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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Malignant transformation of ameloblastic fibroma to ameloblastic fibrosarcoma: A year-old female presented with a 2-month history of an asymptomatic swelling in her left mandible. Computed tomography scan showed extension of the lesion medially and vertically to the nasal septal bone and the inferior orbital rim, respectively [ Figure 2 ]. A biopsy of the mass was sarcomma.

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

Low grade malignant neoplasm. The final histopathological diagnosis was identical to that of the incisional biopsy and an AFS was confirmed.

Intraoral examination and a CT scan evaluation b revealed no evidence of ameloblxstic after two years. In case of oral and maxillofacial pain and symptoms, dentists are usually the first healthcare practitioner people consult. Dentists should be sarcoa with signs and symptoms of malignant tumors of the jaws, especially rare cases with unusual presentation. National Center for Biotechnology InformationU. Published online Mar Pathology and Genetics of Head and Neck Tumours. It can arise de novo, however one-third of cases may arise from a recurrent ameloblastic fibroma, in which case they appear to present at an older age.

The differential diagnosis includes other odontogenic sarcomas, ameloblastic carcinosarcoma and spindle cell carcinoma. Therefore, some investigators recommend a more aggressive treatment for ameloblastic fibromas as compared to previous procedures.


Computerized tomography CT scan showed a large destructive aameloblastic. The majority of reported cases have been found in the posterior region of mandible and rarely maxillary involvement is indicated.

AF is the main differential diagnosis of AFS. However, sometimes, it is seriously difficult to differentiate AFS, especially low-grade tumors from ameloblastic fibroma. No evidence of regional or distant metastasis was noted.

Rapid sarcomatous transformation of an ameloblastic fibroma of the mandible: Histopathological examination revealed a biphasic tumor composed of inconspicuous islands of benign odontogenic epithelium and an abundant malignant mesenchymal component with marked cellularity, nuclear pleomorphism, hyperchromatism, and moderate mitotic figures with clear margins; one year after the surgical procedure, the patient is clinically and radiologically disease-free.

Computed tomography scan shows extensive destruction of the left maxillary sinus. 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. The prognosis associated with AFS is good when treated with surgical resection [ 4 ].


The mean age of presentation is Consent Written informed consent was obtained from the patient for publication of this case report and the accompanying images. Those lesions arising from an AF tend to occur in patients aged approximately a decade older than those arising de novo [ 10 ]. Left half of mandible. Click here for information on linking to our website or using our content or images.

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

Both neoplasms have a biphasic nature; however, AF has no malignant component, unlike AFS in which the mesenchymal component presents marked cellularity, nuclear pleomorphism, hyperchromatism, and a moderate to high number of mitotic figures. Currently, one year after the surgical procedure, the patient is clinically and radiologically disease-free Figure 7. Anaplastic ameloblastic fibrosarcoma arising from recurrent ameloblastic fibroma: Case report and literature review.


Although the immunohistochemical profile of this neoplasm was identical to the one described in the literature and helped to establish the diagnosis, we agree with Kobayashi and most authors, and believe the diagnosis is essentially made by histology.

Pathology Outlines – Malignant tumors: ameloblastic fibrosarcoma

Therefore, also considering epithelial component, the diagnosis based on histopathological examination was not a serious problem. Radiographically, AFS usually appears as an expansile destructive radiolucency with ill-defined margins. Accessed December 31st, Ameloblastic fibrosarcoma of the mandible: Restricted molecular abnormalities of certain genes to the malignant transformation.

Adjuvant radiotherapy has been used with no evidence of recurrence [ 9 ]. In general, safcoma treatment of choice is fkbro excision with clear margins and long-term follow-up. Grossly the tumor may be cystic or solid with a fleshy whitish to yellow appearance [ 7 ].

Paresthesia is also observed. Discussion 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 ]. Oral and Maxillofacial Pathology, 4th Edition, The posterior mandible is the most commonly affected site [ 8 ]. Odontogenic tumors and tumor-like lesions constitute a rare group of heterogeneous diseases that range from nonneoplastic tissue proliferations to malignant tumors with metastatic potential.

The patient was under close follow up and after 6 months, rehabilitative prosthetic treatment was started.