Introduction: Ameloblastoma is regarded as the second most prevalent odontogenic tumor in the light of its prevalence, clinical characteristics, greater incidence of tumor recurrence, and therapeutic challenges. The aim of this systematic review was to establish the prevalence of ameloblastoma in the Indian subcontinent and to establish a national epidemiologic profile for these lesions. Material and Methods: A systematic review was undertaken based on the PRISMA guidelines in search of epidemiologic studies concerning odontogenic tumors and ameloblastoma that are listed by PubMed, EBSCO, and Google Scholar embracing the period from January 2010 to December 2021, to evaluate the prevalence rate in India. A total of 277 publications were retrieved, of which 27 articles were selected, based on the World Health Organization classification of odontogenic tumors. Results: The affected individuals were on average in the third decade of life, with a higher male predominance. The majority of the tumors were multilocular radiolucencies in the posterior mandible, with follicular and plexiform histopathological features. The most common type of malignant lesion is ameloblastic carcinoma. Over 60% of follicular ameloblastoma recurred more frequently than the other types of ameloblastoma. Conclusion: The systematic study indicates a slight male predisposition to ameloblastoma, with a peak incidence in the third decade of life and the mandible as the preferred anatomical site. The solid/multicystic ameloblastoma is the most prevalent histopathologic pattern. More epidemiological research on the prevalence rate of ameloblastoma is required, particularly in India, in an effort to accurately determine the national epidemiological profile of ameloblastoma.
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