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Ameloblastoma is one of the most common benign odontogenic tumors of the jaw that constitutes about 10% of all tumors that arise in the mandible and maxilla. It is a slow-growing but locally invasive tumor that presents with painless swelling of the mandible or maxilla. The World Health Organization (WHO) classification of 2017 describes ameloblastomas of the following four types: ameloblastoma; unicystic ameloblastoma; extraosseous/peripheral ameloblastoma; and metastasizing ameloblastoma.

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Ewing sarcoma is the second most common primary bone tumor in children. Typical Ewing sarcoma most frequently occurs in long bones and within the pelvis. ALES (adamantinoma-like Ewing sarcoma) is a rare subtype of Ewing sarcoma that is characterized by epithelial differentiation in addition to small round blue cells.

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Adamantinoma is a low-grade malignant bone tumor with metastatic potential in the range of 15-20%, commonly affecting mid-diaphyseal tibial region and jaw. Numerous cases of adamantinoma affecting the appendicular skeleton have been reported but only three in the pelvis till date. We present the case of a 24-year-old male, who was initially reported as fibrous dysplasia for a lesion in pelvis.

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Recurrent adamantinoma of the mandible.

Int J Oral Maxillofac Surg

August 2021

Department of Oral and Maxillofacial Surgery, Peking University School and Hospital of Stomatology, Beijing, China. Electronic address:

Adamantinoma is an extremely rare tumour originating from bone that can be divided into classical and osteofibrous dysplasia (OFD)-like adamantinoma. This low-grade malignancy almost exclusively occurs in long bones, and only few cases of mandibular adamantinoma have been reported. Here, we report the case of a 30-year-old female with a 2-year history of right mandible tenderness.

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Diagnosis of odontogenic tumors can be challenging due to their rarity and diverse morphology, but when arising near the tooth, the diagnosis could be suspected. When their location is not typical, like inside the paranasal sinuses, the diagnosis is less easy. Maxillary ameloblastomas are exceedingly rare with only sparse information on their epidemiological, histological and genetic characteristics.

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