• Adult
• Humans
• Child
• Mandibular Reconstruction/methods
• Fibula/surgery
• Free Tissue Flaps
• Mandible/surgery
• Software
• Bone Transplantation/methods
• Mandibular Neoplasms/surgery

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).

To analyze the clinicopathologic features of these lesions and discuss the changes in the 2017 World Health Organization classification.

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.

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.

This recommendation will be relevant for future clinical, microscopic, and molecular studies to better understand the biology of these interesting odontogenic tumors.

• Ameloblastic fibroma
• Ameloblastic fibrosarcoma
• Odontogenic carcinosarcoma
• Odontogenic tumors

Ameloblastic fibro-odontosarcoma (AFOS) is an extremely rare subtype of odontogenic sarcoma, with no more than 19 cases reported in the English literature to date. AFOS is a biphasic neoplasm, with deposits of dentin and enamel matrix. We herein present a case of AFOS with active epithelial proliferation in a 31-year-old female patient. The patient was referred to the West China Hospital of Stomatology (Chengdu, China) due to a 6-month history of a swelling in the left mandible. Following clinical and radiological examination, the initial preoperative diagnosis was ameloblastoma, with local invasion and the possibility of malignant transformation. Left hemimandibular resection was subsequently performed. The postoperative histopathological diagnosis was AFOS, accompanied by active epithelial proliferation. Immunohistochemically, cytokeratin (CK)14 and CK19 were intensely positive in the epithelium, whereas the mesenchymal cells were strongly positive for vimentin. The Ki-67 labeling index was considerably higher in the mesenchymal component (mean, 40%) compared with that in the epithelial element (mean, 5–8%). Three months after the surgical procedure, the patient remained clinically and radiologically disease-free.

• ameloblastic fibro-odontosarcoma
• histopathological
• immunohistochemistry
• odontogenic sarcoma
• odontogenic tumor

• Ameloblastoma/diagnosis
• Ameloblastoma/therapy
• Biopsy
• Fibrosarcoma/diagnosis
• Fibrosarcoma/therapy
• Humans
• Jaw Neoplasms/diagnosis
• Jaw Neoplasms/therapy
• Male
• Radiography, Panoramic
• Young Adult

• Ameloblastic fibro-odontosarcoma
• Ameloblastic fibrosarcoma
• Mandible
• Odontogenic tumor

• ameloblastic fibro-odontoma
• ameloblastic fibro-odontosarcoma
• chemotherapy response
• radiotherapy
• relapse
• transformation