Oral and maxillofacial pathology
Anaplastic ameloblastic fibrosarcoma arising from recurrent ameloblastic fibroma: restricted molecular abnormalities of certain genes to the malignant transformation

https://doi.org/10.1016/j.tripleo.2006.07.013Get rights and content

A rare case of anaplastic ameloblastic fibrosarcoma (AS) arising in an ameloblastic fibroma (AF) of the maxilla of a 48-year-old patient 10 years after the primary excision is presented. The recurrent tumor retained focal areas of AF but manifested heterogeneous malignant features ranging from low-grade spindle to highly pleomorphic sarcomas. Biomarker analysis showed alterations of the p53 and c-KIT genes restricted to the sarcomatous component. The biological implications of these findings in the future management of these tumors are discussed.

Section snippets

Case Report

A 48-year-old Hispanic male presented with a one-year history of increased temporal swelling, left eye pain, and proptosis for which he wore protective safety glasses. The patient was originally treated 10 years earlier for AF of the maxilla with left infrastructure (partial) maxillectomy. Subsequently, he underwent unknown surgical procedures for probable recurrence reported 4 years after initial surgery. Rapid growth and a marked increase in proptosis was noted before his current admission (

Discussion

We report a rare example of anaplastic sarcomatous development in an AF 10 years after the primary excision. The tumor manifested heterogeneous cellular features including low-grade spindled, epithelioid, and undifferentiated malignant components. The phenotypic spectrum of malignant sarcomatous features in our case is distinctly different from those previously reported 3, 6 and suggests progressive transformation from the benign spindle cells of the primary lesion. We contend that phenotypic

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    This indicates that the present tumor was not an ameloblastic carcinoma either. Ameloblastic fibrosarcoma is regarded as the malignant counterpart of the benign ameloblastic fibroma [5,6]. The mesenchymal tissue surrounding the epithelial component shows histological evidence of malignancy while the epithelial component appears bland.

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

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    AFS can arise de novo10 or can transform from a pre-existing benign lesion, such as AF, ameloblastic fibro-odontoma, or odontoameloblastoma.11-14 Because patients with AFS are typically 10 years older than those with AF, it is hypothesized that this occurs through a stepwise malignant transformation.14 More than 50% of reported cases had histologic documentation of AF in the same site, providing supporting evidence of malignant transformation.3

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