Ameloblastic fibrosarcoma of the upper jaw: Report of a rare case with long-term follow-up
A 26-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 2010. He complained of continuous dull pain in the upper left region of his face from 2 months ago and visual disturbances in recent weeks. The upper left second and third molars had been extracted by a dentist one month ago, assuming that the pain was of dental origin. As expected, the pain persisted and healing of the extraction site was not normal. Therefore, a more serious process was suspected and the patient was referred to an OMFS for further examination. He performed an incisional biopsy and sent it to a general pathology service for histopathologic examination. The microscopic diagnosis was “adenomatoid odontogenic tumor” which did not match the clinical and radiographic findings. The surgeon decided to ask for a second opinion and sent the documents to our center for consultation.\nClinical examination revealed a bony hard swelling of the left posterior region of maxilla. Intraorally, nontender buccal and palatal expansion of the alveolar ridge at the same area was observed. Also, the extraction site of the second and third molar was filled with an irregular exophytic ulcerated mass which bled easily on palpation []. 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.\nPanoramic radiographs demonstrated an ill-defined unilocular radiolucent lesion in the left posterior region of maxilla and the left maxillary sinus was totally obliterated. Computed tomography scan showed extension of the lesion medially and vertically to the nasal septal bone and the inferior orbital rim, respectively [].\nMicroscopic 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. Nuclear hyperchromatism, pleomorphism, and scattered mitotic figures were identified in some areas as well as giant and bizarre cells and the tumor was diagnosed as “ameloblastic fibrosarcoma” accordingly [Figures and ].\nNo 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. Histopathologic examination of the whole specimen confirmed the diagnosis of AFS. All surgical margins were free of tumor infiltration. However, adjuvant radiotherapy was also performed in order to ensure a better outcome. The patient was under close follow up and after 6 months, rehabilitative prosthetic treatment was started. At present, after 2 years, the patient is free of any recurrence and in a good health status [Figures and ].