Cases reported "Odontogenic Tumors"

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1/13. The calcifying epithelial odontogenic tumour-- report of four cases; two with long-term follow-up.

    Four cases of calcifying epithelial odontogenic tumour are reported, all with pain as a presenting symptom. Two of the cases have long-term follow-up, one with a recurrence after 16 years and the other with no recurrence after 24 years. Only 17 per cent of cases reported in the literature have follow-up of 10 years or more, and yet the possibility of recurrence after this length of time must be considered. Recommendations for treatment are made based on the biological behaviour and recurrence rate following various types of treatment reported in the literature. It is recommended that patients in whom the diagnosis of calcifying epithelial odontogenic tumour has been made should be reviewed continuously.
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2/13. Ameloblastic fibrosarcoma: report of a case. Immunohistochemical study and review of the literature.

    Ameloblastic fibrosarcoma is a rare malignant odontogenic tumour characterized by a benign epithelial component within a malignant fibrous stroma. Its behaviour is relatively benign, with absence of metastatic disease, and the prognosis is reported to be good. It is a paradoxical neoplasm with "sarcomatous" morphological and immunohistochemical patterns but with a favourable clinical course. We report a new case of this tumour in a mandibular ramus of a 31-years-old male patient, that was surgically excised and treated with adjuvant chemotherapy and radiotherapy. Five years later the patient is free of disease. The growth potential of ameloblastic fibrosarcoma is evaluated and compared with a related lesion, the ameloblastic fibroma. The sarcomatous mesenchymal component of ameloblastic fibrosarcoma is positive to Ki67, PCNA and p53, in front of the negativity of ameloblastic fibroma.
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3/13. Unusual jaw lesions in the paediatric and adolescent patient: a management challenge.

    While major maxillofacial pathology in the young patient is relatively uncommon, non-malignant conditions may pose a significant treatment dilemma due to their aggressive or unpredictable behaviour. Several such diseases managed by the Oral and Maxillofacial Surgery Unit at the Royal Children's Hospital of Melbourne have been selected for review. Illustrative case reports to highlight the principles of management are presented.
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4/13. Genetic portrait of malignant granular cell odontogenic tumour.

    Odontogenic tumours are rare neoplasms whose classification is sometime controversial. Among these entities, granular cell odontogenic tumour (GCOT) is extremely rare and usually has a benign clinical behaviour. While the histogenesis of GCOT remains to be clarified, we documented the existence of a malignant counterpart of this neoplasm and proposed the name of malignant GCOT. Expression profiling by cDNA microarrays is a molecular technology that enables a global gene expression analysis. By using cDNA microarrays, we identified in malignant GCOT several genes with significantly differentially regulated genes when compared to non neoplastic tissues. These cancer specific genes include a range of functional activities: (1) transcription, (2) signaling transduction, (3) cell-cycle regulation, (4) apoptosis, (5) differentiation and (6) angiogenesis. In conclusion, we show that cDNA microarrays is a useful approach to investigate the biology of tumours. Moreover, this technology might lead to identification of gene targets for cancer therapy and to molecular classification of odontogenic tumours.
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5/13. Central granular cell odontogenic tumour: report of the first malignant case and review of the literature.

    Granular cell odontogenic tumours (GCOT) are rare neoplasms that usually manifest a benign clinical behaviour. We document the first case of GCOT exhibiting clinico-pathological features of malignancy that occurred in the maxilla of a 40-year-old male. The lesion appeared as an intra-oral polypoid mass and, at CT scan, as a poorly demarcated radiolucency eroding the cortical plate. Histologically, the tumour consisted of clusters of granular cells, exhibiting nuclear pleomorphism, prominent nucleoli and mitotic figures, and spindle cells in a collagenous stroma containing cementicles and strands of odontogenic epithelium. Morphologic transition from fibroblast-like to granular cells was frequently detected. The tumour cells extensively invaded the oral and respiratory mucosae and the adjacent soft tissues and exhibited vimentin and CD 68 immunoreactivity and high (21%) Ki 67 immunolabeling but not cytokeratins, E.M.A. actin, desmin, myosin or S-100 protein positivity. The patient experienced tumour recurrence 16 months after radical surgery. While the histogenesis of GCOT remains to be clarified, we document the existence of a malignant counterpart of this tumour and propose the name of malignant GCOT or granular cell odontogenic sarcoma for such entity.
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6/13. Metastasising clear cell odontogenic carcinoma: a case report and review of the literature.

    Primary odontogenic carcinomas are rare and examples which have metastasised are even more uncommon. We describe the first reported case of a clear cell odontogenic carcinoma which metastasised to distant bones, namely the 5th lumbar vertebra and hip, 3 years after initial diagnosis. The initial incisional biopsy was thought to represent a calcifying epithelial odontogenic tumour, but in the subsequent resection the tumour showed a prominent clear cell component admixed with squamous cells showing peripheral palisading, widespread infiltration and necrosis indicating a malignant neoplasm. Radiologically guided biopsy revealed a metastatic lesion in L5 vertebrae and left hip, confirmed by immunohistochemistry. The metastatic lesion had similar appearances to the first biopsy, and diagnosis was confirmed by comparison of histological features, immunohistochemistry and exclusion of a second primary lesion by clinical examination and imaging. The diagnosis of clear cell odontogenic carcinoma is a difficult one to make. The behaviour of these tumours is unpredictable. This case confirms that clear cell odontogenic carcinomas have the potential for distant metastasis and require long-term follow up.
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7/13. Ameloblastic carcinoma: case report and literature review.

    Ameloblastic carcinoma is a rare malignant lesion with characteristic histologic features and behaviour that dictates a more aggressive surgical approach than that of a simple ameloblastoma. However, reliable evidence of its biologic activity is currently unavailable due to the scarcity of well-documented cases. It occurs primarily in the mandible in a wide range of age groups; no sex or race predilection has been noted. It may present as a cystic lesion with benign clinical features or as a large tissue mass with ulceration, significant bone resorption and tooth mobility. Because the lesion is usually found unexpectedly after an incisional biopsy or the removal of a cyst, a guide to differential diagnosis is not usually useful. The identifying features of ameloblastic carcinoma must be known and recognized by dental practitioners. Our understanding of the histologic features of ameloblastic carcinoma is somewhat vague. The tumour cells resemble the cells seen in ameloblastoma, but they show cytologic atypia. Moreover, they lack the characteristic arrangement seen in ameloblastoma. The clinical course of ameloblastic carcinoma is typically aggressive, with extensive local destruction. Direct extension of the tumour, lymph node involvement and metastasis to various sites (frequently the lung) have been reported. Wide local excision is the treatment of choice. Regional lymph node dissection should be considered and performed selectively. radiotherapy and chemotherapy seem to be of limited value for the treatment of ameloblastic carcinomas. At the moment, there are too few reported cases to make a definite recommendation regarding treatment. Close periodic reassessment of the patient is mandatory.
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8/13. Ameloblastic fibrosarcoma or odontogenic carcinosarcoma: a matter of classification?

    A biphasic odontogenic tumor with aggressive clinical behaviour is reported. The tumor arose posterior to the right mandibular third molar involving the angle of the mandible and the ascending ramus. Within a 5 years period the patient suffered from two episodes of local progression and final disease generalization occurred 6 years after initial surgical therapy. On histopathologic evaluation, both recurrences and the distant metastasis preserved the biphasic pattern seen in the primary tumor with both epithelial and mesenchymal components exhibiting clear cytological features of malignancy. There was no evidence of sarcomatous overgrowth within the progression of the tumor. By contrast, the proportion of the epithelial component was even enlarged in the second recurrence. Thus, both pathologic features and clinical behaviour clearly distinguishes this tumor from ameloblastic fibrosarcoma and demonstrates the clear morphological as well as clinical characteristics of a true malignant mixed type tumor with a definite sarcomatous and carcinomatous component. Thus, even though a very rare lesion, our case supports the consideration of the odontogenic carcinosarcoma as an individual entity.
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9/13. Salvage of an impacted canine associated with an adenomatoid odontogenic tumour: a case report.

    The adenomatoid odontogenic tumour (AOT) has been known by a number of descriptive names (adenoameloblastoma, ameloblastic adenomatoid tumour, glandular ameloblastoma, and adenomatoid ameloblastoma) since it was first reported and later recognised as a distinct odontogenic lesion unrelated to ameloblastoma. Although it was considered to be a variant of ameloblastoma at one time leading surgeons to perform unduly aggressive surgery, the treatment outcome experience has borne out the benign, nonaggressive nature of this lesion. The AOT is now considered to be a hamartoma with completely benign behaviour. recurrence seldom if ever occurs after surgical curettage. Thus, it appears needless to extract involved anterior teeth associated with the tumour, especially in children. We report a case in which surgical and orthodontic treatment helped to salvage an impacted mandibular canine associated with an AOT in an adolescent girl. We have not found such a procedure to have been done for this tumour in the literature.
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10/13. Central odontogenic fibroma interesting the maxillary sinus. A case report and literature survey.

    Odontogenic fibroma (OF) is a rare benign odontogenic tumor deriving from the dental mesenchymal tissue and accounting for less than 5% of all odontogenic tumors. This paper presents an aggressive histologically diagnosed central odontogenic fibroma (COF) in a 17-year-old girl characterized by asymptomatic rapid growth with massive replenishment of the left maxillary sinus. We carried out a review of the literature to retrieve all published cases of COF especially focused on radiographic aspects and surgical treatment of cases characterized by clinical aggressive behaviour, as we observed in our patient. Search strategy included retrieval of English language papers, published from 1966 to today, in dental journals on medline/pubmed and EMBASE, and hand-searching of the bibliography of retrieved papers. Sixty-nine cases of COF were identified from 1954 to 2003 and a new one was added. We have compared characteristics of COFs according to age, gender, location, clinical and radiographic findings of aggressive development, and histology. We discuss clinical and radiographic aspects of our case compared with COFs previously published. We give suggestions for surgical treatment of COF in case of aggression to important anatomical structures.
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