Case Of Ameloblastic Fibrosarcoma Research Articles

AMELOBLASTIC FIBROSARCOMA OF THE MANDIBLE: REPORT OF TWO NEW CASES

The aim of the present study is to report the clinicopathologic features of 2 new cases of ameloblastic fibrosarcoma (AFS). Case 1 was a 9-year-old boy presenting with a painful ulcerated swelling in the posterior mandible causing facial asymmetry. Case 2 presented as an asymptomatic swelling in the anterior mandible of a 14-year-old female patient. Imaging exams revealed destructive multilocular radiolucencies with irregular margins, associated with impacted teeth, causing exuberant cortical expansion and tooth displacement. Microscopically, both cases showed mesenchymal cells with marked hypercellularity and nuclear pleomorphism containing small islands of odontogenic epithelium with peripheral palisading. Both patients were treated by surgical excision; case 1 showed recurrence after 2 years of follow-up. AFS usually affects the posterior mandible of young adults with previous history of ameloblastic fibroma; nevertheless, clinicians should also consider this rare odontogenic sarcoma when evaluating aggressive ill-defined radiolucent lesions in the mandible of pediatric patients.

Ameloblastic fibrosarcoma of the maxilla with EGFR exon 20 insertions: Relevance of whole-exome sequencing in molecular understanding and therapeutic proposals for rare cancers

Ameloblastic fibrosarcoma (AFS) is the most common odontogenic sarcoma, but the incidence is relatively low, and its molecular biology is poorly understood. We experienced a young female patient with a rapidly growing soft tissue tumor of the left maxilla, which eventually occupied the left side of the oral cavity. Histologically, the tumor mainly consisted of a proliferation of atypical spindle to polygonal cells without any specific differentiation, but a small number of benign odontogenic epithelial foci mainly in the tumor periphery were also noted; thus, a diagnosis of AFS was made. We performed whole-exome sequencing (WES) on the tumor to investigate its molecular features and identify therapeutic options. We found that the tumor harbored EGFR exon 20 insertions and MDM2 amplification; the former may be a target for newly developed tyrosine kinase inhibitors in case of recurrence. To the best of our knowledge, this is the first case of AFS for which WES was performed and with EGFR mutation. Our case provides new genetic information on AFS and suggests that comprehensive genetic analysis can clarify the molecular biology in rare cancers, potentially leading to the proposal of therapeutic strategies.

Aggressive ameloblastic fibrosarcoma in maxilla: case report and new perspectives based on the current literature

ABSTRACT Ameloblastic fibrosarcoma (AFS) is a rare malignant tumor characterized by benign epithelial component within a malignant fibrous stroma. It occurs as a de novo lesion or from a previous ameloblastic fibroma. The aim of this paper is to report an aggressive and recurrent case of AFS in the maxilla of a 38-year-old patient. Histopathological diagnosis can be challenging, especially when it is based on incisional biopsy specimens. Hence, this report highlights not only the importance of the histological features for diagnosis but also the clinical behavior of AFS based on current literature evidence.

Ameloblastic Fibrosarcoma of the Mandible

Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor. We report 2 pediatric cases of AFS from 2 different centers but reviewed by the same pathologist, which were unusual in that they were chemosensitive. Management was wide surgical resection, chemotherapy, and adjuvant radiotherapy. One case was a local AFS recurrence after incomplete surgery and the other was an AFS that was overlooked during earlier diagnosis of an ameloblastic fibroma. Both tumors responded very well to preoperative chemotherapy, with a reduction in tumor size and histologically proven decrease in viable tumor cell number. Both patients are well after 13 and 14 months of follow-up.

CD34 expressing ameloblastic fibrosarcoma arising in the maxilla: a new finding

Ameloblastic fibrosarcoma (AFS) is a rare malignant tumor of the jaw. The malignant mesenchymal component of AFS has been described as 'fibroblast-like', although little is known about the immunophenotype, except for vimentin expression. Here, we present a case of AFS in a 62-year-old woman. The mesenchymal component displayed the features of either dermatofibrosarcoma protuberans or fibrosarcoma, and was positive for CD34. This is the first reported case of CD34 expressing AFS in the maxilla.

良性，悪性成分が混在した下顎骨エナメル上皮線維肉腫の１例

Ameloblastic fibrosarcoma (AFS) is a rare malignant tumor composed of benign odontogenic epithelial components and malignant ectomesenchymal components. We report a case of AFS occurring in the mandible of a 15-year-old boy. The patient was referred to our department for evaluation and treatment of gingival swelling. The lesion was diagnosed as a benign ameloblastic fibroma on examination of a biopsy specimen. The lesion was excised under general anesthesia, and the pathological diagnosis was AFS. At present, the postoperative course is good, with no recurrence as of about 4 years since the operation.

下顎大臼歯部に発生したエナメル上皮線維肉腫のｌ例

A case of ameloblastic fibrosarcoma in the molar region of the mandible of a 17-year-old girl is reported. Previously, the lesion had been diagnosed as benign ameloblastic fibroma on examination of a biopsy specimen. The final histological diagnosis after marginal resection of mandible was ameloblastic fibrosarcoma with ameloblastic fibroma. Therefore, another operation was performed. At present, the patient is well, and has had no problems as of 40 months since the last operation.

Mouth: A case of ameloblastic fibrosarcoma with an electron microscope observation

Mouth: A case of ameloblastic fibrosarcoma with an electron microscope observation