Odontogenic Tumor Research Articles

TRATAMENTO CIRURGICO DE CERATOCISTOS ODONTOGÊNICOS: RELATO DE CASO CLÍNICO

Introduction: The keratocyst or keratocyst is an odontogenic tumor characterized as a lesion that affects the maxillary and mandibular regions. It is considered a benign lesion, reaching a prevalence of 3% to 11% among all odontogenic cysts, resulting in major bone loss. It can be asymptomatic and is usually diagnosed through routine radiographs or histopathological findings. Objective: To present a clinical case concerning the diagnosis and surgical treatment of an odontogenic keratocyst in the anterior mandibular region. Clinical case: Male, normosystemic, melanodermic patient. An increase in extraoral volume was observed on the right side of the face in the anterior region of the mandible. Panoramic radiography revealed well-defined, unilocular radiolucent images in the areas of the right lower first premolar, left lower third molar and bilateral upper third molars. On intraoral physical examination, bone bulging was observed in the region of the tooth in question. A computerized tomography scan was then requested, in which extensive expansive bone lesions were detected. Marsupialization and local decompression of the two upper lesions and the lower right lesion were performed, detecting the presence of liquid content. Firstly, an incisional biopsy was carried out and a decompression drain was installed in the three lesions. The histopathological report was of an odontogenic keratocyst. The decompression device remained in place for 30 days in the lower right lesion and approximately 15 days in the upper lesions, showing a significant reduction in the lesions. The lower right lesion was then enucleated. The 6-month post-operative image showed bone formation with no signs of recurrence. Conclusion: The proposed treatment proved to be effective in treating odontogenic keratocysts, but follow-up imaging is extremely important in view of the possibility of recurrence.

Huge calcifying epithelial odontogenic tumor of the mandible and management with a teeth preserving surgical approach: a case report

BackgroundCalcifying epithelial odontogenic tumor is a rare benign tumor that predominantly occurs in posterior sites of the mandible in adults.Case presentationThis case report describes a case of calcifying epithelial odontogenic tumor in a 37-year-old Iranian female with the chief complaint of swelling in the anterior region of the mandible and tooth displacement. This paper summarized the pathological findings, radiographical features and clinical management of the patient. Clinical examinations revealed a bony hard swelling ~ 3 cm × 4 cm in size with tenderness in the chin area. Radiography revealed an expansile mixed radiolucent and radiopaque lesion with cortical borders. A minimally aggressive surgical approach is used to remove the lesion.ConclusionThis case report outlines the successful outcomes of this surgical approach and the positive outcome associated with the restoration of lost functions of displaced mobile teeth in an arranged and nonmobile way after treatment.

Histone H3K9 Methylation Is Differentially Modified in Odontogenic Cyst and Tumors.

Histone modification in odontogenic lesions is mostly unexplored. Trimethylation of histone H3 at lysine residue 9 (H3K9Me3) has been studied in various pathologic conditions and showed biological significance promising for future therapeutic application. This study aimed to investigate the level and clinical relevance of the H3K9Me3 histone modification in odontogenic cysts and tumors. A total of 105 cases of odontogenic lesions, comprising 30 odontogenic keratocysts (OKCs), 30 adenomatoid odontogenic tumors (AOTs), 30 ameloblastomas, and 15 dental follicles, were included in the study. The paraffin-embedded tissues were immunohistochemically stained for H3K9Me3. Both the intensity and the distribution of staining were evaluated and calculated as H-score. The correlation between the H3K9Me3 expression and the clinical characteristics of each lesion was evaluated. The Kruskal-Wallis test followed by Bonferroni's correction was performed to assess the differences in H-score among groups. In addition, Pearson's chi-squared test or Mann-Whitney U test was used to analyze potential factors that could affect protein expression. The reduced enamel epithelium of the dental follicle showed uniformly strong H3K9Me3 expression. All odontogenic cysts and tumors examined demonstrated a significantly reduced H3K9Me3 level compared with dental follicles. The AOT showed the lowest H3K9Me3 level, followed by OKC and ameloblastoma. Its immunoreactivity was mainly localized in the basal and parabasal cells of OKC and the whorled/duct-like structures of AOT. Ameloblastoma exhibited marked variation in the H3K9Me3 level among cases. Notably, the upregulated H3K9Me3 was related to multilocularity of OKC and ameloblastoma. Histone H3K9 methylation is differentially expressed in odontogenic cysts and tumors. This epigenetic modification may contribute to the pathogenesis and aggressive behavior of odontogenic lesions.

Multiple Compound Composite Odontomas Of The Maxilla: A Report Of Two Cases

Odontomas are benign mixed odontogenic tumours, considered to be developmental anomalies resulting from the growth of differentiated epithelial and mesenchymal cells. It is the most common benign odontogenic tumour, usually asymptomatic and are often discovered during routine radiographic examinations. It can occur at any age, but are most common in the first two decades of life. It is more commonly seen in the anterior maxilla and frequently cause delayed eruption of teeth or retention of primary teeth. Morphologically odontomas are classified as compound and complex odontomas, in which the former resembles teeth whereas the latter being an irregular mass. This paper describes two cases of compound composite odontomas of anterior maxilla in 10 year old and 5 year old patients, with detailed clinical presentation, histopathological features and treatment aspects.

Odontogenic carcinosarcoma of the mandible: A case report.

Odontogenic carcinosarcoma, a rare and challenging diagnosis, was identified in a 60-year-old male through histopathology, revealing a biphasic neoplasm with malignant epithelial and mesenchymal components. Surgical resection is crucial for management, highlighting the importance of vigilant postoperative follow-up to ensure early detection of any recurrence. One rare mixed malignant odontogenic tumor is odontogenic carcinosarcoma, which comprises malignant epithelial and mesenchymal components. Diagnosing odontogenic carcinosarcoma is challenging due to its rarity and atypical clinical presentation. This study reports a 60-year-old male patient who presented with a painless swelling on the right side of his face and experienced facial asymmetry for 6 months, ultimately diagnosed with odontogenic carcinosarcoma. A biphasic neoplasm with malignant alterations in both epithelial and mesenchymal components was identified upon histopathological examination. MRI imaging showed an expansile multilobulated lytic lesion with cortical erosion and extraosseous extension in the posterior region of the right mandibular body and ramus. Following contrast administration, homogeneous lesion enhancement was observed, with a few small non-enhancing necrotic areas in central parts. The patient subsequently underwent a right hemi-mandibulectomy with resection of adjacent soft tissues and neck dissection due to lymph node involvement. The resulting defect was reconstructed using a pectoralis major flap. No recurrence or metastasis was reported during the 6-month follow-up, reinforcing the positive results. This case highlights the importance of recognizing odontogenic carcinosarcoma and underscores the challenges in diagnosing and managing this rare tumor. Early identification and aggressive treatment can lead to positive outcomes, as evidenced by the absence of recurrence or metastasis in this patient during the follow-up period.

Histopathological spectrum of primordial odontogenic tumor with co-existing dentigerous cyst: 1st reported case of the world with a proposed ‘updated diagnostic criteria’

BackgroundPOT is a relatively newly described benign odontogenic tumor with very few cases registered to date. We present the 1st case of Primordial odontogenic tumor (POT) from Sub-Saharan Africa with unique clinicopathological features; also, this is the first case to report POT’s existence as a Hybrid Odontogenic lesion (HOL), with a pertinent review of the literature.Case presentationThis was a 17-year-old patient who presented with slow-growing, painless posterior mandibular swelling. The imaging revealed a well-defined, unilocular, expansile, lytic lesion with internal calcific foci surrounding an impacted #36, indicating a calcifying odontogenic cyst. The incisional biopsy revealed the presence of POT. The tumor was excised along with the involved tooth.ConclusionPOT is predominantly a non-aggressive and mostly affects the pediatric population. Hence, clinicians must be updated on all the aspects of this tumor to diagnose it appropriately and avoid any undue over—or under-treatment.

Central odontogenic fibroma: retrospective study of six cases with variable histopathologic features using 2022 WHO classification

BackgroundCentral odontogenic fibroma (COF) is a rare benign odontogenic tumor with a wide range of histopathologic features. We evaluated COF diagnosed in our institute with 16 years’ experience using 2022 WHO classification.MethodsOur archives were reviewed and cases diagnosed as COF were selected. Clinical, radiographic and microscopic features were tabulated and analyzed.ResultsOf 13,736 specimens, six cases (0.04%) of COF were discovered. Patients ranged in age from 14 to 44 years. There were two males and four females. Maxilla and mandible were affected equally. Five cases showed radiolucent appearance (4 unilocular, 1 multilocular) and one case showed a mixed radiolucent-radiopaque pattern. Histopathologically, three cases were conventional type (2 epithelium-rich, 1 epithelium-poor). Two cases were the hybrid COF with central giant cell granuloma (CGCG) and one of which was also associated with secondary aneurysmal bone cyst (ABC). The last case with a mixed radiolucent-radiopaque pattern was the ossifying subtype.ConclusionOur results demonstrated that COF is a rare odontogenic tumor and exhibits diverse radiographic and microscopic appearances. The triphasic tumor consisting of the COF, CGCG and ABC, is reported here for the first time, while the ossifying subtype is considered the tenth case reported in the English-language literature. Oral and maxillofacial pathologists and other healthcare personnel must be aware of this rare odontogenic tumor and its variants to achieve the definite diagnosis.Clinical trial numberNot applicable.

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.

Rare Head and Neck Cancers and Pathological Diagnosis Challenges: A Comprehensive Literature Review.

Head and neck cancers (HNCs) arise from anatomically adjacent sites and subsites, with varying etiological factors, diagnostic strategies, prognoses, and treatment approaches. While conventional squamous cell carcinoma (SCC) is the most common histology in the head and neck district, HNCs encompass a variety of rare histopathological entities, categorized into epithelial tumors such as salivary gland cancers, sinonasal tumors, neuroendocrine tumors, malignant odontogenic tumors, and SCC variants versus non-epithelial tumors including soft tissue sarcomas, mucosal melanomas, and hematological malignancies. Rare HNCs (R-HNCs) represent a diagnostic and clinical challenge, requiring histopathological expertise, the availability of peculiar molecular analysis, and the personalization of local and systemic treatments, all guided by a multidisciplinary tumor board. Here, we provide a comprehensive literature review on R-HNCs, emphasizing key histopathological and molecular characteristics that are crucial for guiding treatment decisions. An insight about the latest developments in systemic treatments is also reported.

Immunohistochemical Evaluation of p300, H2AacK5 and H3AcK27 in Odontogenic Cysts and Tumors.

The acetylation of histones H2A on lysine 5 (H2AacK5) and H3 on lysine 27 (H3AcK27) modulate several cellular mechanisms through the p300 enzyme in pathological lesions; however, their role in odontogenic lesions has not been addressed. This study aims to evaluate the immunoexpression of p300, H2AacK5, and H3AcK27 in samples of ameloblastoma (AMB) (n = 30), odontogenic keratocyst (OK) (n = 15), adenomatoid odontogenic tumor (AOT) (n = 10), odontogenic fibroma (OF) (n = 8), calcifying odontogenic cyst (COC) (n = 8), odontogenic myxoma (MIX) (n = 10), and ameloblastic fibroma (AF) (n = 06). The percentage of p300-positive cells was higher in AOT and decreased in COC, OK, AMB, AF, OF, and MIX. H2AacK5-positive cells were higher in AF and decreased in AOT, COC, OK, OF, AMB, and MIX, whereas H3acK27-positive cells were higher in AOT and decreased in COC, OK, AF, OF, AMB, and MIX. The expression of these proteins was higher in nonaggressive lesions in comparison to aggressive lesions. There was a positive correlation between p300 and H2AacK5, and H3acK27 in AMB, MIX, and OF, whereas there was a positive correlation between p300 and H2AacK5 in AOT and COC. The histone acetylation may be involved in the biological behavior of these lesions, which could be used to improve their diagnosis and treatment.

Mandibular Defects and Reconstruction Post Oncological Resection in Paediatric Patients

Introduction: Tumours of the paediatric mandible are rare clinical entities, which require a multidisciplinary team management. Surgery is frequently utilized as a modality for local control in the management of paediatric mandibular neoplasms. However, mandibular reconstruction in such age group is more challenging than in their adult counterparts, owing to the developing nature of the mandible and the craniofacial skeleton. Materials & Methods: The medical records of patients below the age of 18 years of age presented to the Paediatric Oncology and Head and Neck Surgical Oncology Departments at the National Cancer Institute of Cairo University from January 2014 to January 2024 with mandibular tumour were reviewed. The review includes; radiological site of the lesion, histo-pathological diagnosis, multidisciplinary team management. Patients that underwent segmental mandibular resection were evaluated for the type of the resultant mandibular defects according to Brown’s classification of mandibular defects and the subsequent reconstruction procedures. Results: Forty-two patients were reported with mandibular neoplasm. Primary mandibular tumours were found in 35 cases (83.3%), of which non odontogenic tumours were diagnosed in 29 cases (69.0%). The anatomical subunits of the mandible; ramus, angle and body were involved in 14 (33.3%), 13 (31.0%) and 15 (35.7%) cases, respectively. Twenty-nine patients underwent segmental mandibular resection. According to Brown’s classification; Class I, Class Ic, Class II, Class IIc, Class III, Class IV and Class IVc were the resultant defects in 0 (0%), 2 (6.9%), 10 (35.5%), 10 (35.5%), 3 (10.3%), 0 (0%), and 4 (13.8%) cases, respectively. Conclusion: The different pathologies of the mandibular neoplasms affecting the paediatric age group frequently involve the ramus of the mandible. With the surgery being the modality of choice in most of such pathologies, the resultant defects following segmental resection and the reconstruction approach in such age group vary greatly from the adult age group. Hence, the free fibular bone segments used for restoration of the ramus projection would be of a relatively larger length. Condylar resection, unfortunately, would hinder the restoration of occlusion relationship, essential for normal development of the maxillofacial skeleton.

Primordial Odontogenic Tumor: A Decade Post-Description Systematic Review.

Primordial odontogenic tumor (POT) is a rare benign tumor arising from odontogenic epithelium and ectomesenchyme. It typically presents in children and young adults. POT is often found in the posterior mandible and frequently presents as asymptomatic swelling. A systematic review of the literature was conducted to comprehensively analyze the clinicopathologic features of this rare entity over the past ten years. A systematic review of POT case series and case reports following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement guidelines was performed. Data on demographics and clinical characteristics, including age, sex, clinical presentation, duration of the lesion, location, and radiographic and histological features, were extracted. A quantitative description of immunohistochemical studies reported in the literature was also performed. Treatment, a follow-up period, and recurrence were collected for analysis. This review included 26 studies involving 36 POT cases. Patients often presented with asymptomatic swelling at a median age of 12 years, with a male-to-female ratio of 1.18:1. The posterior mandible was the most commonly affected site, while three cases were noted in the anterior region exclusively in the maxilla. Most lesions appeared as unilocular radiolucencies with well-defined borders; however, five cases exhibited fine trabeculation or radiopacities. The primary histological features observed in POT included ectomesenchymal stroma lined by columnar cells with nuclear reverse polarity. Most cases were treated through enucleation and curettage (50.0%), followed by tumor excision (36.1%). Only one case demonstrated recurrence among the 29 cases with known follow-up information. This study offers comprehensive and current descriptive data on POT, enhancing the ability of clinicians and pathologists to accurately identify these rare lesions and thereby avoid misdiagnosis and inappropriate management.

Odontogenic myxoma of the maxilla: a case report

BackgroundOdontogenic myxomas are rare tumours of the mesenchymal portion of developing tooth and account for 3–6% of all odontogenic tumours. Microscopically, odontogenic myxomas have a characteristic histologic appearance, which distinguishes it from other odontogenic tumours. However, it can mimic normal dental follicles of a developing tooth, from which it can be differentiated histologically by the nature of connective tissue and also by clinical and radiological appearances.Case presentationWe present a case of a 30-year-old female with a large mass involving the left maxillary sinus. Histopathology showed features of odontogenic myxoma.DiscussionEarly diagnosis of such lesions is critical for avoiding extensive surgical procedures and the subsequent impact on the patient’s quality of life.ConclusionComplete surgical excision is the treatment choice, which can be difficult owing to the tumour’s invasive borders. Hence, the patients should benefit from regular follow-ups.

Diagnostic dilemmas and multidisciplinary management of glandular odontogenic cyst: A case report

Glandular odontogenic cyst (GOC) is a rare odontogenic cyst typically found in the anterior mandible and, in rare cases, the anterior maxilla, characterized by asymptomatic, slow-growing swelling. It mimics other odontogenic cysts and tumors, complicating the diagnosis. Histologically, GOC features mucus-producing cells and gland-like structures, initially suggesting a salivary gland origin. However, the absence of salivary gland markers and the presence of hybrid lesions with other odontogenic tumors confirm its odontogenic nature. Known for its aggressive behavior, GOC can cause significant growth and bone perforation, leading to high recurrence rates. This case report discusses a 34-year-old male with a recurrent cystic lesion initially misdiagnosed as a periapical abscess. Accurate diagnosis and successful management involved root canal treatment and surgical intervention, highlighting the importance of precise diagnosis and prompt treatment.

Epidemiological Features of 4777 Cysts and Odontogenic Tumors Based on the 2022 WHO Classification.

The epidemiology of cysts and odontogenic tumors is important for differential diagnosis and treatment strategies. We aimed to describe the epidemiological features of cysts and odontogenic tumors in the Chilean population using the current WHO classification. We reviewed 22,914 biopsy requests received between January 1984 and September 2023 at the oral pathology department, School of Dentistry, Universidad Mayor, Santiago, Chile. Patients diagnosed with cysts of the jaws and odontogenic tumors were selected and information regarding age, sex, and location was recorded. 4226 (18.4%) were cysts, and 551 (2.4%) were odontogenic tumors, ranging from 2 to 97 years old. Males represented 54.4% and females 45.7% of the total sample. The most prevalent cysts were radicular cysts (58.6%), dentigerous cysts (17.9%), and odontogenic keratocysts (13.3%). The most prevalent odontogenic tumors were odontomas (40.1%) and conventional ameloblastoma (17.6%). Our study was the first retrospective analysis to determine the epidemiological features of both cysts and odontogenic tumors together, based on the 2022 WHO classification. This is relevant as it offers a potential basis for comprehensive comparisons of the epidemiological features of these entities, which could contribute to an accurate differential diagnosis, therefore, leading to more effective therapeutic interventions.

Audit of oral neoplasms in children and young adults in Nigeria

BackgroundOrofacial neoplasms in children and young adults may differ significantly from those observed in adults. Our aim was to describe the epidemiological characteristics of histologically diagnosed orofacial neoplasms among children and young adults in Nigeria.MethodsThis was a multicenter cross-sectional study across geopolitical zones in Nigeria. Annual reports of clinical information and surgical biopsies submitted at the Oral Pathology Laboratory, clinic day registries, surgical day case registries and operative theatre registries were retrieved from January 2008 to March 2024. The relevant demographic data were obtained for each patient. The study subjects were categorized by age into children, adolescents and young adults. Tissue involvement was classified as soft tissue involvement, bony involvement or both soft tissue and bony involvement. The site and behaviour of the lesions were subdivided according to the ICD-10 codes. Statistical analysis was performed via the R programming language.ResultsA total of 1889 cases were observed during the period under review, with a mean age of 15 years. Cases were more common in females (52%) and in young adults (47%). Most cases were benign neoplasms (85%), and bony affectation (54%) was slightly predominant. Odontogenic tumours (38%) and fibro-osseous lesions (20%) were the most common category of lesions observed, whereas salivary gland tumours (2.2%) and neoplasms of epithelial origin (2.5%) were the least common. Neoplasms in children involved mostly soft tissues, whereas those in adolescents and young adults had a preference for bone (p < 0.001). In all age groups, benign lesions were mostly observed in the mouth and pharynx. For malignant lesions, in children, the bones of the skull and face were mostly involved, whereas in adolescents, the mandible was the predominant site (p < 0.001). In children, mesenchymal neoplasms were the most prevalent category of lesions, whereas in both adolescents and young adults, odontogenic tumours were more common. The proportion of malignant neoplasms in males was significantly greater than that in females (p < 0.001).ConclusionThis study revealed that although most biopsied orofacial lesions were more often benign, the proportion of malignant neoplasms in this population was greater than that previously reported.

A Challenging Case Of Giant Maxillary Ameloblastoma: Navigating Rarity And Complexity In Diagnosis And Treatment

Background: Ameloblastoma is a rare, aggressive, benign odontogenic tumor that most commonly occurs in the jaw. Maxillary occurrences are rarely reported. Case Presentation: We report the case of a 61-year-old male presenting with right-sided unilateral nasal obstruction, characterized by a mass occupying the entire right nasal fossa upon anterior rhinoscopy. A computed tomography (CT) scan, combined with magnetic resonance imaging (MRI), revealed a locally advanced mass centered on the right maxillary sinus. The patient underwent wide excision of the mass, and histopathological analysis confirmed the diagnosis of ameloblastoma. Discussion: Naso-sinus ameloblastomas are rare, benign, and slow-growing tumors, accounting for approximately 0.11% of nasal and sinus tumors. More common in men, they typically present between the ages of 20 and 30. Symptoms include nasal obstruction and epistaxis, with occasional facial swelling or headaches. These tumors often originate in the posterior maxillary region and extend into the maxillary sinuses and nasal cavity. Diagnosis is facilitated by imaging, where the tumors appear as solid, radio-opaque masses. Radical surgical resection is the preferred treatment, as it is essential for preventing recurrence. The prognosis is generally favorable, with no documented cases of malignant transformation or metastasis, and recurrences are rare when appropriate surgical management is performed. Conclusion: Medical imaging plays a crucial role in diagnosis and allows for the evaluation of tumor dissemination and anatomical involvement. The treatment of maxillary ameloblastoma typically involves surgical excision, with the complexity of management often linked to the extent of tumor spread.

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.

Odontogenic tumors in pediatric dentistry

Odontogenic tumors in pediatric dentistry

Odontogenic myxoma: A clinicopathological study over 15 years and immunohistochemical analysis

Objectiveand rationale: Odontogenic myxoma is an uncommon odontogenic tumor with locally aggressive behavior. The clinicopathological studies of odontogenic myxoma in Asian countries are very limited and only few studies have investigated the immunohistochemical profiles of the tumor. This study aims to investigate the clinicopathological and immunohistochemical features of odontogenic myxoma at the Faculty of Dentistry, Mahidol University over a 15-year period. MethodsArchives of our institute were reviewed. Cases diagnosed as odontogenic myxoma were retrieved. Demographic, clinical, radiographic, and histopathological features of these cases were analyzed. In addition, immunohistochemical markers including vimentin, Ki-67, Bcl-2, and CD117 were performed. The correlation between immunohistochemical profiles and clinicopathological characteristics was evaluated. ResultsSixteen cases of odontogenic myxoma were discovered. Fourteen cases were central type while two cases were peripheral type. The mean age of patients was 34.6 years with male-to-female ratio of 1:2.2. Mandible (68.8 %) was more affected than the maxilla (31.2 %). Bony expansion or jaw swelling (43.8 %) was the most common clinical feature. Most cases (71.4 %) presented with multilocular radiolucency. Histopathologically, tumors show stellate and spindle-shaped cells in a myxoid stroma with varying amounts of collagen fiber. All cases were positive for vimentin and Bcl-2. Half of the cases showed positive for Ki-67. Mast cells were presented in most cases (75.0 %). A significant correlation was found between the immunoexpression level of Bcl-2 and border of lesion in radiograph (p = 0.024). ConclusionsThis study contributes to better understanding of the characteristics of odontogenic myxoma. Clinicians and pathologists should be aware of odontogenic myxoma, as its clinical and histopathological features may overlap with other tumors. The expression of Bcl-2 and presence of mast cell in this tumor may relate to its growth and aggressiveness. Despite its benign nature, odontogenic myxoma exhibits high recurrence, especially in lesion managed conservatively.