Cases reported "Jaw Neoplasms"

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1/102. Malignant myoepithelioma of the breast metastasizing to the jaw.

    A breast tumor in a 52-year-old female was interpreted as a malignant myoepithelioma based on morphological and immunohistochemical studies. The tumor consisted of elongated cells with clear cytoplasm and lacked glandular components. The tumor cells were stained positively for keratin, S-100 protein, glial fibrillary acidic protein (GFAP) and muscle-specific actin. Distant metastasis in the right jaw developed 8 years after the initial surgery and the metastatic deposit showed a similar morphology and immunoreactivity. Myoepithelial tumors are generally considered as benign or low-grade lesions and distant metastasis has been rarely documented. The present case presents the possibility of delayed occurrence of distant metastasis in myoepithelial tumor of the breast.
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2/102. multiple myeloma involving the jaws and oral soft tissues.

    A case of multiple myeloma with involvement of the oral cavity in a 75-year-old white man is reported. The patient had a two-year history of multiple myeloma. He had four intraoral soft tissue masses that were diagnosed as plasma cell myeloma. Each of these lesions was over a radiographically evident osteolytic lesion of the jaw. The patient was treated by irradiation and chemotherapy.
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3/102. Well-differentiated intraosseous osteosarcoma of the jaws: experience of two cases from the Instituto Nacional de Cancerologia, mexico.

    Osteosarcomas of the jaws represent less than 10% of all osteosarcomas, and most of them are high-grade neoplasms. Prognostic factors in overall survival include tumor size, location and histologic grade. Examples of well-differentiated (low-grade) intraosseous osteosarcomas of the jaws (WDIOJ) have been rarely reported. This article presents two cases of this unusual lesion, one of which was located in the maxilla of a 17-year-old man and the other developed in the mandible of a 37-year-old woman. CT scan was necessary to detect the small foci of penetration into the thinned cortical bone and the reactive periosteal bone formation, which are important findings to establish the correct diagnosis of WDIOJ and help to exclude other benign intraosseous lesions that may be very similar histologically, such as fibrous dysplasia, ossifying and desmoplastic fibromas. In spite of tumor size (mean 5.2 cm), their well-demarcated borders allowed complete removal of both tumors. There is no evidence of tumoral activity in any of our patients after follow-up periods of 15 months and 5 years. Wide excision seems to be the treatment of choice for this subgroup of osteosarcomas.
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4/102. Periosteal osteosarcoma of the jaws: report of 2 cases.

    osteosarcoma (OS) occurs most often in the long bones. OS of the jaws has clinical and biologic aspects different from those of the long bones. They tend to occur at an older mean age, pain and swelling are more typical, and prognosis is more favorable. Nearly all OS shows a very prominent central intramedullary bone component. Only rarely are juxtacortical (peripheral) OS located in the jaws. There are 2 main types of juxtacortical OS, periosteal and parosteal. We present 2 cases of OS of the jaws where the clinical, radiologic, and histologic findings pointed to a diagnosis of periosteal OS. Both patients presented, in fact, with lesions located superficially on the bone surface with no marrow involvement. Both tumors were characterized by the presence of a moderately differentiated chondroblastic tumor with foci of osteoid and bone formation. Periosteal OS should be differentiated microscopically from periosteal chondrosarcoma, intramedullary OS with periosteal extension, high-grade surface OS, and parosteal OS. The clinical differential diagnosis was done, in these cases, for epulis, gingival tumors, peripheral odontogenic fibroma, peripheral ossifying fibroma, pyogenic granuloma, peripheral giant cell granuloma, and mesenchymal malignant tumors.
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5/102. A genotypic and histopathological study of a large Dutch kindred with hyperparathyroidism-jaw tumor syndrome.

    Familial primary hyperparathyroidism is the main feature of 2 familial endocrine neoplasia syndromes: multiple endocrine neoplasia type 1 (MEN 1) and hyperparathyroidism-jaw tumor syndrome (HPT-JT). The latter is a recently described syndrome that has been associated with ossifying fibroma of the jaw and various types of renal lesions, including benign cysts, Wilms' tumor, and hamartomas. To further illustrate the natural history of this syndrome, we describe a large, previously unreported Dutch kindred in which 13 affected members presented with either parathyroid adenoma or carcinoma; in 5 affected individuals, cystic kidney disease was found. Additionally, pancreatic adenocarcinoma, renal cortical adenoma, papillary renal cell carcinoma, testicular mixed germ cell tumor with major seminoma component, and Hurthle cell thyroid adenoma were also identified. Linkage analysis of the family using MEN1-linked microsatellite markers and mutation analysis excluded the involvement of the MEN1 gene. Using markers from the HPT-JT region in 1q2531, cosegregation with the disease was found, with a maximum logarithm of odds score of 2.41 obtained for 6 markers using the most conservative calculation. Meiotic telomeric recombination between D1S413 and D1S477 was identified in 3 affected individuals, and when combined with previous reports, delineated the HPT-JT region to 14 centimorgan. Combined comparative genomic hybridization and loss of heterozygosity data revealed complex genetic abnormalities in the tumors, suggesting different possible genetic mechanisms for the disease. In conclusion, we report a family with hyperparathyroidism linked to chromosome 1q, and exhibiting several types of renal and endocrine tumors that have not been previously described.
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6/102. Aggressive cemento-ossifying fibroma of the jaws.

    The cemento-ossifying fibroma is probably the most frequent fibro-osseous lesion seen by oral pathologists. Occasionally, reports of lesions behaving in an aggressive fashion appear in the literature. At the present time, the relationship if these "aggressive" lesions to the "usual" fibromas is unclear. Two cases of "aggressive" cemento-ossifying fibroma are reported. Certainly, clinical, radiological, and histological characteristics of aggressive and usual fibromas do coincide. There are, however, diverging features warranting separation of different forms. These mild differences may be detected during preoperative assessment of the lesion, allowing a diagnosis of aggressive form to be made.
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7/102. Primary diagnosis of ameloblastoma by fine-needle aspiration: a report of two cases.

    ameloblastoma is the most common epithelial odontogenic tumor, comprising 1% of tumors and cysts arising in the jaws. We describe two cases of ameloblastoma of the jaw diagnosed by fine-needle aspiration cytology. The patients presented with swelling in the parotid region. Cytological examination of the particulate and sticky bloodstained fluid obtained on aspiration showed tightly packed groups of basaloid cells arranged in nests with rounded edges. Palisading epithelial cells and squamous cells with spherical keratinized bodies were the distinctive cytological features. Histologic examination confirmed the presence of ameloblastoma.
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8/102. Gigantiform cementoma: clinicopathologic presentation of 3 cases.

    Gigantiform cementoma is a rare, benign fibro-cemento-osseous disease of the jaws, seen most frequently in young girls. Radiographically, it typically presents as multiquadrant, expansile, mixed radiolucent-opaque lesions that cross the midlines of the jaws. Although cases with a familial pattern are noted in a few publications, sporadic cases have been reported without a family history. The term gigantiform cementoma has been used interchangeably with designations of other fibro-osseous entities, yet its application should be restricted by the criteria defined herein. In this article, we report 3 cases of gigantiform cementoma. Clinical, radiographic, and microscopic features of these lesions are presented, along with criteria to differentiate gigantiform cementoma from other fibro-osseous diseases of the jaws. A possible pathogenetic mechanism and treatment recommendations are discussed.
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9/102. osteosarcoma of the jaw. The Chaim Sheba Medical Center experience.

    OBJECTIVE: The purpose of this article is to present 14 cases of osteosarcoma of the jaw treated at our medical center from 1989 to 1998. These cases are discussed in the light of a comprehensive review of 774 cases reported in the English literature over the past 3 decades. Differences between osteosarcoma of the jaws and osteosarcoma of the long bones are examined. SUBJECTS AND methods: The patients ranged in age from 8 to 78 years, the mean age being 33 years. Each patient had a histopathologically established diagnosis of osteosarcoma of the jaw. Records were reviewed for epidemiologic data, treatment modalities, and survival. RESULTS: Of the 14 patients, 6 (42%) had tumors in the mandible and 8 (58%) had tumors in the maxilla. Of the mandibular tumors, 5 occurred in the body of the mandible; all maxillary tumors originated in the alveolar ridge and involved the maxillary sinus. The chief complaint was an intraoral or extraoral painless swelling. Histopathologic types included chondroblastic, osteoblastic, fibroblastic, and malignant fibrous histiocytoma-like. Pathologic grade was determined to be high (3 or 4) in 13 cases and low (1) in only 1 mandibular case. All patients underwent surgical resection and immediate reconstruction. Adjuvant therapy included postoperative radiation (5 patients), postoperative chemotherapy (2 patients), and preoperative chemotherapy and postoperative radiation (1 patient). CONCLUSIONS: The results of the present study support the literature indicating that osteosarcoma of the jaw differs from osteosarcoma of the long bones in its biological behavior even though they have the same histologic appearance. Because of differences in tumor characteristics, the introduction of chemotherapy did not dramatically alter the prognosis of osteosarcoma of the jaw. early diagnosis and radical surgery are the keys to high survival rates.
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10/102. Desmoplastic ameloblastoma (including "hybrid" lesion of ameloblastoma). Biological profile based on 100 cases from the literature and own files.

    The profile of the desmoplastic ameloblastoma (DA) is presented, based on a survey of 100 cases. DA is a benign, locally invasive variant of the intraosseous, infiltrative ameloblastoma (IA). Radiologically, the DA shows ill-defined borders with a soap bubble appearance. The finding of a mixed radiolucency-radiopacity in 52% of the cases often leads to a preoperative diagnosis of a fibro-osseous lesion. association with an unerupted tooth is not a typical finding. The DA accounts for 4-13% of all ameloblastomas. The over-all average age is 42.9 years, higher for males (45.9 years) than for females (39.7 years). Thus, the DA occurs at a slightly higher age than the intraosseous ameloblastoma (37.4 years). The male/female ratio is 1:1. The maxilla/mandible ratio is 1/0.9 as opposed to 1/5.4 for the IA. Seven maxillary tumours involved an entire quadrant and 15 maxillary and mandibular tumours crossed the midline of the jaws. Pathogenetically, it seems that the DA is derived from the same sources as the IA, and extra-osseous sources do not seem to play a role. Histologically, the DA reveals the following morphological characteristics: the odontogenic epithelium occurs as irregular, stellate or follicular islands and cords, the center often appearing hypercellular with spindle-shaped or squamatoid cells. Peripheral columnar or cuboidal cells rarely reveal an ameloblast-like appearance. Thus, there are only vague tendencies to mimic a follicular ameloblastoma with acanthomatous features. The most striking feature separating the DA from IA is to be found in the tumour stroma: in DA there is extensive stromal desmoplasia with an abundance of thick collagen fibres that seem to compress the epithelial islands. Nine cases of so-called "hybrid lesion of ameloblastoma" where areas of IA coexist with areas of DA are surveyed in addition to the 100 cases of DA. No conclusions can be drawn about the biological profile of this variant due to the insufficient number of cases.
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