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 majority of reported cases have been found in the posterior region of mandible and rarely maxillary involvement is indicated. 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. 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.

AFS is a locally aggressive malignant tumor, with regional and distant metastases being uncommon. Histologically our case had very few islands of odontogenic epithelium compared to the extensive malignant mesenchymal component, a feature commonly seen.

Sign up for our Email Newsletters. Malignant transformation of ameloblastic fibroma to ameloblastic fibrosarcoma: 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.

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

Low fibri malignant neoplasm. Postoperative chemotherapy and radiotherapy has been used successfully in a few reported cases. In case of oral and maxillofacial pain and symptoms, dentists are usually the first healthcare practitioner people consult.

A year-old female presented with one month history of right mandibular mass. CD34 expressing ameloblastic fibrosarcoma arising in the maxilla: Extraoral evaluation revealed a gross swelling over the left mandible Figure 1.

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

Those lesions arising from an AF tend to occur in patients aged approximately a decade older than those arising de novo [ 10 ]. Images hosted on other servers: PCNA was strongly positive in both components, while p53 was strongly positive only in the mesenchymal component. No evidence of regional or distant metastasis was noted. Please review our privacy policy. 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.


Ameloblastic fibrosarcoma of the maxillary sinus in an infant: A diagnosis of AFS was rendered. A Rationale for Diagnosis and Treatment. 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.

All surgical margins were free of tumor infiltration. A case report with long-term follow-up. 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. Report of 2 chemosensitive pediatric cases. AF is the main differential diagnosis of AFS.

The mean age at the time of diagnosis of AFS for 62 cases reviewed by Bregni et al. Panoramic radiograph showing area of mandibular ameloblastoc with no evidence of recurrence 1 year after surgery. Four months later a panoramic radiograph and CT scan of head and neck were performed fibrk locoregional and distant metastases while also revealing fibo the same ill-defined radiolucent lesion around an impacted mandibular left first molar Figure 4.

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: Clinically, patients present with pain and swelling [ 3 ] and the mandible is the ajeloblastic commonly affected site [ 1 ]. Journal of Cranio-Maxillo-Facial Surgery. Abstract Ameloblastic fibrosarcoma AFS is a rare malignant mixed odontogenic tumor which is usually considered as the malignant counterpart of ameloblastic fibroma.

Therefore, some investigators recommend a more aggressive treatment for ameloblastic fibromas as compared to previous procedures. 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 ].


Annals of Diagnostic Pathology. This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3. Support Center Support Center. Grossly the tumor may be cystic or solid with a fleshy whitish to yellow appearance [ 7 ].

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

This article has fobro cited by other articles in PMC. Anaplastic ameloblastic fibrosarcoma arising safcoma recurrent ameloblastic fibroma: Ameloblastic fibrosarcoma AFS is a rare malignant mixed odontogenic tumor which is usually considered as the malignant counterpart of ameloblastic fibroma.

He complained of continuous dull pain in the upper left region of his face from 2 months ago and visual disturbances in recent weeks. Intraorally, nontender buccal and palatal expansion of the alveolar ridge at the same area was observed. Treatment of choice is wide surgical excision, with long-term follow-up.

Ameloblastic fibrosarcoma of the jaws – report of three cases. Ameloblastic fibrosarcoma AFS is a rare mixed odontogenic tumor composed of benign epithelial and malignant ectomesenchymal components. 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.

Ameloblastic fibrosarcoma [title] mandible. 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, fbiro believe the diagnosis is essentially made by histology.

Computerized tomography CT scan showed a large destructive mass. New author database being installed, click here for details.

No evidence of regional lymphadenopathy or distant metastasis was found and a segmental resection of the maxilla along with wide excision of the surrounding soft tissues was performed. Received Jan 11; Accepted Mar 2.