Epithelial Odontogenic Tumor Research Articles

Squamous Cell Carcinoma Arising in a Squamous Odontogenic Tumor of the Maxilla: Case Report and Review of the Literature.

Squamous odontogenic tumor (SOT) is an exceedingly rare, benign epithelial odontogenic tumor showing squamous differentiation. It is composed of variably sized and shaped islands of cytologically bland, mature squamous epithelium within a fibrous stroma. In this report, we present a rare transformation of a squamous odontogenic tumor (SOT) of the maxilla into a well-differentiated squamous cell carcinoma (SCC) with involvement of the pterygoid plates. To the best of our knowledge, only two cases of malignant transformation of SOT has been reported in the literature. Herein, we seek to report this extremely rare occurrence to raise awareness of oral and maxillofacial surgeons and pathologists of this unusual, but serious event and perform a literature review of squamous odontogenic tumors.

Calcifying epithelial odontogenic tumour with an atypical clinical presentation in a paediatric patient: A case report and literature review

AbstractAimWe report a rare case of a calcifying epithelial odontogenic tumour with atypical clinical presentation in an adolescent and present a literature review of this tumour in paediatric patients.Materials and MethodsA 12‐year‐old female patient presented with a painless swelling of the left mandible, with a history of 6 months. Clinical examination revealed a sessile mass with a reddish‐pink colour and firm consistency. Tomographic images showed a hypodense, unilocular lesion involving unerupted tooth #34, with a hyperdense focus in contact with the tooth crown disrupting the buccal cortex.ResultsEnucleation, curettage and peripheral osteotomy of the lesion were performed, and the impacted tooth was removed, followed by the application of autologous leucocyte and platelet‐rich fibrin matrices to the region. The patient evolved favourably with good clinical healing and evidence of new bone formation.ConclusionsDespite its aggressive potential, the conservative approach for CEOT in paediatric patients proved to be effective. Our review found reports of recurrence up to 13 years after surgery. Therefore, long‐term clinical and radiographical follow‐up is indicated.

Ameloblastic Fibro Odontoma in a 10-Year-Old Boy: Case Report

Introduction: Ameloblastic fibro‑odontoma (AFO) is a rare, slow‑growing neoplastic lesion classified as a benign, epithelial odontogenic mesenchymal tumor. This tumor exhibits histological features characteristic of both ameloblastic fibromas and complex odontomas. The clinical manifestation of AFO is typically characterized by the asymptomatic enlargement of the jawbones. Radiographically, it presents as a distinct radiolucent region, indicating the presence of radiopaque substances with varying degrees of irregularities in size and morphology. Standard therapeutic intervention involves enucleation. Despite its benign nature, AFO can cause significant morbidity if left untreated. Therefore, prompt diagnosis and appropriate management are essential to ensure optimal patient outcomes. The present study described the case (clinical presentation and management) of a 10‑year‑old male patient with an AFO lesion located in the posterior mandible. This particular case was treated with conservative measures involving surgical enucleation along with the extraction of the impacted tooth and the curettage of residual bone.

Plexiform Variant of Ameloblastoma in Body of Mandible: Case Report and Review of Literature

Ameloblastoma is a benign but locally aggressive odontogenic epithelial neoplasm arising from remnants of dental lamina and dental organ [1,4]. This neoplasm is more prevalent between 2nd to 4th decade of life and mandible (11%) is more commonly involved than maxilla (1%) [2,5]. It has an aggressive behavior and recurrent course, It is rarely metastatic. Ameloblastoma represents 10% of odontogenic tumors and 1% of all tumors and cysts involving the maxillo mandibular region [1]. The World Health Organization (WHO) classification of 2017 describes ameloblastomas of the following four types: Solid/multicystic ameloblastoma (91%) unicystic ameloblastoma (6%); extraosseous/peripheral ameloblastoma (2%); and metastasizing ameloblastoma (1%). [3,8] A conclusive diagnosis can only be made by histopathology because it has wide range of histologic patterns and has radiographic characteristics with other lesions such the odontogenic keratocyst, giant cell tumour, aneurysmal bone cyst, and renal cell carcinoma metastasis.[5] Treatment for each type of ameloblastoma differs depending on its biological behaviour and rate of recurrence. This report presents a case of ameloblastoma involving the body of mandible with resorption of the root apices of canine, premolars and first molar. The lesion extends till the base of mandible with intact lower border. This was surgically excised preserving the lower border of mandible with safe sound margins of bone.

Molecular pathogenesis of ameloblastoma.

Ameloblastoma (AM) is a benign, although aggressive, epithelial odontogenic tumour originating from tooth-forming tissues or remnants. Its aetiopathogenesis remains unclear; however, molecular analysis techniques have allowed researchers to progress in understanding its genetic basis. The high frequency of BRAF p.V600E as a main driver mutation in AM is well established; nevertheless, it is insufficient to explain its tumourigenesis. In this review, we aimed to integrate the current knowledge about the biology of AM and to describe the main genetic alterations reported, focusing on the findings of large-scale sequencing and gene expression profiling techniques. Current evidence shows that besides BRAF mutation and activation of the MAPK pathway, alterations in Hedgehog and Wnt/β-catenin pathway-related genes are also involved in AM pathogenesis. Recently, a tumour suppressor gene, KMT2D, has been reported as mutated by different research groups. The biological impact of these mutations in the pathogenesis of AM has yet to be elucidated. Further studies are needed to clarify the impact of these findings in the identification of novel biomarkers that could be useful for diagnosing, classifying, and molecular targeting this neoplasm.

Low Detection of High-risk Human Papilloma Virus in Individuals with Ameloblastoma in a Tertiary Hospital in Lagos, Nigeria.

Ameloblastoma is a benign but aggressive epithelial odontogenic neoplasm of unknown etiology. The role of human papilloma virus (HPV) in the etiology of oral squamous cell carcinoma has prompted the investigation of HPV as an etiologic factor in ameloblastoma. This study aimed to determine the frequency of high-risk (HR) HPV in conventional ameloblastoma and the clinical parameters associated with infection. The study was approved by the ethical review boards of the institution. DNA was extracted from fresh tissue collected 750 μL of DNA/RNA Shield (Zymo Research, United States) using Invitrogen PureLink Viral RNA/DNA Mini Kit (Invitrogen, USA). The extracted DNA was assayed for the detection of 14 HR HPV types (16, 18, 31, 33, 35, 39, 45, 51, 52, 56, 58, 59, 66, and 68) using Anyplex™ II HPV HR Detection kit (Cat. No. HP7E00X) (Seegene Inc., Republic of Korea) on CFX-96TM Real-Time Polymerase Chain Reaction (PCR) System (Bio-Rad). Data on gender, age of patient, site of lesion, clinicohistological types of ameloblastoma and history of smoking, alcohol consumption, and practice of oral sex were collected. Data analysis was performed using analysis program SPSS version 25 and statistical significance was set at P < 0.05. Two cases of conventional ameloblastoma were positive with HPV and none of the ameloblastic carcinoma cases were positive. The HPV 16 serotype was observed in both cases. While 5 of the cases had a history of alcohol consumption, none of these cases were positive for HPV serotype. HPV 16 positivity was detected in two cases of conventional ameloblastomas and none in ameloblastic carcinoma using real-time PCR. There was no effect of exposure to smoking, alcohol consumption, and practice of oral sex and HPV in the etiology of ameloblastoma. Data available are suggestive of a limited role of HPV in the etiology of ameloblastoma.

Desmoplastic histological subtype of ameloblastoma in 16 dogs.

Ameloblastoma in dogs most often has a highly conserved acanthomatous cell morphology and is recognized as canine acanthomatous ameloblastoma (CAA) while conventional ameloblastoma (CA) makes up a smaller yet morphologically diverse group of epithelial odontogenic tumors. In humans, a rare desmoplastic histological subtype has distinctive clinical, radiological, and microscopic features. Desmoplastic ameloblastoma (DA) has not previously been described in dogs, although it has been rarely referenced in the veterinary literature. This is the first thorough description of a case series of DA in dogs and describes clinical presentation, diagnostic imaging findings, histopathological features for diagnosis, and treatment outcome. Clinically, DA most often presents as a mass or swelling in the rostral mandible or maxilla of middle age to older dogs. On diagnostic imaging, the lesion has a radiolucent or mixed pattern with well-defined borders and variable loculation. As a solid, fibrous tumor with obscured odontogenic epithelium, DA is challenging to diagnose histologically and can mimic several other oral tumors, both benign and malignant. As an ameloblastoma, the biological behavior of DA is locally destructive yet benign and prognosis is favorable following surgical excision.

Imaging Characteristics of Embedded Tooth-Associated Cemento-Osseous Dysplasia by Retrospective Study.

Since there are many differential diagnoses for cemento-osseous dysplasia (COD), it is very difficult for dentists to avoid misdiagnosis. In particular, if COD is related to an embedded tooth, differential diagnosis is difficult. However, there have been no reports on the characteristics of the imaging findings of COD associated with embedded teeth. The aim of the present study was to investigate the occurrence and imaging characteristics of cemento-osseous dysplasia (COD) associated with embedded teeth, in order to appropriately diagnose COD with embedded teeth. The radiographs with or without histological findings of 225 patients with COD were retrospectively analyzed. A retrospective search through the picture archiving and communication system (PACS) of the Division of Oral and Maxillofacial Radiology of Kyushu Dental University Hospital was performed to identify patients with COD between 2011 and 2022. Fifteen COD-associated embedded mandibular third molars were identified in 13 patients. All 13 patients were asymptomatic. On imaging, COD associated with embedded mandibular third molars appeared as masses that included calcifications around the apex of the tooth. On panoramic tomography, COD showed inconspicuous internal calcification similar to that of odontogenic cysts or simple bone cysts, especially in patients with COD only around the mandibular third molar region. Those with prominent calcification resembled cemento-ossifying fibroma, calcifying epithelial odontogenic tumor, calcifying odontogenic cyst, adenomatoid odontogenic tumor, and so on, as categories of masses that include calcifications on panoramic tomography and computed tomography. The current investigation is the first to report and analyze the imaging characteristics of COD associated with embedded teeth. It is important to consider the differences between COD and other cystic lesions on panoramic tomography, and the differences between COD and masses that include calcifications on CT.

METTL1 facilitates ameloblastoma invasive growth via MAPK signaling pathway

ObjectivesAmeloblastoma (AM), a common odontogenic epithelial tumor, exhibits aggressive growth due to incomplete encapsulation within the jawbone. Postoperative recurrence is a significant concern, closely associated with its invasive nature. We investigate the role of tRNA N-7 methylguanosine (m7G) modification mediated by Methyltransferase-like 1 (METTL1) in AM's invasive growth and prognosis. Materials and MethodsMETTL1 expression was analyzed in diverse cell lines and clinical AM tissues. Its association with postoperative AM recurrence was examined. Functional experiments included METTL1 gene silencing using shRNA in hTERT-AM cells, assessing cell proliferation, migration, and invasion. Xenograft tumor model was constructed to investigate tumor growth. Molecular mechanisms behind METTL1′s role in AM invasiveness were elucidated using Ribosome nascent-chain complex-bound mRNA sequencing (RNC-seq) and experimental analysis. ResultsHigh METTL1 expression was significantly associated with postoperative recurrence in AM. The inhibition of AM development following METTL1 knockdown has been corroborated by experiments conducted both in vitro and in vivo. Analysis of RNC-seq data revealed that downregulated genes were predominantly enriched in the mitogen-activated protein kinase (MAPK) signaling pathway, suggesting that METTL1 may promote AM's invasive growth through the MAPK signaling pathway. ConclusionOur study elucidates the functional role of METTL1 in AM's invasive development and prognosis. High METTL1 expression is linked to postoperative recurrence, and METTL1 appears to promote AM invasiveness through the MAPK signaling pathway. These findings contribute to a better understanding of AM pathogenesis and may guide future therapeutic strategies.

Comprehensive management of Pindborg tumor in childhood: a case report

Relevance. The calcifying epithelial odontogenic tumor, also known as Pindborg tumor, is a locally aggressive neoplasm characterized by strands and medullary formations of flat and clear cells, often accompanied by spherical calcifications and amyloid-stained hyaline deposits. The Pindborg tumor is rare, representing less than 1% of odontogenic tumors, and typically manifests in individuals aged 20 to 60 years. However, there is a growing incidence of neoplasms of diverse origins in childhood. This article presents a clinical case detailing the comprehensive multi-stage treatment of a pediatric patient with a Pindborg tumor, facilitating complete rehabilitation before reaching adulthood.Clinical case description. This article presents a clinical case detailing the comprehensive management of a patient with a Pindborg tumor. The individual initially sought treatment at the Department of Pediatric Maxillofacial Surgery of A.I. Evdokimov Moscow State Medical and Dental University in 2020, undergoing complete combined treatment until November 2023. Full rehabilitation was successfully achieved by the time of adulthood through a series of four consecutive surgeries, including dental implantation, followed by prosthetics utilizing a non-removable orthopedic structure in the final stage.Conclusion. Initiating comprehensive rehabilitation at an early stage, including dental implantation before reaching adulthood, facilitates complete social adaptation in society and contributes to the patient's harmonious development.

Unusual Variant of Ameloblastoma with Calcifying Epithelial Odontogenic Tumor-like Areas in a Three-Year Old Child

The Calcifying Epithelial Odontogenic Tumor (CEOT) is a benign odontogenic tumor, comprising approximately 1% of all odontogenic tumors. It presents as a slow-growing tumor in posterior mandibular region. A three year-old female child from Multan, reported a swelling in right lower jaw progressively increasing in size eventually causing facial asymmetry. On intraoral examination, a protuberance was seen arising from posterior mandibular region and causing displacement of overlying teeth. The swelling was cystic on palpation and mildly tender. The patient underwent complete excision of lesion at a tertiary care center in her locality. The excision biopsy specimen was received at our lab which was intact and well preserved. A diagnosis of ameloblastoma with Calcifying epithelial odontogenic tumor-like areas was made. The patient was followed up after six months however no complaint of any recurrent swelling in the area of was recorded.

DNA content and clinicopathological features aid in distinguishing ameloblastic carcinoma from ameloblastoma.

Ameloblastoma and ameloblastic carcinoma are epithelial odontogenic tumors that can be morphologically similar. In the present study, we evaluated the DNA content and Ki-67 index in the two tumors. The paraffin blocks of the tumors were selected to obtain sections for the immunohistochemical reactions and preparation of the cell suspension for acquisition in a flow cytometer. The Random Forest package of the R software was used to verify the contribution of each variable to classify lesions into ameloblastoma or ameloblastic carcinoma. Thirty-two ameloblastoma and five ameloblastic carcinoma were included in the study. In our sample, we did not find statistically significant differences in Ki-67 labeling rates. A higher fraction of cells in 2c (G1) was correlated with the diagnosis of ameloblastoma, whereas higher rates of 5c-exceeding rate (5cER) were correlated with ameloblastic carcinoma. The Random Forest model highlighted histopathological findings and parameters of DNA ploidy study as important features for distinguishing ameloblastoma from ameloblastic carcinoma. Our findings suggest that the parameters of the DNA ploidy study can be ancillary tools in the classification of ameloblastoma and ameloblastic carcinoma.

Hybrid Calcifying Epithelial Odontogenic Tumor and Ameloblastoma: A Report of an Extremely Rare Condition.

Calcifying epithelial odontogenic tumor (CEOT) and ameloblastoma are types of odontogenic tumors accounting for 1%, and 10% of all the odontogenic tumors. While sharing same odontogenic origin, these tumors are found to exhibit distinct clinicopathological features. In the present study, we present the third hybrid CEOT/Ameloblastoma tumor ever reported. The current CEOT/Amelobastoma is occurred after a previously operated CEOT in the same area. The patient was refered with distict clinical features of swelling and paresthesia. In the radiographic examination, a unilocular lesion with mixed internal structure and ambiguous periphery was seen which exhibited buccal and lingual cortical expansion, thining, and perforation as well as inferior alveolar canal perforation. The histopathology results suggested a CEOT/Ameloblastoma lesion. After the tumor removal, the patient was set up for further follow-ups and maxillofacial prosthesis.

Adenoid Ameloblastoma- A very rare, odontogenic tumor variant with less than 20 reported cases in the literature: Report of two cases and literature review

Abstract Introduction/Objective We describe two cases of Adenoid Ameloblastoma (AA), a very rare, hybrid variant, of two of the most common odontogenic neoplasms (Ameloblastoma and adenomatoid odontogenic tumors). AA is a benign epithelial odontogenic tumor characterized microscopically by epithelium resembling conventional ameloblastoma, with additional duct-like structures, epithelial whorls, and cribriform architecture. Dentinoid deposits, clusters of clear cells, and ghost-cell keratinization may also be present. These tumors do not harbor BRAF or KRAS mutations and their molecular basis appears distinct from conventional ameloblastoma but remains unknown. The lesion tends to recur, and sometimes, only correctly diagnosed after recurrence. Methods/Case Report Adenoid Ameloblastomas were identified after a review of the Temple University, Department of Pathology archive from 2012-2022. A total of four specimens (two patients) were identified. Results (if a Case Study enter NA) Case# 1 22-years-old female Biopsy Location/ size: Mandible, left, 2.1 cm. Microscopic: Neoplastic proliferation composed of odontogenic epithelium in a plexiform pattern with the formation of pseudo glandular structures of variably sized cystic spaces, the peripheral epithelial cells are palisaded with reversed polarity. The central cells have clear cytoplasm and resemble stellate reticulum with occasional tight whorls of cells noted. Hemorrhage and giant cells are present in granulation tissue. The lesion was incompletely excised. Diagnosis: Benign odontogenic tumor. Case#2 (1st occurrence): -49-years-old male -Biopsy -Location and size of lesion: Maxilla-left anterior, 0.9 cm. -Microscopic: Cyst lined by loosely arranged epithelial cells with a basal cell layer having hyperchromatic and palisaded nuclei. Knots of squamous cells are present within the cyst lining. The luminal layer is flattened to cuboidal and nonkeratinized. Duct-like openings are present within the epithelium and contain myxoid stromal tissue. Focally the epithelium extends into the connective tissue as thin anastomosing strands surround the myxoid tissue. -Diagnosis: Benign odontogenic tumor. Case#2 (2nd occurrence): -Biopsy -Diagnosis: Benign odontogenic tumor Case#2 (3rd occurrence): -Excisional biopsy -Diagnosis: AA Conclusion AA is a rare, aggressive odontogenic tumor variant with morphologic overlap with other odontogenic lesions; therefore, oral pathologists and clinicians should be familiar with this entity to achieve an accurate diagnosis and prompt management.

Non-Calcifying/Langerhans Cell-Rich Calcifying Epithelial Odontogenic Tumour: A Critical Review of the Rare and Distinctive Entity.

The study aims to analyse the non-calcifying/Langerhans cell rich (NCLC) subtype of calcifying epithelial odontogenic tumour (CEOT). METHOD: The features of cases of the NCLC subtype of CEOT noted in the English literature by PubMed as well as 3 new cases were reviewed. Overall, twenty-one cases were noted. Many were women in the fourth to sixth decades (male-to-female ratio =1 to 2). Radiologically, the lesion is often unilocular with resorption of the affected teeth. Nineteen of the 21 cases occurred in the maxilla, especially the anterior portion. On pathological examination, epithelial cells are noted in non-calcifying amyloid-rich fibrous stroma. The main differential diagnosis is the amyloid subtype of central odontogenic fibroma. Immunohistochemical studies revealed the tumour epithelial cells were positive for cytokeratins and p63 and contained CD1a, S-100, and langerin-positive Langerhans cells. On a median follow-up of 2 years, one patient had a recurrence one year after curettage. The NCLC subtype of CEOT is unique as it contains significant numbers of Langerhans cells and has clinicopathological features distinctive from classic CEOT.

Calcifying odontogenic cyst – A three dimensional radiological evaluation by cone beam computed tomography

Calcifying odontogenic cysts is an odontogenic cyst that gives rise to painless swelling which expand and occasionally perforate bone. Most of them are between 2 to 4 cm in diameter. Approximately 75% occur in anterior canine-incisor region. The highest incidence of occurence is between 10 and 29 years. Radiographically they appear as well-circumscribed, unilocular cyst like areas with scattered radiopacities which range from mere flecks to large masses. The related tooth usually gets displaced. Radiopaque flecks occur in radiographic features of some lesions which undergo osteolysis during their development. They occur as a result of dystrophic calcifications in long standing chronic mature lesions. The location of such entities whether pericoronal or periapical is also crucial in narrowing the diagnosis. Such flecks in radiographs occur near the pericoronal region in calcifying epithelial odontogenic tumour, ameloblastic fibro-odontoma, whereas in calcifying odontogenic cyst, they appears small, discrete, pebble-like with a smooth outline in periapical region. Such pebbles of radiopaque specks were incidentally seen in cone beam computed tomography in a 20-year-old male evaluated radiographically in orthogonal planes in coronal, axial, sagittal slices and three-dimensional reconstructed view and treated by surgical enucleation was described here.

Collision Tumour Comprising Desmoplastic Ameloblastoma and Squamous Odontogenic Tumour in the Anterior Maxilla: A Case Report.

Collision tumours, characterized by the simultaneous occurrence of two distinct neoplasms within the same anatomical site, are exceedingly rare in oral pathology. This case report presents an uncommon collision tumour involving desmoplastic ameloblastoma and squamous odontogenic tumour in the anterior maxilla of a 52-year-old male from the Indian population. Desmoplastic ameloblastoma is a variant of ameloblastoma known for its unique histopathological features, while squamous odontogenic tumour is a benign epithelial odontogenic tumour with distinctive clinical behaviour. The rarity of this occurrence emphasizes the need for accurate diagnosis and effective treatment strategies. This report discusses the clinical presentation, radiographic findings, and histopathological characteristics of this collision tumour. Through the presentation of this case, we aim to contribute to the understanding of these rare entities and their management considerations.

Profile of ameloblastoma patients at Dr. Soetomo general hospital, Surabaya: A descriptive study

Link of Video Abstract: https://youtu.be/_nqEUazGnE0 Background: Ameloblastoma is a rare odontogenic epithelial tumor affecting the jawbone (maxilla or mandible). According to the WHO in 2005, there are four different classifications of ameloblastoma: solid or multicystic, unicystic, peripheral, and desmoplastic. Despite being benign, ameloblastoma can seriously damage bones, resulting in deformity and even death. This research aims to know the profile of ameloblastoma due to limited data in Indonesia. Methods: This study is an observational descriptive study. Cross-sectional data collection was used. Information was gathered from the medical records of patients with ameloblastoma treated at Dr. Soetomo general hospital, Surabaya between January 2018 and December 2022. A descriptive analysis was conducted to determine the frequency and proportion of each research variable. Results: Forty-two ameloblastoma patients participated in the study. Patients with ameloblastoma ranged in age from 14 to 75, with a mean age of 39.6 (15.7) years. The majority of patients (57.1%) were female. The majority of the cases were mandibular ameloblastoma. Only 4.8% of ameloblastoma cases occurred in the maxilla, while 76.2% of ameloblastoma locations were found in the left mandible. Up to 97.61% of the surgeries conducted include extensive resection. Most patients (95.2%) with ameloblastoma had successful outcomes. Conclusion: Although rare, the profile of ameloblastoma patients reveals that there are still incidences of fatality, necessitating further discussion of patient mortality risk factors.

Calcifying epithelial odontogenic tumor- Review of literature

The Calcifying epithelial odontogenic tumour (CEOT), also known as Pindborg tumour, is a rare odontogenic neoplasm makes up 1% of all odontogenic tumours, characterized by its distinct histopathological features and challenging clinical management. CEOTs are benign epithelial odontogenic tumour that secretes an amyloid protein tending towards calcification, however they can be locally aggressive and have recurrence rates of 10% to 15%. This comprehensive review aims to provide a detailed overview of the CEOT, encompassing its epidemiology, clinical findings, radiographic features, histopathological characteristics and therapeutic strategies.

504 A Curious Case of a Slow-Growing Intra-Oral Swelling in a Paediatric Patient

Abstract Background A 15-year-old male attended the paediatric dental department as an urgent self-referral. Clinical examination revealed a large, firm lesion in the right maxilla which had been slowly increasing in size for the past year. No altered sensation and facial numbness was detected. The upper right central incisor (UR1) was missing however, a primary central incisor was extracted at the dentist three months prior. Investigations An orthopantomogram (OPG) at initial presentation illustrated a well-defined radiolucency extending from the upper right first molar (UR6) to the upper left central incisor (UL1) with root displacement of adjacent teeth. A subsequent cone beam computed tomography (CBCT) scan identified a well-defined corticated radiolucency with an area of radioopacity of mixed density in the posterior-medial aspect. Buccal expansion and root displacement in the premolar segment were noted. The unerupted UR1 was extremely displaced, lying horizontal and just inferior to the nasal cavity. Diagnosis/Management Differential diagnoses for the anterior right maxilla radiolucency included calcifying epithelial odontogenic tumour, adenomatoid odontogenic tumour and an odontome. The histopathology of the cystic lining taken under general anaesthetic during enucleation and curettage revealed the diagnosis of a calcifying odontogenic cyst. Discussion Calcifying odontogenic cysts are very rare developmental lesions occuring due to proliferation of odontogenic epitheluim and scattered nest cells. As in this case, these cysts are usually associated with unerupted/impacted teeth. Awareness of dental eruption timelines is essential in identifying impacted teeth and associated pathology. Slow growing lesions in the maxilla should be identified early and investigated within multidisciplinary teams.