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.

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.

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.

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.

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.

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.

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.

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.

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.

An Unusual Case Report of Unicystic Ameloblastoma of the Mandible.

There are plenty of benign lesions that can result in swelling of the mandible, and these can be classified as odontogenic and non-odontogenic lesions. Among the categories of odontogenic lesion, ameloblastoma is the most occurring lesion that takes origin from the epithelial cellular elements and dental tissues in their different stages of development. Ameloblastoma is the most serious odontogenic neoplasm due to its prevalence and clinical characteristics. Ameloblastoma is a broad class which encompasses 80% of solid multicystic type of ameloblastoma with unicystic ameloblastoma (UA) variant included as vital clinicopathological form claiming the rest 20% along with peripheral ameloblastoma variant. UA refers to cystic lesions that seem like jaw cysts clinically, radiographically, or grossly but are lined by typical ameloblastomatous epithelium, with or without luminal and/or mural tumor development, on histologic investigation. Around 5-15% of all ameloblastic lesions do not have a propensity to metastasis, and this is UA. Unicystic mural form, although slow growing overall, is very invasive locally and has a high recurrence rate. As UA tumors show very close features with dentigerous cyst, a very sharp differential diagnosis protocol need to be executed to exclude the other unicystic odontogenic lesions considering the clinical, radiological, and biological characteristics along with proper follow-up and seeing any recurrence of the lesion taking place. Here, we report the case of a twenty-one year male patient with UA of the mandible and review of the literature.

Adenomatoid odontogenic tumor: A histopathologic profile of 43 cases with evidence supporting a mixed odontogenic origin.

BACKGROUND: Adenomatoid Odontogenic Tumor (AOT) accounts for 3% of all odontogenic tumors. It has been classified by WHO as an odontogenic tumor of purely epithelial origin. The current study attempts to establish the origin of the tumor along with detailed histopathological and clinicoradiographic analysis of 43 cases of AOT. MATERIAL AND METHODS: Forty-three cases were reviewed from the departmental archives for demographic data, radiographic features and histological features. Further, histopathological slides were stained with Picrosirius Red (PSR) and observed under polarised light. RESULTS: A majority of the cases were seen in the anterior jaws (76.7%), and were less than 3 cms (76.7%) in greatest dimension. Equal number of cases were of follicular and extra-follicular location while one was peripheral. Predominantly solid histological pattern was noted in 53.5%. Varied sub-patterns were observed with most cases exhibiting solid nodules and strands of tumor cells. Few cases showed melanin pigmentation. Over a third of cases (37.2%) showed dentigerous cyst like areas and one case each showed features of ossifying fibroma and focal cemento-osseous dysplasia. Tumor droplets, hyaline rings within duct-like structures, dentinoid material and osteodentin showed reddish yellow birefringence when observed under polarised microscopy post PSR staining. CONCLUSION: This study highlights the diverse histopathological variation of AOT with evidence to reclassify it as a mixed odontogenic tumor based on the polarising microscopic findings with PSR staining.

Recurrent cementoblastoma with multifocal growth and cellular atypia: a case report.

BACKGROUND: Cementoblastoma is a rare odontogenic tumor characterized by the formation of osteocementum-like tissue on a tooth root directly by neoplastic cementoblasts. Although it is categorized as benign, it has a high potential for growth with a certain degree of recurrence risk. However, there are only a few studies describing the features of recurrent cementoblastoma. The diagnosis of recurrent cementoblastoma is challenging not only due to its cytological atypia but also because of its large size and multicentric growth pattern. These characteristics suggest a potential for malignancy. CASE PRESENTATION: A 29-year-old woman was transferred to our university dental hospital complaining of swelling of the right mandible. She had a history of enucleation of cementoblastoma associated with the third molar of the right mandible. Five years after the initial treatment, imaging demonstrated well-circumscribed multicentric radiopaque lesions in the same area. Histologically, the lesion consisted of osteocementum-like tissue rimmed with polygonal or plump tumor cells. Several cells were large epithelioid cells with bizarre nucleoli, which may be reminiscent of malignant tumors. Otherwise, there were no apparent malignant findings, including proliferative activity or atypical mitotic figure. Besides, tumor cells were positive for c-FOS, a marker of osteoblastoma and cementoblastoma. Eventually, the patient was diagnosed with recurrent cementoblastoma. CONCLUSIONS: Pathological analyses of this case suggested that the recurrent event in the cementoblastoma altered its growth pattern and tumor cell shape. Moreover, in the case of enucleation surgery, long-term follow-up is important because there is some recurrent risk of cementoblastoma, although it is not high.

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.

Surgical treatment of feline inductive odontogenic tumor by marginal resection in a cat.

A 1-year-old mixed-breed cat was referred for an approximately 2-cm mass centered on the upper right canine tooth. Computed tomography (CT) revealed the lesion extended to the nasal cavity and orbit, causing thinning and expansion of the adjacent cortical bone. Excisional biopsy confirmed the diagnosis of a feline inductive odontogenic tumor. Based on the findings of CT imaging, the primary alveolar bone lesion was removed with the tumor, while the adjacent bones, which had been expanded and thinned, were preserved by marginal resection including the surrounding periosteum-like membrane. No local recurrence was observed for seven years. To validate the therapeutic outcome of this case, further research in diagnostic imaging and pathology will be crucial.

Pediatric Odontogenic Cysts and Tumors.

Pediatric odontogenic cysts and tumors are rare and often associated with developing or impacted teeth. Odontogenic cysts are broadly categorized as inflammatory or developmental while odontogenic tumors are classified histologically as epithelial, mesenchymal, or mixed tumors. This article will discuss the presentation, diagnosis, and treatment of odontogenic cysts and tumors in the pediatric population.

Curettage combined with decompression for the treatment of ameloblastoma in children: report of two cases.

BACKGROUND: Ameloblastoma (AM) is the most common benign odontogenic tumor, which is more often detected in the mandible than maxilla, especially the mandibular body and mandibular angle. Pediatric AM is a rare disease, especially in patients aged 10 and younger. Compared with the mainstream osteotomy and reconstructive surgery for adult ameloblastoma, there is more room for discussion in the treatment of pediatric ameloblastoma. The postoperative functional and psychological influence can not be ignored. Especially for children in the period of growth and development, an osteotomy is often challenging to be accepted by their parents. We report two patients with ameloblastoma under 10 years old who are treated with curettage and fenestration, which is a beneficial method for children with ameloblastoma. CASE PRESENTATION: We present two cases of classic ameloblastoma in children. We describe in detail the patients' characteristics, treatment processes, and follow-up result. The bone formation and reconstruction in the lesion area after fenestration decompression and curettage are recorded at every clinic review. The surgical details and principles of curettage and decompression are also described and discussed. The two patients have good bone shape recovery and no recurrence. CONCLUSIONS: Children are in the growth and development period and possess an extremely strong ability of bone formation and reconstruction. Based on the principles of minimally invasive and functional preservation, we believe that curettage combined with decompression can be the first choice for treating AM in children, especially for mandibular lesions.

Cholesterol Granuloma From a Developmental Odontogenic Cyst: A Report of a Rare Case and a Literature Review.

Dentigerous cysts are the second most common developmental odontogenic cysts that develop around the crown of unerrupted teeth with the maxillary canine region being one of the common sites of occurrence. The cystic lining of this lesion has been shown to develop into ameloblastoma, Muco epidermoid carcinoma, and squamous cell carcinomas. However, the development of cholesterol granuloma (CG) in the cystic lining of a dentigerous cyst is extremely rare. CG is a histological observation distinguished by the presence of a conglomeration of connective tissue and granulation tissue. The condition is predominantly seen in the field of otolaryngology, with very few cases reported in the maxillofacial region, most of which are associated with the maxillary sinus. This article presents the findings of a CG in a 39-year-old male patient that developed within the dentigerous cyst and discusses the possible etiopathogenesis, surgical management, and histological presentation.

Histopathologic Evaluation of Periapical Radiolucencies Clinico-Radiographically Diagnosed as Endodontic Lesions: A Retrospective Analysis.

Statement of the Problem: Periapical cyst and granuloma are inflammatory endodontic lesions. Periapical granuloma usually heals spontaneously after endodontic treatment; however, periapical cyst mostly needs to be removed via surgical approaches. Although some clinicians believe that microscopic examination of periapical lesions is unnecessary, it is proved that some of them has non-endodontic nature that need critical consideration. Purpose: The purpose of this study was to assess the disagreement between clinico-radiographic and microscopic diagnosis of periapical cysts and granulomas in a major center of oral pathology service in Iran. Materials and Method: In this retrospective, descriptive cross-sectional study, the archives of the oral and maxillofacial pathology department of Shahid Beheshti University of Medical Sciences served as the source of the material during an 18-year-period for this retrospective, descriptive cross-sectional study. The reports of all patients whose initial clinical diagnosis was a periapical cyst/granuloma were extracted. Results:  In the present study, 474 cases were diagnosed with a periapical cyst/granuloma clinico-radiographically, of which 61 cases (12.86%) received a microscopic diagnosis of a non-endodontic pathology. The most frequent lesion was odontogenic keratocyst (n= 12, 19.67%) followed by infected odontogenic cyst (n= 12, 19.67%). About 21.31% of diagnoses were non-cystic lesions and 4.9% were malignancies. The most odontogenic tumors that were diagnosed as periapical cyst/granuloma in clinico-radiography were the ameloblastoma variants (n= 4, 6.55%). Conclusion:  A wide variety of microscopic diagnoses, including aggressive lesions such as ameloblastoma, as well as other malignant lesions was noted in this study. These misdiagnoses can lead to an inappropriate treatment plan. It is important to microscopically examine all lesions removed from the jaw.

Metamorphosis of Dentigerous cyst: A case Series with Insight into Molecular Profiling.

The objective of this study was to review the clinical, radiologic, and histologic aspects of cases of dentigerous cysts metamorphosing into different entities along with comments on the molecular factors involved in the process. A series comprising 8 cases of dentigerous cysts was performed retrospectively along with a comprehensive literature review. Two cases of dentigerous cyst converting into mucous metaplasia were included, out of which one case was transformed into ameloblastoma after a year. The other two cases were reported as acanthomatous and unicystic ameloblastoma, whereas four transitioned to AOT. The complete review of cases and literature with molecular profiling concluded that the lining of dentigerous cysts has the potential for transforming into benign odontogenic tumors. Therefore, a careful clinical and histopathological examination is crucial for the correct diagnosis. Also, a thorough molecular understanding of the cystic lining is required.