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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The clinical, radiographic, and histopathological features were described in addition to the patient’s management and present status. This knowledge along with a high index of suspicion results in correct differential diagnosis and early detection of serious cases and could have a tremendous impact on the prognosis and total outcome of the disease. It is critical to differentiate AFS, especially low-grade variant, from ameloblastic fibroma, and recently, several immunohistochemical studies with Ki67, Bcl-2, PCNA, c-KIT, and P53 have been performed, suggesting that proliferating markers in association to histopathologic features could be useful for identifying the malignant tumor.

Computed tomography scan showed extension of the lesion medially and vertically to the nasal septal bone and the inferior orbital rim, respectively [ Figure 2 ]. Click here for patient related inquiries. Journal of Oral and Maxillofacial Surgery.

Clinical examination revealed a bony hard swelling of the left posterior region of maxilla. PCNA was strongly positive in both components, while p53 was strongly positive only in the mesenchymal component. The treatment of choice for AFS is radical surgical excision without primary neck dissection.

Sarcomatous overgrowth in recurrent ameloblastic fibrosarcoma. In case of oral and maxillofacial pain and symptoms, dentists are usually the first healthcare practitioner people consult. The prognosis associated with AFS is good when treated with surgical resection [ 4 ]. This led to unnecessary extraction of the teeth and delayed diagnosis of the tumor.


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Diagnosis dependent on clinical, radiologic and pathologic correlation. Radiographically, AFS usually appears as an expansile destructive radiolucency with ill-defined margins. The majority of reported cases have been found in the posterior region of mandible aameloblastic rarely maxillary involvement is indicated. Open in a separate window. All surgical margins were free of tumor infiltration.

Accessed December 31st, Extraoral evaluation revealed a gross swelling over the left mandible Figure 1.

Maryam Khalili 1 and Pouyan Amini Shakib 2.

Abstract Ameloblastic fibrosarcoma is an uncommon odontogenic tumor composed of a benign epithelial component and a malignant ectomesenchymal component most frequently seen in the third and fourth decades of life. AFS is a very rare intraosseous odontogenic malignancy with approximately 71 cases published to date.

Received May; Accepted Nov. In view of these histopathological and immunohistochemistry findings, an AFS was diagnosed.

No other intra- or extra-oral sign was observed; the remaining head and neck examination and general physical evaluation were unremarkable and no significant event was noted in the medical history. To the best of our knowledge, less than documented cases have been reported in the English sarfoma literature ameloboastic 6 ].

Such location is the most frequent affected site reported in the literature. Malignant odontogenic tumors are classified as odontogenic carcinomas and odontogenic sarcomas [ 1 ].

Pathology Outlines – Malignant tumors: ameloblastic fibrosarcoma

New author database being installed, click here for details. A diagnosis of AFS was rendered. Oral and Maxillofacial Pathology: Journal of Oral Pathology and Medicine. Introduction 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.


Ameloblastic fibrosarcoma of the upper jaw: Report of a rare case with long-term follow-up

Assessment of growth sarcomq by MIB-1 immunohistochemistry in ameloblastic fibroma and related lesions of the jaws compared with ameloblastic fibrosarcoma. Resection with a wide margin is the optimal treatment strategy and close follow-up is amelobastic due to its relatively high recurrence rate. Considering additional 9 cases reported so far,[ 23456891011 ] no significant alteration was observed in age and gender distribution [ Figure 5 ] or location prevalence the mandible to maxilla ratio: Histologically, it consisted of a mixed epithelial-mesenchymal odontogenic neoplasm composed of benign islands of well-differentiated ameloblastic epithelium within a malignant fibrous stroma consisting of spindle cells or fibroblasts with a brisk mitotic activity.

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 ]. Malignant transformation of ameloblastic fibroma to ameloblastic fibrosarcoma: He complained of continuous dull pain in the upper maeloblastic region of his face from 2 months ago and visual disturbances in recent weeks.

Please review our privacy policy. Adjuvant chemotherapy has also been used with moderate success [ 17 ], although no specific chemotherapy protocols have been established yet. Ameloblastic fibrosarcoma AFS is a malignant odontogenic tumor characteristically composed of a benign epithelium and a fibrl mesenchymal component [ 2 ].