1/67. Lesions associated with familial polyposis coli: studies of lesions of lesions of the stomach, duodenum, bones, and teeth.The entire gastrointestinal tract and bones were examined systematically in 24 patients (10 pedigrees) in whom familial polyposis coli was diagnosed. Polypoid lesions were observed in the stomach in as high as 68.2% of the cases, and in the duodenum in 90%. Abnormalities were noted in the skeleton in 50% and in the mandible in 81.3%. The authors emphasize the possibility that familial polyposis coli is substantially the same entity as Gardner's syndrome.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
2/67. Squamous odontogenic tumor. Report of six cases of a previously undescribed lesion.Six cases of a previously unnamed oral lesion are described and the name squamous odontogenic tumor is applied. The lesion causes radiolucent areas of bone destruction adjacent to the roots of teeth. In one case there were multiple separate lesions. light microscopy revealed numerous islands of benign squamous epithelium with focal areas of keratinization and calcification. Electron microscope examination in one case showed normal squamous epithelial cells with abundant glycogen granules, tonofilaments and myelin bodies. Complete surgical excision seems the best therapy, as only one lesion recurred following treatment.- - - - - - - - - - ranking = 0.2keywords = bone (Clic here for more details about this article) |
3/67. 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.- - - - - - - - - - ranking = 0.4keywords = bone (Clic here for more details about this article) |
4/67. 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.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
5/67. 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.- - - - - - - - - - ranking = 0.65185556203672keywords = endocrine (Clic here for more details about this article) |
6/67. A case of Burkitt's lymphoma that presented initially with resorption of alveolar bone.A 16-year-old male was evaluated for a 1-month history of alveolar bone resorption, which had been treated with endodontics by a neighborhood dentist. Intraoral examination showed slight gingival swelling and teeth mobility. However, no tumor mass was seen. The panoramic image showed resorption of alveolar bone and loss of teeth lamina dura. Because he complained of general fatigue, he was introduced to the internist. Biopsies of gingiva and bone marrow aspiration revealed a massive proliferation of lymphoblasts expressing CD10, 19, 20 and hla-dr antigens on the surface. Their karyotypes were abnormal; 46, XY, t (8;14) (q24;q32). Accordingly, he was diagnosed as Burkitt's lymphoma, and received intensive chemotherapy which relieved his symptoms and decreased his tumor. However, his disease soon became refractory to chemotherapy, and he died 11 weeks after the onset.- - - - - - - - - - ranking = 1.4keywords = bone (Clic here for more details about this article) |
7/67. Multiple odontomas in the facial bones. A case report.A rare case of multiple compound odontoma involving the facial bones and erupting into the oral cavity of a 15-year-old Nigerian girl is presented. The unacceptable facial appearance and the surgical approach used makes this case worth reporting.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
8/67. 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.- - - - - - - - - - ranking = 0.4keywords = bone (Clic here for more details about this article) |
9/67. Unusual presentation of mastoid eosinophilic granuloma in a young patient.eosinophilic granuloma is a well-recognized form of Langerhans cell histiocytosis, most commonly involving the skull bones, usually with an excellent prognosis. Recurrent and difficult to recognize osteolytic lesions of the skull are encountered only rarely. A patient with recurrent eosinophilic granuloma of the skull is reported. In spite of appropriate multimodality treatment, there were several recurrences, most recently with involvement of the mastoid process. Imaging studies revealed extensive involvement of surrounding structures with expansion of the tumor into the middle cranial fossa and slight pressure on the antero-medial portion of the temporal lobe of the brain. Despite extensive involvement, the patient had no complaints. Because of the rarity of such silent and unpredictable lesions, a systematic approach with regular CT and MRI follow-up is suggested.- - - - - - - - - - ranking = 0.2keywords = bone (Clic here for more details about this article) |
10/67. Gnathodiaphyseal dysplasia: a syndrome of fibro-osseous lesions of jawbones, bone fragility, and long bone bowing.We report an unusual generalized skeletal syndrome characterized by fibro-osseous lesions of the jawbones with a prominent psammomatoid body component, bone fragility, and bowing/sclerosis of tubular bones. The case fits with the emerging profile of a distinct syndrome with similarities to previously reported cases, some with an autosomal dominant inheritance and others sporadic. We suggest that the syndrome be named gnathodiaphyseal dysplasia. The patient had been diagnosed previously with polyostotic fibrous dysplasia (PFD) elsewhere, but further clinical evaluation, histopathological study, and mutation analysis excluded this diagnosis. In addition to providing a novel observation of an as yet poorly characterized syndrome, the case illustrates the need for stringent diagnostic criteria for FD. The jaw lesions showed fibro-osseous features with the histopathological characteristics of cemento-ossifying fibroma, psammomatoid variant. This case emphasizes that the boundaries between genuine GNAS1 mutation-positive FD and other fibro-osseous lesions occurring in the jawbones should be kept sharply defined, contrary to a prevailing tendency in the literature. A detailed pathological study revealed previously unreported features of cemento-ossifying fibroma, including the participation of myofibroblasts and the occurrence of psammomatoid bodies and aberrant mineralization, within the walls of blood vessels. transplantation of stromal cells grown from the lesion into immunocompromised mice resulted in a close mimicry of the native lesion, including the sporadic formation of psammomatoid bodies, suggesting an intrinsic abnormality of bone-forming cells.- - - - - - - - - - ranking = 3.4keywords = bone (Clic here for more details about this article) |
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