A Case of Ameloblastic Fibrodentinosarcoma of the Mandible

Hirayama, Masatoshi

A Case of Ameloblastic Fibrodentinosarcoma of the Mandible

Masatoshi Hirayama DDS, Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Hideki Nakayama , Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Toshihisa Fujiie , Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Kazutoshi Ota , Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Akimitsu Hiraki , Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Masakazu Yoneda , Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Kenta Kawahara DDS, PhD, Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Masashi Nagata , Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Naohiro Takao , Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Akiyuki Hirosue DDS, PhD, Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Akihiro Muta DDS, Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Masanori Shinohara , Department of Oral and Maxillofacial Surgery, Kumamoto University, Kumamoto, Japan

Ameloblastic fibrosarcoma (AFS) is an odontogenic tumor with a benign epithelial and a malignant ectmesenchymal component. It is regarded as the malignant counterpart of the ameloblastic fibroma. On the other hand, Ameloblastic fibrodentinosarcoma (AFDS) is an extremely rare odontogenic tumor. This tumor is characterized by the formation of dysplastic dentin in addition to the typical features of AFS. To the best of our knowledge, only cases less than 20 have been reported in the literature. We herein repot a case of AFDS arising in the mandible. An 18-year-old male was referred to our hospital for evaluation of the swelling and pain in the right mandible. A panoramic radiograph demonstrated a multiocular radiolucent area with a sunray periosteal reaction in the right mandibular molar to premolar region. After performing a biopsy, which confirmed a primary diagnosis of ameloblastoma, surgical excision was performed under general anesthesia. Three weeks after the pathologic diagnosis, we performed the segmental resection of the right mandible and the mandibular reconstruction with the iliac bone graft. However, the resected specimen presented some malignant features with the formation of dysplastic dentin. Therefore, we discussed with some pathologists and evaluated the specimen in more detail by the immunohistochemical analysis. As a result, we obtained the final pathologic diagnosis of the AFDS. According to the pathologic definite diagnosis, we reassessed the spread of the disease by the whole-body PET/CT, and neither the cervical lymph node nor the distant metastasis was detected. The patient’s postoperative course was uneventful, and no signs of recurrence have been found 2 years after the operation.