Ameloblastic fibro-odontoma (AFO) is a rare, slow-growing neoplastic lesion classified as a benign, epithelial odontogenic mesenchymal tumor. This tumor exhibits histological features characteristic of both ameloblastic fibromas and complex odontomas. The clinical manifestation of AFO is typically characterized by the asymptomatic enlargement of the jawbones. Radiographically, it presents as a distinct radiolucent region, indicating the presence of radiopaque substances with varying degrees of irregularities in size and morphology. Standard therapeutic intervention involves enucleation. Despite its benign nature, AFO can cause significant morbidity if left untreated. Therefore, prompt diagnosis and appropriate management are essential to ensure optimal patient outcomes. The present study describes the case (clinical presentation and management) of an 18-year-old male patient with an AFO lesion located in the posterior mandible. This particular case was treated with conservative measures involving surgical enucleation along with the extraction of the impacted tooth and the curettage of residual bone.

BACKGROUND: The BRAF p.V600E genetic variant facilitates the pathogenesis of various tumors by triggering tumor proliferation and progression. The aim of this study was to analyze the prevalence of BRAF p.V600E in benign mixed epithelial and mesenchymal and malignant odontogenic tumors. In addition, we discussed the different detection methods used to assess for aberrant BRAF.
METHODS: This systematic review followed the PRISMA guidelines and was registered in Prospero (CRD42023445689). A comprehensive search of the PubMed/MEDLINE, Scopus, Web of Science, and Embase electronic databases was performed to answer the question \"What is the prevalence of the BRAF p.V600E mutation in benign mixed and malignant odontogenic tumors?\" The methodological quality of the selected studies was assessed using the JBI\'s Critical Appraisal Tool.
RESULTS: Initially, 387 records were identified, but only 11 articles met the inclusion criteria. A total of 70 patients with benign mixed epithelial and mesenchymal odontogenic tumors and 63 with malignant odontogenic tumors were included in the analysis. We found that the BRAF p.V600E mutation had a prevalence of 31.42% in mixed tumors and 26.98% in malignant odontogenic tumors. Moreover, immunohistochemistry showed high concordance with DNA-based molecular methods.
CONCLUSIONS: In general, the BRAF p.V600E variant exhibited a prominent prevalence in mixed and malignant odontogenic tumors. However, most of the findings are based on small cohorts of patients and further studies with larger cohorts are needed.

UNASSIGNED: Odontogenic lesions composed of two or more separate odontogenic cysts are uncommon. The calcifying odontogenic cyst (COC) and odontogenic keratocyst (OKC) are odontogenic cysts that exhibit some neoplastic features. Concurrence of these lesions is extremely rare. We report a rare case of COC combined with OKC in a patient and review the relevant literature systemically.
METHODS: In this article, we present a case report of an 18-year-old boy patient with no medical history whose chief complaint was facial asymmetry. A painless swelling in the right side of the posterior mandible was observed. Histopathological and radiographic investigations revealed a combined odontogenic lesion composed of inflamed OKC with COC.
UNASSIGNED: Long-lasting debates on the true nature of OKC and COC, whether cystic or neoplastic, has been existed so far. Their genetic-derived mechanisms can justify the concurrent occurrence of these cysts in a patient. The treatment plan for this lesion included decompression followed by enucleation, curettage, and application of Carnoy\'s solution.
CONCLUSIONS: We reported a rare case of an inflamed OKC with COC-like features. Based on the literature, the coincidence of these two odontogenic cysts has been reported for the third time here.

Primordial odontogenic tumor (POT) is a rare mixed odontogenic tumor composed of primitive ectomesenchyme similar to the dental papilla. The outer surface consists of columnar/cuboidal odontogenic epithelium similar to the inner enamel epithelium, and there is no hard tissue formation. Until now, 27 cases have been reported in the English literature. This article describes the clinicopathological characteristics of one case of POT, representing the oldest patient (aged 26 years) reported to date.

Odontogenic carcinosarcoma (OCS) is a rare malignant odontogenic tumor (OT) with only a few cases reported in the literature. Its synonyms are ameloblastic carcinosarcoma, malignant mixed OT. It is characterized by a true mixed tumor showing malignant cytology of both epithelial and mesenchymal components. The tumor invaded into adjacent tissues by destroying the bone. A 24-year-old patient visited the outpatient clinic of GITAM Dental College and Hospital, with a chief complaint of growth in the lower right back tooth region for 6 months. Based on clinical and radiographic features, it has been diagnosed as an aggressive central jaw lesion. The patient was further referred for histological examination for confirmatory diagnosis. It has been diagnosed as an adenomatoid OT. The OCS most commonly affects the posterior part of the mandible. A larger number of cases were reported recently, and prolonged follow-up is needed to further clarify the nature of OCS.

Ameloblastic fibro-odontoma (AFO) is a relatively rare, benign noninvasive mixed odontogenic neoplasm derived from epithelial and ectomesenchymal elements of the dental tissues. It usually presents with a mean age of 11.5 years and in the posterior segment of the mandible. It is extremely rare in the posterior maxilla. Although the latest WHO edition classified AFO as developing odontoma, here we present a locally aggressive AFO in a 21-year-old male involving the posterior maxilla and sinus with bone destruction. The patient presents with a two-year history of slowly progressive left facial swelling with malodorous drainage. The CT scan revealed a 5.5 x 4.3 cm well-circumscribed expansile mass with mixed attenuation and peripheral calcification occupying the left maxilla and sinus with bone destruction of the hard palate and orbital rim. According to the literature, most of the AFO cases were treated adequately through a conservative approach with just enucleation or surgical curettage. To our knowledge, our case is the first case treated aggressively with left maxillectomy, palatectomy, and reconstruction surgery because of its radiologic findings, which suggested a locally invasive neoplasm. Histologically, the specimen showed a mixture of proliferative epithelial, mesenchymal tissue elements, and variable amounts of mineralized deposits consisting of enamel matrix and dentinoid deposits, and the final diagnosis was AFO. In conclusion, we present a rare case of AFO with an unusual aggressive presentation, age group, and site involved. The radiographic, histopathologic features, and therapeutic approaches of this unusual locally aggressive tumor are presented with the review of relevant literature.

Developing odontoma is a rare mixed odontogenic tumor that can arise with other odontogenic lesions. The association of COC with ameloblastic fibro-odontoma is extremely rare. We report an extremely rare case of developing odontoma arising from a calcifying odontogenic cyst in a 17-year-old girl.

Ameloblastic fibro-odontoma is a rare tumor affecting the pediatric population and young adults. The World Health Organization (WHO) in 2005 defined it as \"A neoplasm composed of proliferating odontogenic epithelium in a cellular ectomesenchymal tissue with varying degrees of inductive changes and dental hard tissue formation.\" There exists a controversy on its histogenesis designating it as a hamartoma (developing complex odontoma [CO]) or a true neoplasm since both the lesions appear similar histologically. Recently, the WHO in 2017 has clubbed both these lesions as the same entity. Most cases are reported in males and in mandible, while cases in maxilla are scarce. This article describes a recurrence of a previously reported case of ameloblastic fibroma which showed maturation into AFO or CO in a girl aged 6 years in the posterior maxilla. This case is reported due to its rarity and a brief review with differential diagnosis is also discussed.

The World Health Organization (WHO) classification of odontogenic tumors, in its different iterations, has seen several modifications in benign mixed odontogenic tumors. Odontoma is a benign mixed odontogenic tumor that shows dental hard tissue formation to varying degrees. Although odontomas are the most common odontogenic tumors, they are mostly seen in the form of compound odontomas, where the dental hard tissues resemble tooth-like structures. Complex odontoma shows a haphazard mass of dental hard tissues and is generally identified at an older age (mean age of around 20 years). Incidence of complex odontoma in very young children is quite rare, with only around 10 cases associated with the primary dentition being reported in the literature till date. Unlike other odontomas, our case showed an active epithelial component in the form of tooth buds and ameloblastic follicles along with the hard tissues, which made it difficult to distinguish from other similar lesions like ameloblastic fibroma with hard tissue formation and ameloblastoma arising from an odontoma. This article reports the 11th case of complex odontoma associated with the primary dentition, and the youngest such case to be reported in the Indian population.

Ameloblastic fibro-odontoma (AFO) is a benign, epithelial odontogenic tumor with odontogenic mesenchyme having the histologic characteristics of both ameloblastic fibroma and complex odontoma. This report describes the case of a 14-year-old girl with AFO on the right posterior mandibular region that mimics complex odontoma on incisional biopsy due to the presence of atypical dentin- and cementum-like areas. On histological examination, sections of excisional biopsy showed odontogenic epithelial islands with embryonic connective tissue and decalcified sections showed atypical dentin with dentinal tubules and islands of cementum. These features led to the diagnosis of AFO.