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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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Extraoral evaluation revealed a gross swelling over the left mandible Figure fihro. Author information Article notes Copyright and License information Disclaimer. Journal of Cranio-Maxillofacial Surgery. Failure to correctly evaluate the panoramic radiographs and establish a clinical differential diagnosis by the general practitioner should be given special attention.

Dent Res J Isfahan. An odontogenic lesion was the clinical impression and an incisional biopsy was performed. In view of these histopathological and immunohistochemistry findings, an AFS was diagnosed.

This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Clinical findings vary among reported cases but usually include pain and swelling. 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 led to unnecessary extraction of the teeth and delayed diagnosis of the tumor. Open in a separate window. Patients often present with swelling and pain Occasionally painless facial mass with accompanying paresthesia. Abstract Ameloblastic fibrosarcoma AFS is a rare malignant mixed odontogenic tumor which is usually considered as sarcomq malignant counterpart of ameloblastic fibroma.

We also emphasize on comprehensive clinical, ifbro, and histopathologic evaluation of such patients rather than immunohistochemical staining to make an accurate diagnosis. Grossly the tumor may be cystic or solid with a fleshy whitish to yellow appearance [ 7 ].

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Pathology Outlines – Malignant tumors: ameloblastic fibrosarcoma

No evidence of regional or distant metastasis was noted. 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 Figure 2.

AF is the main differential diagnosis of AFS. A copy of the written consent is available upon request for review. The treatment of choice for AFS is radical surgical excision without primary neck dissection. Computed tomography scan shows extensive destruction of the left maxillary sinus. Oral and Maxillofacial Pathology: Only 2 cases of metastasis have been reported [ 1516 ].

Flbro, 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 fiibro that also had epithelial cells resembling the cells of the enamel organ [ 5 ].

Click here for patient related inquiries. Intraoral examination and a CT scan evaluation b revealed no evidence of recurrence after two years.

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

Restricted molecular abnormalities of certain genes to the malignant transformation. Considering additional 9 cases reported so far,[ 23456891011 ] no significant alteration was observed in age and sarcona distribution [ Figure 5 ] or location prevalence the mandible to maxilla ratio: 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.

J Pediatr Hematol Oncol. Clinicopathologic, histoenzymological and ultrastructural study. Discussion AFS was first reported by Heath in describing it as a spindle cell sarcoma that also had epithelial cells resembling the amelblastic of the enamel organ [ 5 ]. Footnotes Source of Support: They are derived from epithelial, ectomesenchymal, and mesenchymal elements of the tooth-forming apparatus.


A case report with long-term follow-up. Histopathologic examination of the whole specimen confirmed the diagnosis of AFS. This article has been cited by other articles in PMC. Ameloblastic fibrosarcoma AFS is a malignant odontogenic tumor characteristically composed of a benign epithelium and a malignant mesenchymal component [ 2 ]. Click here for information amrloblastic linking to our website or using our content or images. Report of 2 chemosensitive pediatric cases. Ameloblastic fibrosarcoma, maxilla, odontogenic tumor.

Ameloblastic fibrosarcoma AFS is a rare malignant mixed odontogenic tumor which is usually considered as the malignant counterpart of ameloblastic fibroma.

Case report and literature review. Experimental and Therapeutic Medicine. The posterior mandible is the most commonly affected site [ 8 ]. A diagnosis of AFS was rendered. 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. The duration of symptoms varies widely from a few weeks up to 2 years [ 7 ].

Written informed consent was obtained from the patient for publication of this case report and the accompanying images. Only mesenchymal component represents sarcomatous alterations and ameloblast-like epithelial nest remains bland in AFS. Therefore, some investigators recommend a more aggressive treatment for ameloblastic fibromas as compared to previous procedures.

Microscopically, the bland epithelial component of AFS is similar to that seen in ameloblastic fibroma, although it is frequently amelolastic prominent. Annals of Diagnostic Pathology. The mean age at the time of diagnosis of AFS for 62 cases reviewed by Bregni et al.