs more frequently in maxilla than mandible. [7] There is a low risk of malignant transformation with four reported cases. [7],[8],[9],[10]


 > Conclusion Top


CEOT is a rare jaw tumor, masquerading as malignancy, especially when located in the maxilla. Pathological dilemma makes it a diagnostic pitfall. High index of suspicion and clinico-radiological correlation are the key features in diagnosis. Wide excision with tumor-free margin is treatment of choice to avoid recurrence.

 
 > References Top

1.Pindborg JJ. A calcifying epithelial odontogenic tumor. Cancer 1958;11:838-43.  Back to cited text no. 1
[PUBMED]    
2.Takata TS, Slootweg PJ. Calcifying epithelial odontogenic tumor. In: Barnes LE, Eveson JW, Reichart P, Sidransky D, editors. Pathology and Genetics of Head and Neck Tumors. 1 st ed. Lyon: WHO IARC Press; 2005. p. 302-3.  Back to cited text no. 2
    
3.Takeda Y, Suzuki A, Sekiyama S. Peripheral calcifying epithelial odontogenic tumor. Oral Surg Oral Med Oral Pathol 1983;56:71-5.  Back to cited text no. 3
[PUBMED]    
4.Philipsen HP, Reichart PA. Calcifying epithelial odontogenic tumor: Biological profile based on 181 cases from the literature. Oral Oncol 2000;36:17-26.  Back to cited text no. 4
    
5.Pindborg JJ, Kramer IR. Histological typing of odontogenic tumors, jaw cysts, and allied lesions. Geneva: World Health Organization; 1971.  Back to cited text no. 5
    
6.Vap DR, Dahlin DC, Turlington EG. Pindborg tumor: Theso-called calcifying epithelial odontogenic tumor. Cancer 1970;25:629-36.  Back to cited text no. 6
[PUBMED]    
7.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.  Back to cited text no. 7
[PUBMED]    
8.Veness MJ, Morgan G, Collins AP, Walker DM. Calcifying epithelial odontogenic (Pindborg) tumor with malignant transformation and metastatic spread. Head Neck 2001;23:692-6.  Back to cited text no. 8
    
9.Kawano K, Ono K, Yada N, Takahashi Y, Kashima K, Yokoyama S, et al. Malignant calcifying epithelial odontogenic tumor of the mandible: Report of a case with pulmonary metastasis showing remarkable response to platinum derivatives. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:76-81.  Back to cited text no. 9
    
10.Basu MK, Matthews JB, Sear AJ, Browne RM. Calcifying epithelial odontogenic tumor: A case showing features of malignancy. J Oral Pathol 1984;13:310-9.  Back to cited text no. 10
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