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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Computed tomography scan showed extension of the ameloblasric medially and vertically to the nasal septal bone and the inferior orbital rim, respectively [ Figure 2 ]. Histologically our case had very few islands of odontogenic epithelium compared to the extensive malignant mesenchymal component, a feature commonly seen. No evidence of regional lymphadenopathy or distant metastasis was found and a segmental resection of the maxilla along with vibro excision of the surrounding soft tissues was performed.

In this article, we reported a case of maxillary AFS. 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. Open in a separate window.

Oral and Maxillofacial Pathology: 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.

Paresthesia is also observed. This is an open-access article distributed under the terms of the Creative Commons Ameloblxstic Alike 3.

Pathology Outlines – Malignant tumors: ameloblastic fibrosarcoma

The majority of reported cases have been found in the posterior region of mandible and rarely maxillary involvement is indicated. A biopsy of the mass was performed. A diagnosis of AFS was rendered. In case of oral and maxillofacial pain and symptoms, dentists are usually the first healthcare practitioner people consult.


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

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. Ameloblastic fibrosarcoma is an uncommon odontogenic tumor composed of a benign epithelial component and a malignant amelobkastic component most frequently seen in the third and fourth decades of life.

Rare, More common in amelobalstic 1. However, adjuvant radiotherapy was also performed in order to ensure a better outcome. The differential diagnosis includes other odontogenic sarcomas, ameloblastic carcinosarcoma and spindle cell carcinoma.

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

Malignant odontogenic tumors are classified as odontogenic carcinomas and odontogenic sarcomas [ 1 ]. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.

sxrcoma World Health Organization Classification of Tumours: Open in a separate window. Intraorally, nontender buccal and palatal expansion of the alveolar ridge at the same area was observed.

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

The upper left second and third molars had been extracted by a dentist one month ago, assuming that the pain was of dental origin.

Treatment of choice is wide surgical excision, with long-term follow-up. Intraoral view shows buccal and palatal expansion in the left maxillary region and the exophytic ulcerated mass. Therefore, some investigators recommend a more aggressive treatment for ameloblastic fibromas as compared to previous procedures.


Adjuvant chemotherapy has also been used with moderate success [ 17 ], although no specific chemotherapy protocols have been established yet. Panoramic radiographs demonstrated an ill-defined unilocular radiolucent lesion in the left posterior region of maxilla and the left maxillary sinus was totally obliterated.

Clinically, patients present with pain and swelling [ 3 ] and the mandible is the most commonly affected site [ 1 ]. Immunoexpression of Ki67, proliferative cell nuclear antigen, and Bcl-2 proteins in a case of ameloblastic fibrosarcoma. Author information Article notes Copyright and License information Disclaimer.

The duration of symptoms varies widely from a few weeks up to 2 years [ 7 ]. Histologically, 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.

However, sometimes, it is seriously difficult to differentiate AFS, especially low-grade tumors from ameloblastic fibroma. New author database being installed, click here for details. 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.