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Ameloblastic fibroma (AF) and ameloblastic fibro-odontoma (AFO) are uncommon benign mixed odontogenic neoplasms. Although unusual microscopic changes including hybrid tumors have been documented in publications, their clinical outcome prediction and treatment modality selection are still challenging due to scarcity. Objective: Analysis of AF/AFO?s unusual microscopic variants in order to improve histopathologic diagnosis and to help clinicians in making informed treatment choices. An electronic search was performed in PubMed?s database using keywords: ?ameloblastic fibroma?, ?ameloblastic fibroodontoma?, ?ameloblastic fibro-odontoma?. The search scheme was limited to articles in English, dated ?January 1998? to ?October 2018?, with full texts (case reports and series) and human studies. Eligibility criteria included publications having enough clinical, radiological, and histological data to confirm their diagnosis. Age, sex, lesions? location, radiologic features, signs, symptoms, treatment approaches, and recurrences were recorded and analyzed. In this systematic review, 11 articles (reporting 14 cases) were selected. Patients? mean age was 13.75 years (male/female = 1.8). The posterior region of the mandible was the lesions? commonest location (57.14%). Swelling was reported in 78.57% of the cases, pain in 28.57% but 21.42% were asymptomatic. Radiolucent unilocular appearance was the commonest radiographic feature, but 28.57% of the cases showed a mixed radiolucent-radiopaque appearance. Other reported radiographic findings were impacted tooth (78.57%), root resorption (28.57%), tooth mobility (35.71%), and cortical perforation (14.28%). No recurrences were reported. Calcifying odontogenic cyst (COC) was the commonest lesion associated with AF/AFO (53.33%). Unicystic ameloblastoma and cystic changes without prominent epithelial lining were other reported hybrid lesions. Reported microscopic variations were pigmentation and ghost cell differentiation. COC was the commonest lesion associated with AF/AFO. Although COC commonly occurs in the jaws? anterior region, hybrid cases were more common in the posterior area. No malignant transformations were reported. The treatment modality is mostly chosen based on the lesion?s most aggressive part.
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