1. Eur Ann Otorhinolaryngol Head Neck Dis. Feb;(1) doi: / Epub Jul 9. 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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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.

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

It mainly presents as a painful maxillary or mandibular swelling. Radiographs show a radiolucent mass with ill-defined borders. Radical surgical excision and long-term follow-up are the suggested treatment. We report the case of a year-old female with a 2-month history of an asymptomatic swelling in her left mandible. The patient underwent a left hemimandibular resection.

Histopathological examination revealed a biphasic tumor composed of inconspicuous islands of benign odontogenic ameooblastic 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.

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.

They are derived from epithelial, ectomesenchymal, and mesenchymal elements of the tooth-forming apparatus. Malignant odontogenic tumors are classified as odontogenic carcinomas and odontogenic sarcomas [ 1 ].

Ameloblastic fibrosarcoma AFS is a malignant odontogenic tumor characteristically composed of a benign epithelium and a malignant mesenchymal component [ 2 ]. amelbolastic

Clinically, patients present with pain and swelling [ 3 ] and the mandible is the most commonly affected site [ 1 ]. The prognosis associated with AFS is good when treated with surgical resection [ 4 ]. A year-old female presented with a 2-month history of an asymptomatic swelling in her left mandible. Extraoral evaluation revealed a gross swelling over the left mandible Figure 1. Radiographic examination showed an extensive ill-defined unilocular radiolucent lesion around an impacted mandibular left first molar.

An odontogenic lesion was the clinical impression and an incisional biopsy was performed. Histopathological examination revealed a biphasic tumor composed of inconspicuous islands of benign odontogenic epithelium and an abundant malignant ameloblstic component with marked cellularity, nuclear pleomorphism, hyperchromatism, and moderate mitotic figures Figure 2. 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 fibrosatcoma epithelium.

In view of these histopathological and immunohistochemistry findings, an AFS was diagnosed. Four months later a panoramic radiograph and CT scan of head amelonlastic neck were performed disregarding locoregional and distant metastases while also revealing again the same ill-defined radiolucent lesion around an impacted mandibular left first molar Figure 4.

The patient underwent a left hemimandibular resection and immediate fibular free flap reconstruction. Histopathological examination showed the same biphasic tumor previously described.

PCNA was strongly positive in both components, while p53 was strongly positive only in the mesenchymal component. The final histopathological diagnosis was identical to that of the incisional biopsy and an AFS was confirmed. Currently, one year after the surgical procedure, the patient is clinically and radiologically disease-free Figure 7. Panoramic radiograph a and axial CT scan b revealing an ill-defined radiolucent lesion around an impacted mandibular left first molar.

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Gross photograph of the cut surface of the left half of the mandible showing a solid gray tumor mass. Panoramic radiograph showing area of mandibular resection with no evidence of recurrence 1 year after surgery. AFS was first reported by Heath in describing it as a spindle cell sarcoma that also had epithelial cells resembling the cells of the ameolblastic organ [ 5 ]. To the best of our knowledge, less than documented cases have been reported in the English language literature [ 6 ].

The usual clinical presentation consists of a patient who complains of a painful but occasionally painless facial mass with accompanying paresthesia or dysesthesia. The duration of symptoms varies widely from a few weeks up to 2 years [ 7 ].

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

The mean age of presentation is AFS can arise de novo or from a previous ameloblastic fibroma AF. Those lesions arising from an AF tend to occur in patients aged approximately a decade older than those arising de novo [ 10 ]. The posterior mandible is the most commonly affected site [ 8 ].

Radiologically, AFS presents as a radiolucent mass with ill-defined borders. Grossly the tumor may be cystic or solid with a fleshy whitish to yellow appearance [ 7 ].

The histological architecture of AFS is characterized by benign epithelial islands that are composed of columnar or cuboidal peripheral cells arranged in a palisading pattern. At the center of these islands is polyhedral cell reminiscent of stellate reticulum. The mesenchymal component consists of plump and spindle stromal cells which show mild to moderate cytologic atypia and numerous mitotic figures [ 11 ].

AF is the main differential diagnosis of AFS. 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.

Only 2 cases of metastasis have been reported [ 1516 ]. Due to lack of experience, there is no consensus on the treatment yet. In general, the treatment of choice is surgical excision with clear margins and long-term follow-up. Adjuvant radiotherapy has been used with no evidence of recurrence [ 9 ]. Adjuvant chemotherapy has also been used with moderate success [ 17 ], although no specific chemotherapy protocols have been established yet. Our patient presented an AFS in the posterior mandible.

Such location is the most frequent affected site reported in the literature. Her age at presentation was 22 years old, a little younger than the mean age of presentation, but still in the third decade of life, like most patients from previous reports.

The origin of our patients AFS and her age are two important features to consider since they seem to further support the observation made by Noordhoek et al. Histologically fibrosarcomw case had very few islands of odontogenic epithelium compared to the extensive malignant mesenchymal component, a feature commonly seen.

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 Fibgosarcoma and most authors, and believe the diagnosis is essentially made by histology. 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 or de novo.

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Resection with a wide margin is the optimal treatment strategy and close follow-up is advised due to its relatively high recurrence rate. Written informed consent was obtained from the patient for fibrossarcoma of this case report and the accompanying images.

A copy fjbrosarcoma the written consent is available upon request for review. National Center for Biotechnology InformationU. Journal List Case Rep Pathol v.

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

Published online Mar Author information Article notes Copyright and License information Disclaimer. Received Jan 11; Accepted Mar 2. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. This article has been cited by other articles in PMC.

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. 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.

Case Report A year-old female presented with a 2-month history of an asymptomatic swelling in her left mandible. Open in a separate window. 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 ]. Consent Written informed consent was obtained from the patient for publication of this case report and the accompanying images.

Conflict of Interests The authors have no conflict of interests to declare. World Health Organization Classification of Tumours: Pathology and Genetics of Head and Neck Tumours. Ameloblastic fibrosarcoma of the jaw: Journal of Cranio-Maxillo-Facial Surgery.

Ameloblastic fibrosarcoma of the mandible: Oral and Maxillofacial Pathology: A Rationale for Diagnosis and Treatment. Lectures on certain diseases of the jaws.

Experimental and Therapeutic Medicine. Journal of Oral and Maxillofacial Surgery. Malignant transformation of ameloblastic fibroma to ameloblastic fibrosarcoma: Journal of Cranio-Maxillofacial Surgery. Odontogenic Tumors and Allied Lesions. Immunohistochemical study and review of the literature. Immunoexpression of Ki67, proliferative cell nuclear antigen, and Bcl-2 proteins in a case of ameloblastic fibrosarcoma.

Annals of Diagnostic Pathology. Assessment of growth potential by MIB-1 immunohistochemistry in ameloblastic fibroma and related lesions of the jaws compared with ameloblastic fibrosarcoma.

Journal of Oral Pathology and Medicine. Rapid sarcomatous transformation of an ameloblastic fibroma of the mandible: Ameloblastic fibrosarcoma of the jaws – report of three cases. Clinicopathologic, histoenzymological and ultrastructural study. Pathology Research and Practice. Support Center Support Center. Please review our privacy policy.