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Adenomatoid odontogenic tumour: immunohistochemical demonstration of transferrin, ferritin and alpha-one-antitrypsin.

Three cases of adenomatoid odontogenic tumour (AOT) were examined by morphological and immunohistochemical methods, to define the nature of tumour cells and to determine the correlation between the occurrence of extracellular eosinophilic amorphous material and epithelial tumour cells. The epithelial tumour cell components observed in this study were divided into three cell types (cell type I: small compact cells in a solid nodule and pseudoglandular cells in a duct-like structure; cell type II: peripheral elongated cells and spindle-shaped cells in a cribriform pattern; and cell type III: metaplastic squamous cells). The mesenchymal components consisted of eosinophilic amorphous material and calcified material. Immunohistochemically, the type I cells reacted positively with antibodies to transferrin, ferritin and alpha-one-antitrypsin (alpha1-AT), whereas the type II cells constantly indicated intense expression only for transferrin and alpha1-AT. All types of epithelial tumour cells reacted negatively with lactoferrin, alpha-one-antichymotrypsin, S-100 protein, S-100alpha subunit and S-100beta subunit. Moreover, the eosinophilic amorphous material and calcified material examined were positive for the antibody against alpha1-AT. These materials expressed immunophenotypes similar to those of the epithelial tumour cells, except for metaplastic squamous cells. The present study showed that iron-binding proteins and proteinase inhibitor might be related to the pathogenesis of AOT. Furthermore, we indicated that the formation of eosinophilic amorphous material was associated with type I and type II cells.

Adolescent↗

Odontogenic tumors in Chile: a study of 362 cases.

BACKGROUND: Odontogenic tumors are infrequent lesions. Thus, the review of a large number of cases becomes a necessity for both the pathologist and the clinician. Studies on odontogenic tumors have been published in many parts of the world, but there is little information available in the English language literature on the relative frequency of odontogenic tumors in Latin America. The aim of this study was to determine the relative frequency of this heterogeneous group of lesions in a Chilean population, and to compare these data with previous reports. METHODS: We reviewed the records of 28,041 specimens from 1975 to 2000 in the Oral Pathology Referral Institute (IREPO), and using the criteria for histological typification published by the World Health Organization in 1992, we reclassified the odontogenic tumors. RESULTS: We confirmed a total of 362 odontogenic tumors. The frequency of odontogenic tumors as a percentage of all pathological specimens in our institute was 1.29%. The most frequent histological type was odontomas (44.7%), followed by ameloblastomas (20.4%) and myxomas (8.8%). CONCLUSIONS: Odontogenic tumors are uncommon lesions in the Chilean population and malignant odontogenic tumors are very rare. The relative frequency of various types of odontogenic tumors, as well as the age and gender distribution are similar to those reported in the North American series and different from those found in recently published Asian and African series.

Adolescent↗

Multiple cemento-ossifying fibroma: report of an 18-year follow-up.

Multiple cemento-ossifying fibroma is a very rare jaw lesion. A unique case is reported involving a 43-year-old Korean woman with rapidly growing, multiple cemento-ossifying fibromas in all four quadrants over an 18-year period which resulted in severe facial deformity and orbital compression.

Adult↗

Radiological and clinical features of calcifying epithelial odontogenic tumour.

OBJECTIVES: To analyse systematically the radiographic features of the calcifying epithelial odontogenic tumour (CEOT). METHOD: Clinical and radiological features of 67 cases of CEOT (four new and 63 from the literature) were analysed. RESULTS: There were 27 (41%) males and 39 (59%) females with a male-to-female ratio of 1 : 1.5. Age ranged from 13 - 77 years (mean 43.5 years), with a peak in the fourth and fifth decades. The mandible was involved in 74% of the cases and 69% of all lesions were in the posterior area. The mixed radiolucent-radiopaque pattern was the most frequent (65%), with 32% radiolucent and 3% radiopaque. Coronal clustering of the radiopaque material was found in 12% and in one case, a 'driven snow' pattern of the radiopaque material was clearly recognizable. Lesions were unilocular in 58%, multilocular in 27% and not loculated in 15%. The unilocular type was more frequent in the maxilla than in the mandible. Borders were well defined and corticated in 20%, defined but not corticated in 59% and diffuse in 21%. Sixty per cent were associated with impacted teeth. Lesions larger than 3 cm tended to be more frequent in the mandible, mixed radiolucent-radiopaque and to have a higher proportion of diffuse borders than the smaller lesions. CONCLUSION: Radiographic features which have been considered characteristic of CEOT, coronal clustering and 'driven snow' patterns, are seen in only a small percentage of cases.

Adult↗

Hyperparathyroidism-jaw tumour syndrome.

Amongst hyperparathyroidism-related syndromes, hyperparathyroidism-jaw tumour syndrome is one of the least common and relatively unknown but its clinical and genetic aspects are not less interesting or important. With the recent identification of its genes, we can now better characterize the disease, both clinically and genetically, which will certainly impact the field of endocrinology and oncology. In this article, we review the clinico-pathological features and genetic basis of this syndrome with the hope that it will create awareness and interest in this disease amongst clinicians and basic scientists.

Genes, Tumor Suppressor↗

Immunohistochemical expression of neural tissue markers (neuron-specific enolase, glial fibrillary acidic protein, S100 protein) in ameloblastic fibrodentinoma: a comparative study with ameloblastic fibroma.

Formalin-fixed paraffin-embedded sections of three cases of ameloblastic fibrodentinoma (AFD) were studied by the avidin-biotin-peroxidase complex method using antibodies against neuron-specific enolase (NSE), glial fibrillary acidic protein (GFAP) and S100 protein and the results were compared with those in ameloblastic fibroma (AF). A striking histopathological characteristic of AFD was the formation of abortive dentin with various degrees of maturation at the epithelial-mesenchymal tissue interface. Central cells of enamel organ-like epithelia with various stages of abortive dentin induction in AFD were generally positive for NSE. Dental lamina-like epithelial cells also showed positive staining in some areas. No cells were positive for NSE in AF. Positive staining for GFAP was observed in the juxta-epithelial mesenchymal tissue of the formation stage of immature dentin with various numbers of entrapped cells in AFD, but GFAP staining was negative in other mesenchymal and epithelial tissues at other stages. In AF, no GFAP-positive cells were found. There were a few S100 protein-positive cells found in the foci of epithelial components in both AFD and AF. Mesenchymal cells showing a dendritic or spindle shape were positive for S100 protein in some areas of AFD and AF. Although such cells in the mesenchymal component of pigmented AFD were more numerous than in non-pigmented AFD and AF, their distribution pattern in the former condition was basically similar to that in the latter. Although the present results, obtained from conventional immunohistochemical procedures, do not directly reflect the expression of neural crest-derived cells in the dentinogenesis of AFD, such results do not disprove the possibility of the expression of neural proteins probably related to neural crest-derived cells in dentinogenesis under certain pathologic conditions in odontogenic mixed tumors. Such a phenomenon may also occur during dentinogenesis in other odontogenic mixed tumors and in normal tooth differentiation, but at an undetectable level.

Adult↗

Dermoid cyst of the maxillary sinus.

Dermoid cysts are rare developmental teratomatous lesions composed of ectodermally derived stratified squamous epithelium and mesodermally derived skin adnexal structures. As part of the teratomatous lesion group, dermoid cysts are related to teratoid cysts, true teratomas, and epignathi. Although several theories have been postulated, the pathogenesis of dermoid cysts, and teratomatous lesions in general, is unclear. Most commonly affecting sites within the head and neck, dermoid cysts may be found in the frontotemporal/lateral brow area, central nasal area, oral cavity, lateral neck, and other sites. We present what is believed to be the first reported case arising within the maxillary sinus and briefly discuss the possible pathogenesis.

Adult↗

Malignant ameloblastoma: classification, diagnostic, and therapeutic challenges.

Ameloblastoma is a benign odontogenic neoplasm of the mandible and maxilla that rarely exhibits malignant behavior. We report the case of an aggressive malignant ameloblastoma of the mandible that presented with an unusual multiphasic, histologic pattern. Initial fine needle aspiration and radiographic findings showed features consistent with a benign, fibro-osseous lesion. However, aggressive growth and the association of enlarged submandibular lymph nodes suggested a more malignant potential. Treatment consisted of an angle-to-angle composite mandibular resection, right modified neck dissection, left functional supraomohyoid neck dissection, and anterior chin skin resection with iliac crest osteocutaneous free flap reconstruction. Microscopic evaluation showed primarily malignant ameloblastoma without cellular atypia and extensive fields of fibro-osseous tissue with smaller fields of clear cell odontogenic tumor. This multiphasic, histologic arrangement may explain the perplexing preoperative microscopic diagnosis, suggesting a benign fibro-osseous lesion. Of the lymph nodes analyzed, one from the right submandibular triangle exhibited metastatic, benign-appearing ameloblastoma without fibro-osseous or clear cell features. The absence of cellular features of malignancy in the tumor mass and lymph node metastasis suggest that the lesion should be classified as malignant ameloblastoma rather than ameloblastic carcinoma or odontogenic carcinoma. A malignant ameloblastoma with all 3 of the aforementioned microscopic features has not been previously reported. We review the classification of epithelial odontogenic malignancies. Lesions showing multiphasic patterns can create diagnostic dilemmas and may require extensive surgical sampling and/or removal to establish an accurate diagnosis.

Aged↗

Clear cell odontogenic carcinoma: evaluation of reported cases.

PURPOSE: We present the cases of 4 patients with clear cell odontogenic carcinoma and a contrasting case of a patient with a calcifying epithelial odontogenic tumor from Massachusetts General Hospital. Differential diagnosis, distinguishing characteristics, and data from 32 cases in the literature are also reported. Because this rare tumor behaves aggressively with both regional and distant spread, a better understanding of its biologic behavior is imperative. PATIENTS AND METHODS: The 4 patients with clear cell odontogenic carcinoma were women and had an average age of 65 years (range, 40 to 77 years). Three tumors were in the posterior mandible and 1 was in the maxilla. Three patients complained of pain; bony expansion and tooth mobility were noted in isolated cases. A comparison is made with other reported cases, and factors associated with recurrence and survival are presented. RESULTS: All patients were treated with resection, and 1 patient received postoperative radiotherapy. Two patients with positive surgical margins underwent further resection. All patients have been followed an average of 2 years (range, 0.5 to 4 years) and presently have no evident disease. CONCLUSIONS: Recognition of this unusual tumor and differentiating it from other clear cell neoplasms is critical in developing an appropriate treatment plan. Management should include wide en bloc resection and long-term follow-up.

Adenocarcinoma, Clear Cell↗

Soft tissue sarcoma of the head and neck region in adults.

Soft tissue sarcomas (STS) are rare solid tumours accounting for less than 1% of all malignancies and are very unusual in the head and neck region. Histopathologically diagnosed cases of STS treated at Regional Cancer Centre (RCC). Trivandrum, India, between January 1989 and November 2000 were analyzed retrospectively. Survival analysis was carried out by Kaplan-Meier method and curves were compared using log rank test. A total of 22 cases were seen during the study period. The mean age of the patients was 36.4 years with male-to-female ratio of 2:1. The neck, lower jaw, tongue, cheek, scalp and maxilla were the commonest sites affected. None of the patients had palpable neck nodes or distant metastasis at presentation. All the patients were treated with primary surgical resection and this was followed by adjuvant treatment in 14 cases (63.6%). After a median follow-up of 14.5 months, two patients died, six developed local recurrence, four developed metastatic disease and another patient developed second primary sarcoma. The overall 5-year survival was 80% while the 5-year disease-free survival rate was 24.1%. The majority of the patients failed within first and second year. None of the parameters except grade of tumour (P=0.008) were found to have a significant effect on survival. The overall survival rate for patients with soft tissue sarcoma of the head and neck is good, however, disease-free survival is poor as the majority of these fail within 2 years of completing treatment.

Adolescent↗

Oral and maxillofacial tumours in north Jordanian children and adolescents: a retrospective analysis over 10 years.

This study evaluates the types and distribution of oral and maxillofacial tumours in north Jordanian children and adolescents. The records of the Department of Pathology at Jordan University of Science & Technology, during the period 1991-2000, were reviewed for patients younger than 19 years with oral and maxillofacial tumours. The tumours were analysed for age, sex, site and type. Out of all maxillofacial tumours, 23% occurred in children and adolescents, of these 10% were malignant and 90% were benign. 73% of the latter were soft tissue tumours and 17% were jaw tumours (58% odontogenic and 42% non-odontogenic). The most common benign soft tissue and jaw tumours were haemangioma and odontoma, respectively. 58% of malignant tumours were sarcomas and 42% were carcinomas. The mean age was 11 years with a female to male ratio of 1:1.2. The intraoral and extraoral sites most commonly affected by benign soft tissue tumours were the lower lip and face, respectively. While the total number of benign jaw tumours was slightly larger in the maxilla than the mandible, odontogenic tumours were more in the maxilla, and nonodontogenic tumours were more in the mandible. Differences found in this study between Jordanian children and adolescents and those from other countries may be attributable to genetic and geographic differences. The majority of benign tumours in the young are probably developmental rather than true neoplasms.

Adolescent↗

CT findings in primary osteosarcoma of the jaw.

Findings of computed tomography (CT) in five patients with osteosarcoma of the jaw are presented. CT played an important role in the assessment of the tumour extent before the planning of treatment for both primary and recurrent jaw osteosarcoma. Plain CT was capable of demonstrating subtle bony change (cortical reaction and calcification) and medullary tumour extension without interference from other superimposed bones. Tumour vasculature was well represented by thin-slice CT scan with intravenous bolus injection of contrast medium. We emphasize the importance of preoperative CT scanning from both a radiological and surgical point of view when confronted with a tumour of the maxilla or mandible.

Adult↗

[Ossifying fibroma of the bone].

In the spectrum of the fibroosseous bone tumours the ossifying fibroma of the bone (OF) is situated as a not yet clearly defined entity with many names. The present paper deals with the question wether a term innovation brings elucidation to clinical management. Therefore 811 previously published and 16 additional cases have been analysed with respect to their clinical and radiological behavior. In addition a simplificated histological reclassification was performed on 284 well documented literature cases. It was found that there are no great differences between these tumours according to their localisation, their clinical behavior, and their prognosis. All tumours of this group are suitable to different categories of histological maturation of the same tumour best called "ossifying fibroma".

Adolescent↗