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Mandible / maxilla – Malignant tumors: ameloblastic fibrosarcoma. A case of an ameloblastic fibrosarcoma in the mandible is described. The primary tumor was seen in a 5-year-old child. In spite of repeated surgical. Introduction. Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor. It can arise de novo, however one-third of cases may arise from a recurrent.

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Diagnosis dependent on clinical, radiologic and pathologic correlation.

Ameloblastic Fibrosarcoma of the Mandible: A Case Report and Brief Review of the Literature

Malignant transformation of ameloblastic fibroma to ameloblastic fibrosarcoma: Home About Us Advertise Amazon. Currently, one year after the surgical procedure, the patient is clinically and radiologically disease-free Figure 7. Nil Conflict of Interest: Please review our privacy policy. They are derived from epithelial, ectomesenchymal, sarcom mesenchymal elements of the tooth-forming apparatus. Only 2 cases of metastasis have been reported [ 1516 ]. Those lesions arising from an AF tend to occur in patients aged approximately a decade older than those arising de novo [ 10 ].

AFS can arise de novo or from a previous ameloblastic fibroma AF. All surgical margins were free of tumor infiltration. Therefore, some investigators recommend a more aggressive treatment for ameloblastic fibromas as compared to previous procedures.

Footnotes Source of Support: A case report with long-term follow-up.

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

Low grade malignant neoplasm. To the best of our knowledge, less than documented cases have been reported in the English language literature [ 6 ]. The posterior mandible is the most commonly affected site [ 8 ]. Clinical examination revealed a bony hard swelling of the left posterior region of maxilla.

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Extraoral evaluation revealed a gross swelling over the left mandible Figure 1. Ameloblastic fibrosarcoma of the jaws – report of three cases.

Computed tomography scan shows extensive destruction of the left maxillary sinus. World Health Organization Classification of Tumours: He performed an incisional biopsy and sent it to a general pathology service for histopathologic examination. Journal List Case Rep Pathol v. In summary, AFS is a rare malignant odontogenic tumor characterized by a benign odontogenic epithelium and a malignant mesenchymal component that can arise from a previous AF fibrk de novo.

It mainly presents as a painful maxillary or mandibular swelling. Paresthesia is also observed. CK was strongly positive in the odontogenic epithelium and negative in the mesenchymal component, while Vimentin was strongly positive in the mesenchymal component and negative in the odontogenic epithelium.

National Center for Biotechnology InformationU. Case Report A year-old female presented with a 2-month history of an asymptomatic swelling in her left mandible. Treatment of choice is wide surgical excision, with long-term follow-up. In view of these histopathological and immunohistochemistry findings, an AFS was diagnosed. Such location is the most frequent affected site reported in the literature.

Appears as an expansive, multilocular radiolucent lesion Often shows cortical perforation. Written informed consent was obtained from the patient for publication of this case report and the accompanying images. The mean age of presentation is AFS was first reported by Heath in describing it as a spindle cell sarcoma that also had epithelial cells resembling the cells of the enamel organ [ 5 ].

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The surgeon decided to ask for a second opinion and sent the documents to our center for consultation.

Rare mixed odontogenic tumor that consists of a benign ameloblastic epithelium and a malignant mesenchymal stroma Thought to be malignant counterpart to ameloblastic fibroma. Assessment of growth potential by MIB-1 immunohistochemistry in ameloblastic fibroma and related lesions of the jaws compared with ameloblastic fibrosarcoma.

Clinicopathologic, histoenzymological and ultrastructural study. Radiographically, AFS usually appears as an expansile destructive radiolucency with ill-defined margins. The definitive diagnosis of AFS is established based on histopathologic evaluation of the mesenchymal component which usually demonstrates various features of malignancy including cellular atypia, pleomorphism, and mitotic figures.

The mesenchymal component consists of plump and spindle stromal cells which show mild to moderate cytologic atypia and numerous mitotic figures [ 11 ]. PCNA was strongly positive in both components, while p53 was strongly positive only in the mesenchymal component. This srcoma is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.

A year-old female presented with one month history of right mandibular mass. Left half of mandible.