Recurrence and Prognosticators of Recurrence in Odontogenic Keratocyst of the Jaws.

Introduction: The incidence of recurrence of OKC varied from 2.5 to 62%. Studies have linked recurrence to treatment methods and also clinical and pathological features. The aim of this study was to evaluate the 5-year recurrence and the factors associated with recurrence in odontogenic keratocysts of the jaws. Methods: A retrospective review of records was done from the Institute's Medical Records Directory from 2010 to 2021. The following data were obtained of the lesion; age at presentation, gender, site, subsite, radiographic presentation (locularity), radiographic borders, presence or absence of satellite cysts, inflammatory infiltrate, and treatment rendered presence or absence of cortical perforation and soft-tissue extension and presence or absence of recurrence. Kaplan Meir estimator was used to evaluate recurrence rate and log rank test was used to compare the survival amongst groups. Cox regression analysis was used to evaluate the odds ratio to find out the possible factors influencing risk of recurrence. A p value of < 0.05 was considered statistically significant at 95% confidence interval. Results: In our study cohort, 27.2% of patients had recurrence. Posterior maxillary lesions, multilocular lesions, lesions with scalloped borders, presence of soft-tissue extension and cortical perforation, presence of satellite cysts and inflammatory infiltrate and enucleation with peripheral ostectomy were significantly associated with recurrence. However, soft-tissue extension, cortical perforation, multilocular lesions and presence of satellite cysts were independent risk factors. Conclusion: There is still debate on the best treatment modality for the management of OKCs. More studies are required to quantify the results.

Exceptional Evolution of a Squamous Odontogenic Tumor in the Jaw: Molecular Approach.

A squamous odontogenic tumor (SOT) is an epithelial locally benign neoplasia derived from the periodontium of the jaws. It is considered a lesion of low incidence. Predominantly, it affects the mandible, although both jaw bones may be involved. Here, we discuss the malignant clinical evolution of an SOT lesion in an 80-year-old female patient. The patient exhibited an expansive triangular lesion at the inferior right quadrant. Surgery was performed and an SOT was diagnosed (2019). Two years after, the lesion grew, and the analysis of the biopsy revealed SOT malignization with pleomorphic atypical squamous cells, characteristics of a squamous cell carcinoma (2021). Massive DNA sequencing of formalin-fixed-paraffin-embedded specimens of the initial and relapsed tumors indicated pathogenic mutations in RET and POLE genes in both tumors, loss of ALK, and gain of CDKN1B and MAP2K in the relapse. In addition, the clinical, radiographic, and microscopic features of this neoplasm are discussed and compared with those already published. The case presented contributes to the better understanding of this SOT tumor entity and to indicates its malignant evolution, together with its biological behavior and its histologic, clinical, and radiographic features. Also, it aims to stress the importance of deeper genetic analyses in rare diseases to uncover mutations that help to select a personalized treatment.

Cone-beam computed tomography imaging of benign odontogenic lesions in the maxilla: A report of 2 cases.

Odontogenic tumors are slow-growing and generally nonaggressive; however, aggressive characteristics appear to exist in particular tumors. The author reports two cases of benign odontogenic lesions at the anterior maxilla. A 44-year-old woman came to an oral-maxillofacial surgeon with a complaint of asymptomatic swelling of the left anterior region of the maxilla 15 years ago, which started gently but gradually increased over time. The patient experienced a physical injury 15 years ago, resulting in the loss of the upper left front teeth. In the other case, a 50-year-old woman complained for 5 years about a little swelling in her left anterior maxilla that became larger over time without causing any discomfort or pain. The Cone-beam computed tomography (CBCT) exam revealed a partially multilocular radiopaque mixed radiolucent lesion in the anterior maxilla, with margins that are both well-defined and ill-defined. Both display features of infiltrative, expansive, and moderately aggressive growths, leading to the erosion and perforation of the cortical plates in the buccal and palatal regions. Based on the biopsy results, both samples showed similar findings, specifically a benign odontogenic lesion, likely desmoplastic ameloblastoma, and squamous odontogenic tumor without any atypic cells or malignancy. This study aims to further our understanding of the clinical and radiological features of these patients and emphasize the importance of investigating specific lesions as potential diagnoses.

Recurrent Aggressive Cementoblastoma - A Rare Case Report.

Cementoblastoma is an odontogenic tumor of benign nature. It is of mesenchymal origin with a relatively low incidence of reappearance. Hereby we present a case report of a 14-year-old male patient with recurrent swelling on the right mandibular posterior region. Despite surgical removal of the tumor mass along with the right first molar, recurrence of the lesion was noticed. Hence, our report gives an insight on the treatment and management of the lesion by en-bloc resection on the right side of the mandible.

A calcified epithelial odontogenic tumor with extension to the maxillary, ethmoid and sphenoid sinuses.

Calcified epithelial odontogenic tumor (CEOT) with extension to the maxillary, ethmoid and sphenoid sinuses is currently unreported. This extension poses a challenge for effective treatment, as CEOT is a benign neoplasm with contreversial potential aggressiveness. We report a case of CEOT that was previously misdiagnosed as an inverted papilloma. A segmental resection of the part of the tumor responsible for the symptoms was performed. Histology revealed polyhedral epithelial cells within an amorphous eosinophilic hyaline material containing round calcified structures and confirmed the diagnosis of CEOT. Postoperative CT Scan showed the partial resection of the tumoral process. A mixed radiolucent/radiopaque aspect of the remaining CEOT was clearly discernible. The patient was warned that due to the incomplete excision of the tumoral process, the risk of recurrence was significant and that a close clinical and radiological follow-up every 6 months was mandatory for a long time.

Follicular Adenomatoid Odontogenic Tumor: A Case Report.

Adenomatoid odontogenic tumor (AOT) is an infrequent odontogenic tumor that typically occurs in adolescent females, usually in the anterior maxilla. There is a controversy about it being a tumor or a hamartoma. It presents clinically as a slowly progressive entity that shows a good prognosis with conservative surgical management. It shows three clinicopathological types: follicular, extrafollicular, and peripheral. This article describes a follicular variety of AOT. An 18-year-old female presented with diffuse intraoral swelling in the maxillary anterior region. An intraoral periapical radiograph (IOPA) revealed a single large pericoronal radiolucency related to the impacted right maxillary lateral incisor. Histopathological evaluation confirmed the diagnosis, and the tumor was treated surgically by enucleation.

Primordial Odontogenic Tumor: A Review of the Literature and Report of Three New Patients.

The primordial odontogenic tumor (POT) is a rare mixed odontogenic tumor composed of mesenchymal cells, columnar odontogenic epithelium, and fibromyxoid stroma affecting the posterior mandible of children and adolescents. Herein, we report 3 patients with POT and the clinicopathological features of POT previously reported in the literature. A 12-year-old, 19-year-old, and 4-year-old patient presented an asymptomatic swelling in the posterior maxilla and posterior mandible. Imaging exams revealed radiolucent lesions associated with unerupted teeth. The lesions were surgically removed, and the histopathological examination revealed spindle-to-ovoid mesenchymal cells in a fibromyxoid stroma surfaced by columnar odontogenic epithelial cells with reverse nuclear polarization. Deposition of mineralized tissue was observed. The final diagnosis was POT, and patients did not exhibit signs of recurrence. POT should be included in the differential diagnoses of intraosseous lesions in the posterior mandible in pediatric patients.

MMP13 Expression and Activity Suggest Its Role in Bone Resorption in Ameloblastomas.

BACKGROUND: Ameloblastoma is a locally destructive benign odontogenic tumor. While the neoplastic cells of conventional ameloblastoma can infiltrate the connective tissue and bone, in unicystic ameloblastoma the epithelium is encapsulated. The mechanisms driving ameloblastoma's bone resorption remains unclear. METHODS: RNA sequencing (RNA-seq) was performed in a discovery cohort of conventional ameloblastoma, and pathway enrichment analysis was carried out. mRNA levels of MMP13, a gene associated with bone resorption, were assessed using RT-qPCR in a larger cohort of conventional ameloblastoma and in unicystic ameloblastoma. Zymogram gels and the immunoexpression profile of collagenase 3 (encoded by MMP13 gene) were evaluated as well. RESULTS: Enriched pathways related to bone mineralization and upregulation of MMP13 were observed in ameloblastomas. Collagenolytic activity of collagenase 3 was detected in the tumor lysates. Collagenase 3 immunopositivity was observed in ameloblastomatous epithelium infiltrating the fibrous capsule of unicystic ameloblastoma. At the tumor-bone interface, collagenase 3 expression was detected in stromal cells, osteoblasts, and osteocytes. CONCLUSION: The results indicate a potential involvement of MMP13 in ameloblastoma-related bone resorption and progression.

Adenoid Ameloblastoma: The Newly Recognized Odontogenic Tumor - A Case Report.

Adenoid ameloblastoma is a newly recognized epithelial odontogenic tumor. Herein, we present the case of a 24-year-old male patient who exhibited swelling in the anterior region and right hemi-mandible. Computed tomography demonstrated the presence of a hypodense osteolytic lesion associated with an impacted tooth. Based on the clinical hypotheses of the dentigerous cyst, odontogenic keratocyst, and ameloblastoma, an incisional biopsy was performed, and the diagnosis of ameloblastoma was rendered. A surgical resection of the tumor was performed. Histopathological examination of the specimen revealed typical areas of ameloblastoma associated with ductiform structures and cell proliferation in a solid storiform pattern, features resembling those found in adenomatoid odontogenic tumor. Based on these findings, the diagnosis of adenoid ameloblastoma was rendered. The accurate diagnosis of this locally infiltrative tumor is essential due to its similarity to other odontogenic neoplasms.

Ameloblastoma With the Hybrid Desmoplastic and Plexiform Pattern: A Case Report.

Ameloblastoma is an epithelial odontogenic tumor with a benign nature and demonstrates local aggressiveness. It frequently occurs between the third and fifth decades of life, showing significant gender predilection. While typically displaying a benign growth pattern, it tends to invade and sporadically metastasize locally. Ameloblastoma is predominantly found in the posterior regions. Periodic recur commonly follows insufficient treatment. Hence, conducting thorough identification of tumors and management is crucial to prevent relapse. Complications and improved prognosis are associated with meticulous surgical techniques, regular follow-up care, and early detection of recurrence. This study presented a report of a 19-year-old male with swelling in the left lower jaw, detailing its area of complaint, radiographic findings, histopathologic characteristics, and different treatment approaches. The uniqueness of the case is the hybrid histopathology of ameloblastoma composed of plexiform and desmoplastic variants.

Traversing the Terrain: Potential Pitfalls within the AJCC 8th Edition Staging System for Lip and Oral Cavity Cancers.

In 1977, the American Joint Committee on Cancer (AJCC) introduced the inaugural Cancer Staging Manual, which implemented the T (tumor extent), N (regional lymph node status), and M (presence or absence of distant metastasis) staging system. This systematic approach aimed to convey the extent of disease across various cancer types, providing clinicians with a practical framework to plan treatment strategies, predict prognosis, and assess outcomes. The AJCC 8th edition, effective from January 1, 2018, continues this tradition. However, certain shortcomings persist in the AJCC 8th edition, as identified through clinical experience. Specifically, challenges arise in accurately assessing depth of invasion in unique histological variants of oral squamous cell carcinoma (e.g., Oral verrucous carcinoma, Carcinoma cuniculatum, and Papillary squamous cell carcinoma) and minor salivary gland tumors. Additionally, discrepancies exist in the perception of bone invasion patterns and in reporting practices. There is also a need for staging guidelines for malignant odontogenic tumors and multifocal tumors of the oral cavity, supplemented by diagrammatic representations. Lastly, there is a call for comprehensive staging criteria for carcinomas of the ear, external auditory canal, and temporal bone. We advocate for the inclusion of these considerations in future editions of the AJCC Cancer Staging Manual.

Central dentinogenic ghost cell tumor of the maxilla: a case report with new imaging findings and review of the literature.

A dentinogenic ghost cell tumor (DGCT) is a rare benign odontogenic tumor that commonly shows characteristics of solid proliferation and has a relatively high risk of recurrence after surgical treatment. We herein report a case of a central DGCT that occurred in the maxilla and resulted in bone expansion. This study highlights new imaging findings (particularly magnetic resonance imaging) along with histopathological observations. In addition, we conducted a review of the existing literature on this rare tumor. A 37-year-old man developed swelling around the right cheek. A benign odontogenic tumor such as ameloblastoma was suspected based on the imaging examination findings (including bone expansion and the internal characteristics of the tumor) on panoramic imaging, computed tomography, and magnetic resonance imaging. The lesion was surgically excised from the right maxilla. Postoperative histopathological examination led to a definitive diagnosis of central DGCT. The tumor comprised epithelial neoplastic islands, resembling ameloblastoma, inside tight fibroconnective tissue; masses of ghost cells and formation of dentin were also observed. We had suspected that the minute high-density region around the molars on the imaging examinations represented alveolar bone change; however, it represented dentin formation. This led to difficulty diagnosing the lesion. Although DGCT may present characteristic findings on imaging examinations, its occurrence is infrequent, and in some cases, the findings may include the presence or absence of an impacted tooth without obvious calcification. The present case suggests that we should consider the possibility of an odontogenic tumor with calcification when high-density structures are observed inside the lesion.

Conservative enucleation for physiologic space closure in adenomatoid odontogenic tumor.

Adenomatoid odontogenic tumor (AOT) is a rare, asymptomatic, slow-growing benign tumor that can be divided into three variants: follicular, extrafollicular, and peripheral. By treating AOT using an enucleation and curettage approach, recurrence can be avoided. We report a case of a 24-year-old female who presented with a lump in the right mandibular premolar area along with diastema between displaced teeth #43 and #44 and was diagnosed with extrafollicular AOT. The patient was managed with enucleation-curettage surgery without additional bone graft procedure along with routine follow-up. A successful outcome without recurrence was achieved, and diastema closure with repositioning of the displaced teeth did not require orthodontic treatment. AOT should be managed via enucleation and curettage to obtain successful outcomes without recurrence. Spontaneous bone regeneration following enucleation can be achieved without guided bone regeneration. Also, diastema closure and repositioning of displaced teeth can occur without orthodontic interventions through physiologic drift.

Ameloblastic fibrosarcoma of the maxilla arising in an old woman, a rare case report and literature review.

BACKGROUND: Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults and typically affecting the mandibular region. We report an exceptionally rare and highly atypical case of AFS in an elderly female patient originating from the maxillary bone. CASE PRESENTATION: A 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up. CONCLUSION: Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.

Odontogenic carcinoma with dentinoid: case report and literature review of a rare entity.

BACKGROUND: Odontogenic carcinoma with dentinoid (OCD) is a rare and controversial entity, which has not yet been included in the current World Health Organization classification of odontogenic lesions. Owing to the small number of reported cases, the clinicopathological characteristics, biological behavior, prognosis, and appropriate treatment strategies for OCD remain to be defined. Herein, we present an additional case of OCD with a focus on the differential diagnosis and review of the pertinent literature, in order to enable better recognition by oral clinicians and pathologists and further characterization of this entity. CASE PRESENTATION: This paper reports a case of OCD in the posterior mandible of a 22-year-old female. Radiography showed a well-defined unilocular radiolucency with radiopaque materials. The intraoperative frozen section pathology gave a non-committed diagnosis of odontogenic neoplasm with uncertain malignant potential. Then a partial mandibulectomy with free iliac crest bone graft and titanium implants was performed. Microscopically, the tumor consisted of sheets, islands, and cords of round to polygonal epithelial cells associated with an abundant dentinoid matrix. Immunohistochemically, the tumor cells were diffusely positive for CK19, p63, and ß-catenin (cytoplasmic and nuclear). No rearrangement of the EWSR1 gene was detected. The final diagnosis was OCD. There has been no evidence of recurrence or metastasis for 58 months after surgery. We also provide a literature review of OCD cases, including one case previously reported as ghost cell odontogenic carcinoma from our hospital. CONCLUSIONS: OCD is a locally aggressive low grade malignancy without apparent metastatic potential. Wide surgical excision with clear margins and long-term period follow-up to identify any possible recurrence or metastases are recommended. Histopathological examination is essential to conclude the diagnosis. Special care must be taken to distinguish OCD from ghost cell odontogenic carcinoma and clear cell odontogenic carcinoma, as misdiagnosis might lead to unnecessary overtreatment. Study of additional cases is required to further characterize the clinicopathological features and clarify the nosologic status and biological behavior of this tumor.

Diagnostic Utility of EWSR1 in Clear Cell Odontogenic Carcinoma: A Systematic Review.

In the 2022, World Health Organisation classification of odontogenic tumours, the clear cell odontogenic carcinoma is designated as a malignant odontogenic tumour with high recurrence and aggressive behaviour. Deceptive behaviour in the context of a wide range of differentials presents a significant diagnostic problem. It is the fifth most commom type of malignant odontogenic tumor. A systematic assessment of published cases, case series, and retrospective investigations of diagnostic significance of EWSR1 gene in clear cell odontogenic carcinoma is presented to determine trends in presentation, diagnostic characteristics, treatment, and patient outcome. To locate papers reporting clear cell odontogenic carcinoma and EWSR1, extensive database searches were carried out. Demographics, tumour location, immunohistochemical and molecular tests, treatment, follow-up, and recurrence were the variables. 34 cases were detected; 52.9% (n = 18) of the cases were females. The average age was 62.5 years, with a range of 43-82 years. The average size ranged from 3.4 to 8 cm. The mandibular body was the most common location, followed by the maxilla. Maximum immunohistochemistry positivity revealed by CK 19, CKAE1/3, EMA and p63. Most common gene fusion detected was EWSR1-ATF1 in 62.4% of cases contributing to its diagnostic attributes. Surgical treatment was used in 97% of cases. The average follow-up period was 30.3 months, and recurrence was reported in 52.4% of the cases. CCOC can metastasize, and the prognosis is fair. This is first systematic review, where we have attempted to consolidate the mutational expression of EWSR1 in Clear cell odontogenic carcinoma. It is difficult to identify from other clear cell tumours of the head and neck region. It is crucial to distinguish it from other clear cell lesions because of its aggressiveness.

Targeted therapies in ameloblastomas and amelobastic carcinoma-A systematic review.

Targeted therapy has the potential to be used in the neoadjuvant setting for odontogenic tumors, reducing the morbidities associated with major surgery. In this regard, the aim of this study was to summarize the current evidence on the different forms of targeted therapy, effectiveness, and drawbacks of this course of treatment. Four databases were searched electronically without regard to publication date or language. Grey literature searches and manual searches were also undertaken. Publications with sufficient clinical data on targeted therapy for odontogenic tumors were required to meet the criteria for eligibility. The analysis of the data was descriptive. A total of 15 papers comprising 17 cases (15 ameloblastomas and 2 ameloblastic carcinomas) were included. Numerous mutations were found, with BRAF V600E being most common. Dabrafenib was the most utilized drug in targeted therapy. Except for one case, the treatment reduced the size of the lesion (16/17 cases), showing promise. Most of the adverse events recorded were mild, such as skin issues, voice changes, abnormal hair texture, dry eyes, and systemic symptoms (e.g., fatigue, joint pain, and nausea). It is possible to reach the conclusion that targeted therapy for ameloblastoma and ameloblastic carcinoma may be a useful treatment strategy, based on the findings of the included studies.

Tumor microenvironment of ameloblastoma with a focus on osteoclastogenesis, cell migration, and malignant transformation.

BACKGROUND: Odontogenic tumors arise in the jawbone and originate from cells associated with tooth development. Therefore, understanding odontogenic tumors requires knowledge of all aspects of dental research, including tooth development and eruption. Ameloblastoma is the most common odontogenic tumor. HIGHLIGHT: Although a benign tumor, ameloblastoma progresses with marked jawbone resorption. Because of its locally aggressive features, it can be treated surgically by resecting the surrounding bone. From a molecular pathology perspective, several genetic mutations and dysregulated signaling pathways involved in ameloblastoma tumorigenesis have been identified. Histopathologically, ameloblastomas consist of peripheral ameloblast-like cells and an inner stellate reticulum. The stromal region consists of fibrovascular connective tissue, showing a characteristic sparse myxoid histology. In general, the tumor microenvironment, including the surrounding non-tumor cells, contributes to tumorigenesis and progression. In this review, we focus on the tumor microenvironment of ameloblastomas. In addition, we present some of our recent studies on osteoclastogenesis, tubulin acetylation-induced cell migration, and hypoxia-induced epithelial-mesenchymal transition in ameloblastomas. CONCLUSION: Further research on ameloblastomas can lead to the development of new treatments and improve patients' quality of life.

Unusual Association of Calcifying Cystic Odontogenic Tumor with Compound Odontoma and Ameloblastoma: Case Report and Review of the Literature.

Calcifying cystic odontogenic tumor (CCOT) is a rare odontogenic cyst accounts for <2% of all odontogenic cysts. Simple unicystic CCOT is commonly encountered accounts for 65%, whereas other forms of CCOT are <3%. CCOT is associated with two or more odontogenic tumors, it is called combined odontogenic lesions or hybrid lesions. The aim of the present article is to report a rare case of CCOT and highlighting the clinical feature and treatment aspects of combined lesions. A 25-year-old female complained of pain and swelling in the left maxilla for 6 months. Examination revealed diffuse swelling in the maxilla, firm in consistency, and tender on palpation. Panoramic radiograph showed impacted canine with multiple radiopaque structures and well-defined lesion in the maxillary sinus. The lesion was surgically removed and histopathologically diagnosed as CCOT with compound odontoma and ameloblastoma. Pub Med database search from 1978 to June 2020 revealed only three case reports of combined odontogenic tumors associated with CCOT.

Ameloblastic Carcinoma of the Maxilla With Uncommon Sinus Manifestations.

Ameloblastic carcinoma (AC) represents a distinct challenge in the realm of odontogenic malignancies due to its rarity and aggressive nature. We present a unique case of AC in a 70-year-old male, retired dry cleaner, with symptoms initially suggestive of chronic allergic rhinitis and recurrent acute sinusitis with asymmetric facial edema and paresthesia. Detailed evaluation revealed a prominent mass in the right maxillary sinus with extensive cortical destruction. Pathological assessment post-right maxillectomy identified a high-grade AC with malignant spindle cell transformation. The patient underwent subsequent interventions, including neck dissection and radiation therapy. Twelve months post-presentation, the patient was recovering appropriately without evidence of recurrence of malignancy. This case highlights the diagnostic challenges posed by AC as well as its unique presentations emphasizing the importance of a comprehensive approach and multidisciplinary management. It also raises considerations about potential chemical exposure implications in AC development.