[Three-dimensional radiographic features of calcifying odontogenic cyst and calcifying epithelial odontogenic tumor].

OBJECTIVE: To analyze the three-dimensional radiographic characteristics of calcifying odontogenic cyst and calcifying epithelial odontogenic tumor using spiral computed tomography (CT) and cone-beam computed tomography (CBCT). METHODS: Clinical records, histopathological reports, and CBCT or non-enhanced spiral CT images of 19 consecutive patients with calcifying odontogenic cyst (COC) and 16 consecutive patients with calcifying epithelial odontogenic tumor (CEOT) were retrospectively acquired, and radiographic features, including location, size, expansion, internal structure and calcification, were analyzed. RESULTS: Among the 19 COC cases (12 males and 7 females, with an average age of 27 years), 89.5% (17/19) of the lesions originated from the anterior and premolar areas, 100.0% of them exhibited cortex expansion, and 78.9% had discontinued cortex. Among the 16 CEOT cases (3 males and 13 females, with an average age of 36 years), 81.3% (13/16) of the lesions were in the premolar and molar areas, 56.3% of them exhibited cortex expansion, and 96.8% had discontinued cortex. According to the distribution of internal calcifications, these lesions were divided into: Ⅰ (non-calcification type): absence of calcification; Ⅱ (eccentric marginal type): multiple calcifications scattered along one side of the lesion; Ⅲ (diffused type): numerous calcifications diffusely distributed into the lesion; Ⅳ (plaque type): with a ≥ 5 mm calcified patch; Ⅴ (peri-coronal type): multiple calcifications clustered around impacted teeth. Calcifications were present in 73.7% of COC lesions, including 9 type Ⅱ, 3 type Ⅲ and 2 type Ⅳ lesions, and 42.8% of CEOT lesions had calcification images, including 2 type Ⅲ and 5 type Ⅴ lesions. Six COC lesions had odontoma-like images. Moreover, 8 of 9 type Ⅰ CEOTs were histologically Langerhans cell-rich subtype, which had a smaller size (with an average mesiodistal diameter of 17.8 mm) and were not associated with impacted teeth. CONCLUSION: COC lesions tended to originate from the anterior part of the jaw and exhibit cortex expansion, and were sometimes associated with odontoma. CEOT commonly occurred in the posterior jaw and had discontinued cortex. Two lesions had significantly different calcification map. Over 70% of COC lesions had calcification images, which were mostly scattered along one side of the cysts, far from the impacted teeth. Approximately 60% of CEOT lesions exhibited smaller size and non-calcification, and the remaining CEOT cases often had calcification images clustered around the impacted teeth.

Aggressive Calcifying Epithelial Odontogenic Tumor of the Maxillary Sinus with Extraosseous Oral Mucosal Involvement: A Case Report.

Calcifying epithelial odontogenic tumors are benign odontogenic neoplasms whose occurrence in the maxillary sinus is rare. Maxillary tumors tend to be locally aggressive and may rapidly involve the surrounding vital structures. We report a case of a large calcifying epithelial odontogenic tumor of the maxilla, involving the maxillary sinus in a 48-year-old woman. The tumor was largely intraosseous. In the canine and first premolar regions, the loss of bone could be palpated but the oral mucosa appeared normal. Histologically, the tumor tissue could be seen in the connective tissue below the oral epithelium. The most significant finding was the presence of an intraosseous tumor with an extraosseous involvement in a single tumor, indicating aggressive behavior and warranting aggressive treatment. In this article, we discuss the rare presentation of the tumor and its radiological appearance and histological features. We also highlight the importance of a detailed histopathological examination of the excised specimen.

Establishment and characterization of a primary calcifying epithelial odontogenic tumor cell population.

UNLABELLED: Calcifying epithelial odontogenic tumors (CEOTs) are rare neoplasms derived from dental tissue with the unique characteristic of calcifying amyloid-like material. OBJECTIVES: To establish primary CEOT epithelial-derived cell populations, investigate the expression of enamel matrix proteins (EMPs), and identify potential ameloblastin (AMBN) and patched 1 (PTCH1) gene alterations. MATERIALS AND METHODS: A 28-year-old patient with a lesion of the posterior maxilla, radiographically characterized by a radiolucency with well-defined borders containing mixed radiopacities, agreed to participate with informed consent. The patient's biopsy confirmed the diagnosis of CEOT, and a small representative tumor fragment was ascertained for cell culture. Explant cultures were established and used to establish primary cell populations. These were analyzed for morphology, cell proliferation, mineralization activity, expression of epithelial-associated markers (qRT-PCR and immunocytochemistry), and gene mutations of AMBN or PTCH1. DNA was extracted from tumor cells and gene coding and exon-intron boundaries overlapping fragments amplified. PCR products were bidirectional DNA sequenced and compared against reference sequence. RESULTS: A CEOT cell population was established and proliferated in culture and could be maintained for several passages. Expression of EMPs, cytokeratin 14 and 17, and patched (PTCH1), as well as ALP activity, was detected. These cells also had the ability to mineralize, similar to the primary tumor. Two AMBN alterations were identified in the sample: c.1323G>A/A441A (rs7680880) and c.1344*+111delA. Two single-nucleotide polymorphisms were identified in the PTCH1 gene. CONCLUSIONS: Our data support the establishment of a CEOT-derived cell population, which expresses known epithelial-associated proteins.

Immunohistochemical study of insulin-like growth factor 1 in calcifying epithelial odontogenic tumor and ameloblastoma: experimental research.

Several studies have indicated that increased levels of insulin-like growth factor 1 (IGF-I) or altered levels of its binding proteins are associated with an increased risk of the most common cancers, including cancers of the colorectal, lung, breast and prostate. The aim of this study is to investigate the expression of IGF-1 in both calcifying epithelial odontogenic tumor (CEOT) and ameloblastoma. Materials and methods: The research sample consisted of 23 paraffin block from Oral Pathology Department, Faculty of Dentistry, Damascus University, including six biopsies of CEOT, two biopsies of the plexiform ameloblastoma and 14 biopsies of the follicular ameloblastoma. All specimen were prepared and immunostained with rabbit polyclonal antibody to IGF-1. Immunostaining was assessed according to German-semiquantitative scoring system, and then the results were aggregated and statistically analyzed using SPSS version 13.0 (Student's test for two independent samples, one-way analysis of variance, Kruskal-Wallis test and Mann-Whitney U-test), the significance level P value less than 0.05 was considered statistically significant. Results: All CEOT and ameloblastoma samples were positive for IGF-1 staining, except one sample of ameloblastoma, which was negative. The results showed that there were no statistically significant differences between CEOT and ameloblastoma in the values of IGF-1 expression (P=0.993) and IGF-1 expression rates (P=0.874) and in the frequency of IGF-1 expression (P=0.761) and IGF-1 staining intensity score (P=0.731). Conclusion: IGF-1 plays an important role in the growth of odontogenic tumors and there are no differences in IGF-1 expression between CEOT and ameloblastoma.

Noncalcifying type of calcifying epithelial odontogenic tumor: A rare case report and literature review.

Key Clinical Message: There has been a recent spike in reporting of noncalcifying variants of CEOT with the WHO 2022 classification of head and neck tumors. The present case describes a rare histopathological variant of CEOT of which a handful of cases have been reported. Abstract: This work aimed to report a rare case of noncalcifying type of Pindborg Tumor involving the right mandible. The absence of calcifications in the calcifying epithelial odontogenic tumor presents a diagnostic challenge and prognostic implications. A literature review of the 16 reported clinical cases of this scarce variant was performed.

Giant calcifying epithelial odontogenic tumor after I-125 seed implantation: A case report.

Calcifying Epithelial Odontogenic Tumor (CEOT), also known as Pindborg tumor, is a rare odontogenic benign tumor. It was first reported by Thoma and Goldman in 1946 and defined as an independent tumor by Pindborg in 1957. Herein, we reported a CEOT case involving most of the mandible after I-125 implantation in a 53-year-old man. We cooperated with governmental and hospital departments to resect the tumors, reconstruct the mandible with a fibular flap graft, and properly dispose of the radioactive particles.

A Rare Case of Cemento-Ossifying Fibroma: A Case Report.

Benign fibro-osseous lesions are a group of pathological conditions characterized by the replacement of normal bone with cellular fibrous connective tissue that undergoes mineralization. The most common types of benign fibro-osseous lesions include fibrous dysplasia, ossifying fibroma, and osseous dysplasia. However, diagnosing these lesions can be challenging due to their overlapping clinical, radiological, and histological features, which can cause a diagnostic dilemma for surgeons, radiologists, and pathologists. One rare type of benign fibro-osseous lesion is the cemento-ossifying fibroma (COF), which is a definitive form of a benign fibro-osseous tumor that affects the craniofacial region, particularly the jaws (70%). Here, we present a case of COF in a 61-year-old female patient in the maxillary anterior region. Due to a clear distinction between the lesion and healthy bone, the lesion was treated with conservative surgical excision followed by curettage and primary closure. However, differential diagnosis of COF can be highly challenging for clinicians due to its overlapping features with other fibro-osseous lesions like Paget's disease and fibrous dysplasia. Ossifying fibroma and fibrous dysplasia often present a histopathological, clinical, and radiological overlap. The post-operative follow-up after eight months was unpredictable, with a radiological picture showing the increased thickness of the frontal bone, parietal bone, and maxilla with obliteration of marrow spaces, alteration of the trabecular pattern with a cotton wool/ground glass appearance, and reduced maxillary sinus space. Proper evaluation and diagnosis of fibro-osseous lesions are necessary before arriving at a final conclusion. Cemento-ossifying fibroma in the maxillofacial skeleton is uncommon, and after eight months, the recurrence rate is rare. This case highlights the importance of considering COF as a differential diagnosis for fibro-osseous lesions in the maxillofacial region and the necessity for proper evaluation and diagnosis to determine the appropriate treatment plan and prognosis. In summary, the diagnosis of benign fibro-osseous lesions can be challenging due to their overlapping features, but early diagnosis and proper evaluation are essential for successful treatment outcomes. COF is a rare type of benign fibro-osseous lesion where other fibro-osseous lesions in the maxillofacial region should be considered as a differential diagnosis, and the necessary steps should be taken to confirm the diagnosis before arriving at a final conclusion.

Malignant Variant of Calcifying Epithelial Odontogenic Tumor with Neuroendocrine Differentiation.

A 60-year-old female presented with asymptomatic failing mandibular dental implants. Computed tomography (CT) showed a partially calcified, hypointense lesion within the soft tissues, measuring 1.3 x 0.8 x 1.0 cm along the buccal cortex. Incisional biopsy demonstrated a basaloid type of tumor composed of sheets of cells with plump ovoid nuclei, distinct nucleoli, and scant eosinophilic cytoplasm. Mitoses were present, averaging about 2 per 10 high power fields with scattered individual apoptotic cells. Numerous laminated calcified bodies (Liesegang rings) were observed with confluence of these bodies to form larger foci of dystrophic mineralization. These features clearly established the malignant nature of this tumor. Immunohistochemically, the tumor was positive for synaptophysin, focally positivity for CAM 5.2 and had a Ki-67 proliferation index of approximately 25%. This is the first report of a tumor with features of a malignant variant of calcifying epithelial odontogenic tumor and neuroendocrine differentiation.

Pindborg tumor associated with a supernumerary tooth: a case report.

The calcifying epithelial odontogenic tumor is a rare benign neoplasm that accounts for approximately 1% of all odontogenic tumors. Most of the cases occur in the posterior mandible, and a few involve the maxilla. Despite their relatively indolent biological behavior, tumors in the maxilla tend to grow fast. We report the case of a 33-year-old female patient exhibiting swelling in the right maxilla. An isodense area associated with an impacted supernumerary tooth was found on imaging examination. The histopathologic diagnosis was a calcifying epithelial odontogenic tumor. The treatment of choice was surgical removal of the lesion and associated dental elements. The patient has been followed up for 11 months and shows no signs of recurrence. Besides describing this case, we reviewed the literature on the association of calcifying epithelial odontogenic tumors with supernumerary teeth and found two case reports addressing this subject.

Epiphora and nasal blockage - An unusual presentation of Pindborg tumor: A case report and literature review.

Odontogenic tumors are silent ailments which can affect any individual. One such lesion is a calcifying epithelial odontogenic tumor (CEOT). It is a rare lesion with a locally aggressive nature. A 33-year-old male presented with nasal blockage and continuous flow of tears from the left eye for the past 7-8 months. Clinical examination revealed slightly protruded left eyeball and altered level of eyeballs with continuous watery discharge. Hess chart confirmed normal eye movements. Intraoral findings were not significant. The final diagnosis of CEOT was established based on the histopathological aspects. CEOT is mostly found in the third to fifth decade of life without gender predilection. Presenting symptoms and signs comprise painless expansile mass, although there are reports associated with pain, nasal obstruction, epistaxis, and proptosis. In this article, we would like to present a case of CEOT with epiphora and nasal blockage as the main and only presentation, an uncommon finding. Furthermore, discussing and posing a question of an adequate period of follow-up required to negate the presence of recurrence. One must stay vigilant enough not only to attest a singular symptom to the commonest ailment related to it but also to explore the possibility of the less known. Furthermore, we need to further research in depth to establish a certain duration after which the likeliness of recurrence is to the minimum.

Mandibular Radiolucencies: A Differential Diagnosis of a Rare Tumor.

The diagnosis and treatment of maxillofacial cystic lesions requires careful evaluation and correlation of the clinical presentation and radiological studies. The Pindborg tumor, also known as the calcifying epithelial odontogenic tumor, is a locally invasive benign neoplasm, with only around 300 cases being published to date. This study presents a new case of this already uncommon neoplasm, not associated with an impacted tooth, and describes the clinicopathological features of this rare entity, along with a review of other reported cases. Despite surgery having been recognized as the treatment of choice for the Pindborg tumor, no firm consensus exists concerning the extension of surgical resection.

Noncalcifying clear-cell variant of calcifying epithelial odontogenic tumor: A case report and review.

Clear-cell tumors of the head and neck are biologically diverse consisting of benign, malignant and metastatic lesions. These tumors pose a diagnostic challenge. In the oral cavity, these may be derived from odontogenic/nonodontogenic epithelium or from mesenchyme or can be metastatic. Odontogenic tumors with clear-cell change are rare. Calcifying epithelial odontogenic tumor (CEOT) is a rare, benign, locally aggressive odontogenic epithelial tumor affecting the jaw. Here, we report a case of clear-cell variant of CEOT with its histopathological differential diagnosis. A 43-year-old male patient with swelling in his lower right back tooth region showed a well-defined radiolucent lesion with smooth corticated periphery on radiograph. On incisional biopsy, tumor showed small sheets, cords and islands of odontogenic epithelium with nests of clear cells with no evidence of calcification. A final diagnosis of CEOT was established by differentiating other odontogenic and nonodontogenic lesions on the basis of clinical, radiographic, histopathologic and special stain features.

CEOT Variants or Entities: Time for a Rethink? A Case Series with Review of the Literature.

The first detailed description of calcifying epithelial odontogenic tumor (CEOT) are ascribed to Jens Pindborg, but this tumor was described some years previously. Subsequently, CEOT was included in the 1971 WHO classification of odontogenic tumors and a since then number of variants have been described, which have added confusion to the diagnostic criteria. We aimed to survey the literature on the variants of CEOT, in parallel with a review of our single institution experience of CEOTs. Cases identified were collated, including available clinical, radiological and histological information and then reviewed, taking into account changes in the understanding and classifications of odontogenic tumors since initial diagnosis. We identified 26 cases from 1975 to 2017 for which histological material was available. Of these, only 13 (50%) showed the "classic" histological appearance, whilst two cases were identified as recognized variants. In 11 cases, other diagnoses or a differential diagnosis were preferred, with no agreed diagnosis in four of these. The proliferation fraction (Ki67) in the 10 cases tested was 2.1% ± 0.18. These findings illustrate the diagnostic challenges in this group of tumors and highlight the gaps in knowledge. Techniques, such as EWSR1 gene cytogenetic analysis, may be helpful in cases with clear cells. However, in other areas of controversy, including the non-calcifying and Langerhans cell rich variants, further investigation, perhaps utilizing sequencing technologies may be needed to refine the classification. Owing to the relative rarity of these lesions it would be beneficial if future work could be pursued as an international collaboration.

Hybrid odontogenic tumor masquerading as a salivary gland lesion: A diagnostic predicament.

Hybrid odontogenic tumors are sporadic, where the distinctive areas of more than one odontogenic tumor tissue type have been reported. The occurrence of adenomatoid odontogenic tumor (AOT) with calcifying epithelial odontogenic tumor (CEOT) like areas histologically simulating salivary gland pathology is an unusual finding that has not been previously reported in the literature. We report the case of a 32-year-old female presenting with slow-growing firm swelling, radiographically as a pear-shaped radiolucent lesion in the interdental region of maxillary incisors. Histologically, the tissue showed nests and anastomosing strands of the bland cuboidal to squamoid epithelial cells showing nuclear pleomorphism, hyperchromatism, and abundant cytoplasm with prominent intercellular bridges focally. Multiple basophilic calcifications, amyloid-like material, duct-like formation, and mucinous spillage are seen. Tumor cells showed immunopositivity for CK 7, CK 19, CK 8/18 and low Ki67, p63, and immunonegativity for S100 suggesting of a hybrid lesion of CEOT with AOT.

Non-calcifying Langerhans Cell Rich Variant of Calcifying Epithelial Odontogenic Tumor and Amyloid Rich Variant of Central Odontogenic Fibroma: A Unique Entity or a Spectrum?

Overlapping clinicopathological features of non-calcifying Langerhans cell rich variant of calcifying epithelial odontogenic tumor (NCLC-CEOT) and the amyloid rich variant of the central odontogenic fibroma (AR-COF) have been recognized recently. It is still under debate whether these two diseases are indeed one unique disease entity or belong to CEOT and COF, respectively. To clarify this issue, we have performed a literature review to compare the similarities and differences in clinicopathological features among NCLC-CEOT, AR-COF, classic CEOT, and classic COF. We aimed to investigate whether NCLC-CEOT and AR-COF might be the same and one distinctive disease entity, or a variant (or variants) of either CEOT or COF; or whether COF, NCLC-CEOT/AR-COF, and CEOT represented a histopathological spectrum of one disease. Our results indicate that NCLC-CEOT and AR-COF cases share many similar clinicopathological features. Thus, we suggest that they are the same disease entity. Due to nearly no reported recurrence of NCLC-CEOT/AR-COF cases, the conservative surgical treatment is appropriate. The NCLC-CEOT/AR-COF cases show some overlapping clinicopathological features with COF rather than the CEOT cases. However, differences in the clinicopathological features are still recognized among the NCLC-CEOT/AR-COF, COF, and CEOT cases. Future research, particularly molecular biological studies, may further elucidate their relationships and assist proper classification of the NCLC-CEOT/AR-COF cases.

Unusual Cystic Variant of Calcifying Epithelial Odontogenic Tumor.

Calcifying epithelial odontogenic tumor (CEOT) is a rare benign odontogenic neoplasm, which is exclusively epithelial in its tissue of origin. Many cases of CEOTs are associated with impacted tooth and simulate dentigerous cyst radiographically. The histologic features of CEOT are unique; however, among its various histologic subtypes, the cystic variant is a rare and less well-understood entity. Our report elucidates a cystic variant of CEOT in the maxilla of a 16-year-old male that presents clinical and radiologic findings conscientious to dentigerous cyst; but histopathological diagnosis came out to be a gold standard in identifying this rare tumor. This case report describes the clinicopathologic features of this rare entity, highlighting the histomorphological findings along with reviewing other reported cases.

Calcifying epithelial odontogenic tumors (Pindborg tumor) of maxilla in pediatric patients.

The calcifying epithelial odontogenic tumor (CEOT) was first described by Pindborg as a distinct entity in 1955. Odontogenic tumors are derived from epithelial, ectomesenchymal, and/or mesenchymal elements that are or have been a part of the tooth-forming apparatus. Of all the odontogenic tumors, CEOT accounts for 1% of the cases. There is no sex predilection, with a 2:1 predilection for the mandible, mostly in the premolar/molar region. The CEOT typically presents clinically as an intraosseous, expansile, and painless mass that exhibits slow growth. It is often locally invasive. Most often, it is associated with an impacted tooth, is asymptomatic, and requires biopsy for diagnosis. Although most of these cases are primarily intraosseous, an extraosseous tumor is also known to occur, first observed by Pindborg in 1966. The lesions were surgically enucleated, and histopathological examination confirmed CEOT. The purpose of this article is to describe one additional case of both variants of CEOT.

Synchronous Calcifying Epithelial Odontogenic Tumor: Case Report and Analysis of the 5 Cases in the Literature.

Calcifying epithelial odontogenic tumor (CEOT), or Pindborg tumor, is infrequent and accounts for less than 1% of all odontogenic tumors. It is benign and usually asymptomatic but displays locally infiltrative and expansile behavior. Synchronous lesions affecting the jaws are exceedingly rare. We present a case of synchronous CEOTs affecting the maxilla and mandible and describe the clinical, radiographic, histologic, and immunohistochemical features. We further review five cases previously described in the literature.

A Rare Case of an Aggressive Clear Cell Variant of Calcifying Epithelial Odontogenic Tumor in the Posterior Maxilla.

A clear cell variant of calcifying epithelial odontogenic tumor is a rare benign odontogenic neoplasm, accounting for 33 cases described in the literature. In this article, we report a challenging example of clear cell variant of calcifying epithelial odontogenic tumor of the posterior maxilla in a 45-year-old female patient showing locally aggressive growth and recurrence. Microscopically, islands of polyhedral cells containing abundant cytoplasm, well-developed intercellular bridges blended with clear cells were observed. The nuclei were frequently pleomorphic and permeated by hyaline calcified material. Immunohistochemistry revealed positivity for pan-cytokeratin (AE1/AE3), cytokeratins (CK-14 and CK-19), Bcl-2, p53, and p63. The Ki-67 proliferative index was ~10%. As odontogenic tumors are rare, when a significant clear cell component is observed, the differential diagnosis with other lesions of the jaws with similar morphology, including other odontogenic tumors with prominent clear cell component, clear cell odontogenic carcinomas, and metastatic tumors, is difficult.

Clinical, radiological and histological features of an unique case of calcifying epithelial odontogenic tumor.

Calcifying epithelial odontogenic tumor (CEOT), also known as Pindborg tumor, is a rare benign but locally aggressive odontogenic neoplasm, accounts for <1% of all odontogenic tumors. CEOT is usually seen in the posterior area of the mandible in-between 30 and 50 years of age without definite sex predilection. A painless, slow-growing swelling with bone expansion is the most common clinical feature of CEOT. Radiographically, it presents as a mixed radiographic lesion may or may not be associated with any impacted tooth. Confirmation of the diagnosis is made by histopathological examination. The tumor has a recurrence rate of 10%-20% and so periodic follow-up is necessary. A unique case of CEOT involving the right mandibular molar-premolar in a 25-year-old female patient with clinical behavior, radiological, histopathological features and surgical managements is discussed herewith.