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.
METHODS: 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.
CONCLUSIONS: 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.

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.
METHODS: 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 β-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.

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.
CONCLUSIONS: 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.
CONCLUSIONS: Further research on ameloblastomas can lead to the development of new treatments and improve patients\' quality of life.

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.

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.

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.
METHODS: 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.

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.

A cyst is defined as a pathological cavity which may or may not have an epithelial lining and which has a fluid, semi-fluid, or gaseous contents and is not formed by the accumulation of pus. The calcifying epithelial odontogenic cyst (CEOC) was first reported by Gorlin et al. in 1962. At that time, it was classified as a cyst related to the odontogenic apparatus. It was later renamed as calcifying cystic odontogenic tumor (CCOT) in the World Health Organization classification devised in 2005 due to its histological complexity, morphological diversity, and aggressive proliferation. CCOT was later recognized by numerous names including Gorlin cyst, calcifying ghost cell odontogenic cyst and/or dentogenic ghost cell tumor. It has a peak incidence during the second and third decades of life and does not demonstrate any gender predilection. Radiographically, CEOC may appear as a unilocular or multilocular radiolucent lesion with either well-circumscribed or poorly-defined margins and may also be observed in association with unerupted teeth. Calcification is an important radiographic feature for the interpretation of CEOC/CCOT. The typical histopathological features of CEOC include a fibrous wall and lining of the odontogenic epithelium with either columnar or cuboidal basal cells resembling ameloblasts. The treatment of choice for CEOC is conservative surgical enucleation; however, recurrence is also not found to be uncommon. Herein, we are reporting a case of the same in a 21-year-old female which was a great dilemma during the diagnostic workup.

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.

This case report documents the diagnosis and successful management of a substantial periapical lesion located in the lower left region of the jaw. The patient presented with clinical symptoms indicative of periapical pathology, and radiographic examination revealed an extensive radiolucent lesion. The chosen treatment approach involved endodontic intervention coupled with surgical decompression, leading to the resolution of the lesion and restoration of oral health. This case underscores the significance of an accurate diagnosis and a multidisciplinary treatment approach in addressing large periapical lesions.