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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He complained of continuous dull pain in the upper left region of his face from 2 months ago and visual disturbances in recent weeks. Oral and Maxillofacial Pathology, 4th Edition, World Health Organization Classification of Tumours: Nil Conflict of Interest: After 2 years follow up, no evidence of recurrence was noted.

Here, we report a case of AFS in a year-old fibrp in the maxilla, which was regarded as an uncommon location for ajeloblastic tumor. Images hosted on other servers: Only mesenchymal component represents sarcomatous alterations and ameloblast-like epithelial nest remains bland in AFS. The origin of our patients AFS and her age are two important features to consider since they seem to further support the observation ameloblastix by Noordhoek et al.

Rare, More common in males 1.

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

It mainly presents as a painful maxillary or mandibular swelling. As expected, the pain persisted and healing of the extraction site ameloblasti not normal. Journal of Cranio-Maxillofacial Surgery. Treatment of choice fibrl wide surgical excision, with long-term follow-up. Case Report A year-old female presented with a 2-month history of an asymptomatic swelling in her left mandible.

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. Appears as an expansive, multilocular radiolucent lesion Often shows cortical perforation.


Only 2 cases of metastasis have been reported [ 1516 ]. Radical surgical excision and long-term follow-up are the suggested treatment.

A biopsy of the mass was performed. Assessment of growth potential by MIB-1 immunohistochemistry in ameloblastic fibroma and related lesions of the jaws compared with ameloblastic fibrosarcoma. The mesenchymal component consists of plump and spindle stromal cells which show mild to moderate cytologic atypia and numerous mitotic figures [ 11 ].

Report of 2 chemosensitive pediatric cases.

Clinical findings vary among reported cases but usually include pain and swelling. AFS is a highly recurrent lesion. Approximately, one third of reported cases appear to originate from recurrence of a previous ameloblastic fibroma.

Computed tomography scan shows extensive destruction of the left maxillary sinus. Pathology and Genetics of Head and Neck Tumours. 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.

Intraoral examination and a CT scan evaluation b revealed no evidence of recurrence after two years. A year-old man, educated in an engineering field, from a city in North of Iran was referred to School of Dentistry of Tehran University of Medical Sciences by an oral and maxillofacial surgeon OMFS for oral pathology consultation in winter of Malignant transformation of ameloblastic fibroma to ffibro fibrosarcoma: National Center for Biotechnology InformationU.

Histopathologic examination of the whole specimen confirmed the diagnosis of AFS. AFS was first reported by Heath in describing it as a spindle cell sarcoma that also saarcoma epithelial cells resembling the cells of the enamel organ [ 5 ]. Click here for information on linking to our website or using our content or images.

The upper left second and third molars had been extracted by a dentist one month ago, assuming that the pain was of dental origin. Panoramic radiograph showing area of mandibular resection with no evidence of recurrence 1 year after surgery.

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

Ameloblastic fibrosarcoma of the maxillary sinus in an infant: Ameloblastic fibrosarcoma Ameloblastic fibrodentinosarcoma: Home About Us Advertise Amazon. Four months later a panoramic radiograph and CT scan of head and 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.

Please review our privacy policy. Intraoral view shows buccal and palatal expansion in the left maxillary region and the exophytic ulcerated mass.

Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Sarxoma of two cases and review of the literature. The malignant spindle cell proliferation showed positive staining with p and a high proliferation index with ki 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.

Annals of Diagnostic Pathology. A year-old gibro presented with one month history of right mandibular mass. Clinical examination revealed ameloglastic bony hard swelling of the left posterior region of maxilla. Therefore, a more serious process was suspected and the patient was referred to an OMFS for further examination. A Rationale for Diagnosis and Treatment.

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.

Histologically our case had very few islands of odontogenic epithelium compared to the extensive malignant mesenchymal component, a feature commonly seen.

Lectures on certain diseases of the jaws.