Ameloblastic fibrosarcoma (AFS) is considered a malignant progression resulting from dysplastic changes in an ameloblastic fibroma (AF). Both tumors are extremely rare, with only a few cases reported in the scientific literature. Notably, BRAF mutations have been identified in ameloblastomas, suggesting a connection between ameloblastic morphology and BRAF mutations, as AF is believed to be the precursor neoplasm leading to AFS. In this study, we present a case of AFS in a 25-year-old male. The tumor tissue underwent molecular analysis, specifically next-generation sequencing (NGS) using the Oncomine Comprehensive Assay v3 System. The analysis revealed pathogenic mutations in TP53 and RB genes, as well as copy number gains in NTRK1, MDM4, and BRAF. Additionally, we provide a summary of the literature\'s findings from the analysis of 107 previously reported AFS cases. Our findings suggest the existence of a molecularly distinct subtype, emphasizing the importance of comprehensive molecular testing for these patients.

BACKGROUND: Ameloblastic fibro-dentinoma is considered a rare, benign, mixed odontogenic tumor that occurs mainly in the posterior mandible in the 1st-2nd decade of life. Although the clinical behavior of Ameloblastic fibro-dentinoma is similar to that of ameloblastic fibroma, there is a debate about whether Ameloblastic fibro-dentinoma is a developing hamartomatous odontoma or a separate neoplastic odontogenic tumor like ameloblastic fibroma. However, it is important to understand the histopathogenesis of this rare tumor.
METHODS: A case report presenting an 11-year-old male child with a swelling in the posterior mandible. Radiographic examination revealed a multilocular lesion with mixed radiodensity related to the impacted lower left second premolar tooth. Incisional biopsy was done, and microscopic examination revealed cords and nests of odontogenic follicles lined by ameloblast-like cells and central stellate reticulum-like cells in the primitive ecto-mesenchymal stroma with areas of dentinoid material and osteodentin. The diagnosis was ameloblastic fibro-dentinoma. Surgical excision of the lesion was done, and the patient was followed up for 1 year without evidence of recurrence.
CONCLUSIONS: Reporting such a rare entity clarifies the debate about its nature and the importance of early diagnosis of lesions that are associated with unerupted teeth showing how it is effective in early management and prognosis.

BACKGROUND: Ameloblastic fibromas and ameloblastic fibrosarcomas are rare odontogenic tumors, and controversy exists in the classification of cases presenting hard-tissue production: Ameloblastic fibrodentinoma (AFD) and ameloblastic fibro-odontoma (AFO). These cases are currently considered \"developing odontomas\" (hamartomatous lesions).
OBJECTIVE: To analyze the clinicopathologic features of these lesions and discuss the changes in the 2017 World Health Organization classification.
METHODS: An electronic literature search was performed in the PubMed/MEDLINE database. An electronic search of the English language literature was performed and last updated in September 2020 in the PubMed/MEDLINE database using the following terms: \"ameloblastic fibroma\", \"ameloblastic fibrodentinoma\", \"ameloblastic fibro-odontoma\", \"ameloblastic sarcoma\", \"ameloblastic fibrosarcoma\", \"ameloblastic fibrodentinosarcoma\", \"ameloblastic fibroodontosarcoma\" and \"odontogenic carcinosarcoma\". The inclusion criteria were odontogenic tumor series, case reports and systematic reviews that provided sufficient clinical, radiological and microscopic documentation to confirm the diagnosis.
RESULTS: The database search strategy resulted in 947 papers. Articles focusing on other topics, articles that were not in English, duplicate articles, and articles without fulfilling the inclusion criteria were excluded. Finally, 96 publications were included in this review to describe and discuss the main features of the searched entities. Several aspects of AFO and AFD, such as biological behavior, age of occurrence, amount of hard tissue, and potential for malignant transformation into odontogenic sarcomas, support the neoplastic nature in most of the reported cases. Considering the clinical, radiographic, histopathological and molecular characteristics of odontogenic lesions with hard tissue production, we suggest that these types of lesions should continue to be recognized as odontogenic tumors by maintaining the classically used terms.
CONCLUSIONS: This recommendation will be relevant for future clinical, microscopic, and molecular studies to better understand the biology of these interesting odontogenic tumors.

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.

Calcifying odontogenic cyst is an uncommon odontogenic lesion that represents less than 2% of all odontogenic cysts and tumors. It usually occurs in incisor and canine areas during the second to fourth decades of life. It can be associated with other lesions like odontoma, ameloblastic fibroma, ameloblastoma, adenomatoid odontogenic tumors, odontoameloblastoma, and odontogenic myxoma. Ameloblastic fibroma is a truly mixed tumor usually diagnosed within the posterior mandible during the first two decades of life. In the present article, a hybrid odontogenic tumor composed of calcifying odontogenic cyst and ameloblastic fibroma in a 14-year-old white Persian female is described.

Ameloblastic fibrosarcoma (AFS) now designated as odontogenic sarcoma is a malignant odontogenic tumor characteristically composed of a benign epithelium and a malignant mesenchymal component. It can arise de novo without any preexisting lesion or it can result from the malignant transformation of ameloblastic fibroma (AF). Hereby, we report an extremely rare case of odontogenic sarcoma which was transformed from AF over a period of about 2 years. This is the first case to be reported after it has been reclassified as odontogenic sarcoma. A systematic review was also done to evaluate the studies that reported AFS arising de novo and AFS arising from AF. The objective of this study is to systematically review the studies that reported AFS arising de novo and AFS arising from AF. Articles that reported AFS arising de novo and AFS arising from AF were collected from PubMed, Medline, Embase, Cochrane, Google search and manual search. The results of the systematic review showed that six studies (46.1%) reported AFS arised de novo with no previous history of AF. Seven studies (53.84%) reported that amelobastic fibrosarcoma arised from AF. A rare case of odontogenic sarcoma transformed from AF is reported here. This is the first case report to be published on odontogenic sarcoma after the World Health Organization reclassification. AF once diagnosed should be treated immediately without any delay to avoid the chances of its malignant transformation into odontogenic sarcoma.

Ameloblastic fibro-odontoma (AFO) is a rare benign odontogenic tumor with the histologic features of ameloblastic fibroma (AF) but also contains enamel and dentin. It is most commonly observed in the pediatric population. Distinction between AFO and AF becomes important as ameloblastic fibromas are associated with higher recurrence rates of up to 18%, and 35% of these recurrent lesions can undergo malignant transformation to ameloblastic fibrosarcoma. Hence, for amelobastic fibroma, conservative curettage is recommended for the initial lesion and marginal resection is considered for recurrent cases. In contrast, AFO can be treated with simple curettage and the recurrence rate is approximately seven percent. Malignant transformation of AFO is exceedingly rare. Therefore, the treatment and prognosis differs for these two histologically similar neoplasms. We present a case of a 17-year-old boy who was initially diagnosed with ameloblastic fibroma upon biopsy, with subsequent curettage specimen showing AFO, which carries a better prognosis.

OBJECTIVE: To integrate the available data published to date on ameloblastic fibromas (AF) and ameloblastic fibrosarcomas (AFS) into a comprehensive analysis of their clinical/radiological features.
METHODS: An electronic search was undertaken in July 2017. Eligibility criteria included publications having enough clinical, radiological and histological information to confirm a definite diagnosis.
RESULTS: A total of 244 publications (279 central AF tumours, 10 peripheral AF, 103 AFS) were included. AF and AFS differed significantly with regard to the occurrence of patients\' mean age, bone expansion, cortical bone perforation and lesion size. Recurrence rates were as follows: central AF (19.2%), peripheral AF (12.5%), AFS (all lesions, 35%), primary (de novo) AFS (28.8%) and secondary AFS (occurring after an AF, 50%). Larger lesions and older patients were more often treated by surgical resections for central AF. Segmental resection resulted in the lowest rate of recurrence for most of the lesion types. AFS treated by segmental resection had a 70.5% lower probability to recur (OR 0.295; P = .049) than marginal resection; 21.3% of the AFS patients died due to complications related to the lesion.
CONCLUSIONS: Very long follow-up is recommended for AF lesions, due to the risk of recurrence and malignant change into AFS. Segmental resection is the most recommended therapy for AFS.

Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic neoplasm of the jaw. AFS is characteristically composed of a benign odontogenic epithelium and a malignant mesenchymal component. The posterior region of the mandible is the predominantly occupied site. In the present report, a new case of AFS in a 22-year-old male that originated from ameloblastic fibroma was described. Histologically, the tumor showed biphasic components: Benign epithelium and a malignant mesenchymal component. Immunochemical findings revealed that the tumor cells were positive for cluster of differentiation (CD) 34, vimentin, Ki-67 and p53, but negative for smooth muscle actin, S-100, CD68 and desmin. The clinical presentation, radiographic appearances and treatment measures were additionally described and reviewed.