Ameloblastic fibrosarcoma of the mandible: case report and literature review

Abstract

--Introduction: Ameloblastic fibrosarcoma is a rare malignant odontogenic tumor. Over 50% reported cases have histological evidence of ameloblastic fibroma at the same site. The mortality rate of the tumor is 19% and its recurrence rate is 37%, which mandates a total resection along with long-term follow-up. Observation: We present the case of a 14-year-old male diagnosed with ameloblastic fibrosarcoma, who underwent multiple stages of treatments and was followed up over 8 years. The initial management consisted of a total tumor resection with chemoradiotherapy. Then, for bone, soft, mucosal, and dental restoration after resection, mandibular reconstruction with a fibula free flap, onlay iliac graft, iliac graft fixation, facial lipofilling treatments (two), vestibular deepening procedures (two), and implant-supported dental prosthesis were performed. Comments and conclusion: This is the first case reported in the literature for the global management of mandibular ameloblastic fibrosarcoma, from oncological treatment to functional and aesthetic long-term rehabilitation.

A maxillary ameloblastic fibrosarcoma tumor: a rare case report from Syria

“…AFS is a rare malignant odonto-genic tumor. It represents <5% of all odonto-genic tumors [ 1 ]. It was first described by Heath in 1887 [ 4 ].…”

“…The average age of AFS is about 26 years [ 4 ]. Clinically, AFS causes intra-oral and/or extra-oral swelling, which was the main symptom in our case [ 1 ]. Patients usually complain of a painful facial mass [ 2 ].…”

“…AFS has low potential for distant metastasis (only two reported cases), but a high reported recurrence rate of 37% and a mortality rate of 19% [ 2 , 4 ]. Histologically, AFS is characterized by benign epithelial islands which are made up of columnar or cuboidal peripheral cells arranged in a palisading form with a malignant mesenchymal component, composed of ovoid and fusiform cells, in addition to plump and spindle stromal cells that show hyper-cellularity and nuclear polymorphisms associated with cytologic atypia, mitotic figures and anaplasia [ 1 , 2 ]. Despite AFS has the last mentioned malignant mesenchymal component and a high labeling indices unlike AF, we still consider AF the main differential diagnosis of AFS as both have a biphasic nature and similarize in the benign epithelial tissue [ 2 , 4 ].…”

“…Later reconstruction may require local or free flaps, cutaneous or mucosal, bone grafts, etc, according to the squeal of excision. Once reconstruction is performed, an implant-based rehabilitation may be performed [ 1 ].…”

“…Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor composed of benign epithelium and malignant mesenchyma [ 1 , 2 ]. It could originate from ameloblastic fibroma (AF) (two-third of the reported AFS cases) or as a malignant tumor (de novo) (one-third of the reported AFS cases).…”