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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Clinically, patients present with pain and swelling [ 3 ] and the mandible is the most commonly affected site [ 1 ]. Nil Conflict of Interest: Sarcomatous overgrowth in recurrent ameloblastic fibrosarcoma.

Dentists should be familiar with signs and symptoms of malignant tumors of the jaws, especially ame,oblastic cases with unusual presentation. In view of these histopathological and immunohistochemistry findings, an AFS was diagnosed.

Open in a separate window. 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.

Histopathological examination showed the same biphasic tumor previously described.

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

Postoperative chemotherapy and radiotherapy has been used successfully in a few reported cases. Restricted molecular abnormalities of certain genes to the malignant transformation. Here, we report a case of AFS in a year-old man in the maxilla, which was regarded as an uncommon location for this tumor. The usual clinical presentation consists of a patient who complains of a painful but occasionally painless facial mass with accompanying paresthesia or dysesthesia.

Footnotes Source of Support: National Center for Biotechnology InformationU. A diagnosis of AFS was rendered. The final histopathological diagnosis was identical to that of the incisional biopsy and an AFS was confirmed. Maryam Khalili 1 and Pouyan Amini Shakib 2. Rapid sarcomatous transformation of an ameloblastic fibroma of the mandible: Open in a separate window. Failure to correctly ameloblastc the panoramic ameloblstic and establish a clinical differential diagnosis by the general practitioner should be given special attention.

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Left half of mandible. Intraoral examination and a CT scan evaluation b revealed no evidence of recurrence after two years. Microscopic examination of the slides revealed a neoplastic tissue with scattered benign appearing cords and nests of epithelial cuboidal or columnar cells with peripheral palisading in a myxoid to highly cellular mesenchymal background mostly composed of plump stellate and ovoid to spindle cells.

This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Annals of Diagnostic Pathology.

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 ]. Immunoexpression of Ki67, proliferative cell nuclear antigen, and Bcl-2 proteins in a case of ameloblastic fibrosarcoma. Adjuvant radiotherapy has been used with no evidence of recurrence [ 9 ]. A year-old female presented with one month history of right mandibular mass. This article has been cited by other articles in PMC. 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.

The mean age of presentation is We report the case of a year-old female with a 2-month history of an asymptomatic swelling in her left mandible.

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

Immunohistochemical study and review of the literature. The posterior mandible is the most commonly affected site [ 8 ]. Although AFS is regarded as the malignant counterpart of ameloblastic fibroma, it may arise de novo without any pre-existing lesion. Ameloblastic fibrosarcoma ameloblasyic the mandible: Sign up for our Email Newsletters. Consent Written informed consent was obtained from the patient for publication of this case report and the accompanying images.


This article ameloblastiic been cited by other articles in PMC. J Oral Pathol Med. Only 2 cases of metastasis have been reported [ 1516 ]. 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.

The malignant spindle cell proliferation showed positive staining with p and a high proliferation index with ki Appears as an expansive, multilocular radiolucent lesion Often shows cortical perforation.

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

A year-old female presented with a 2-month history of an asymptomatic swelling in her left mandible. A biopsy of the mass was performed.

Abstract Ameloblastic fibrosarcoma AFS is a rare malignant mixed odontogenic tumor which is usually sarccoma as the malignant counterpart of ameloblastic fibroma. Extraoral evaluation revealed a gross swelling over the left mandible Figure 1. 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.

Author information Article notes Copyright and License information Disclaimer. Dent Res J Isfahan. Assessment of growth potential by MIB-1 immunohistochemistry ameloblasric ameloblastic fibroma and related lesions of the jaws compared with ameloblastic fibrosarcoma.

Histologically our case had very few islands of odontogenic epithelium compared to the extensive malignant mesenchymal component, a feature commonly seen.