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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nora's tumour is an uncommon benign tumour, involving mostly tubular bones of hands and feet, and characterized by a proliferation of chondroid, bony and fibrous tissues. Main differential diagnoses are osteochondroma, chondrosarcoma, and osteosarcoma. The authors report a case involving the left foot. The diagnosis was suspected by imaging characteristic features and was confirmed by histological examination. Treatment was surgical, with complete excision. There are no clinical or radiological signs of recurrence on further review one year postoperatively.
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PMID:Bizarre parosteal osteochondromatous proliferation (Nora's lesion) of the forefoot. 1881 Oct 47

A retrospective analysis of all bone tumors accessioned at a large referral center (Instituto Nacional de Rehabilitacion) in Mexico City between 2000 and 2005 is presented. A total of 6216 biopsies and surgical resection specimens were reviewed during this period, of which 566 corresponded to bone tumors. Benign bone tumors accounted for 71.6% of cases and malignant bone tumors for 28.4%. The tumors affected men in 53.7% of cases and women in 46.3% of cases, with an average age at presentation of 25 years. The femur was the most common location of the tumors (39.9%), followed by the tibia (17.7%) and humerus (11.8%). The commonest malignant bone tumors were osteosarcoma (46.6%) and chondrosarcoma (8.7%). Of malignant bone tumors, 18.6% corresponded to metastases of carcinomas from internal organs and 8.1% were multiple myeloma. The most common benign bone tumor was osteochondroma (43.7%) followed by giant cell tumor of bone (14.6%) and enchondroma (10.1%). The age distribution showed a peak in children and adolescents comprised predominantly of benign lesions and a second peak in young adults that corresponded to malignant bone tumors (principally osteosarcoma). Malignant bone tumors most often involved the femur, vertebra, and tibia. Our results parallel the findings previously reported in the world literature and show a similar distribution and epidemiology as in other developed and underdeveloped countries. Geographic location does not appear to represent a risk factor for any particular type of bone tumor and does not affect the age distribution, location, or histopathologic type of the lesions.
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PMID:Epidemiology of bone tumors in Mexico City: retrospective clinicopathologic study of 566 patients at a referral institution. 1911 77

This article provides a review of the MR imaging features of the major primary malignant and benign bone tumors and tumorlike conditions encountered in the pediatric population. Malignant tumors discussed include osteosarcoma, Ewing sarcoma, chondrosarcoma, lymphoma, and malignant fibrous histiocytoma. Benign lesions discussed include simple bone cysts, aneurysmal bone cysts, giant cell tumor, osteochondroma, enchondroma, chondroblastoma, osteoid osteoma, osteoblastoma, nonossifying fibroma, fibrous dysplasia, osteofibrous dysplasia, hemangioma, and histiocytosis. The use of MR imaging in the diagnosis of these lesions is discussed, and the text is enhanced with imaging examples of the lesions.
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PMID:MR imaging of primary bone tumors and tumor-like conditions in children. 1952 97

Studies to determine the etiology of osteosarcoma involve epidemiologic and environmental factors and genetic impairments. Factors related to patient characteristics include age, gender, ethnicity, growth and height, genetic and familial factors, and preexisting bone abnormalities. Rapidly proliferating cells may be particularly susceptible to oncogenic agents and mitotic errors which lead to neoplastic transformation. Genetic aberrations that accompany osteosarcoma have received increasing recognition as an important factor in its etiology. Osteosarcoma tumor cells exhibit karyotypes with a high degree of complexity which has made it difficult to determine whether any recurrent chromosomal aberrations characterize osteosarcoma. Although extremely rare, osteosarcoma has occasionally been observed in several members of the same family. No other clinical abnormalities in the proband or the affected members were reported. Pathologic examination of the tumors revealed no unusual features. Genetic testing was not available in most of these reports. The patients generally responded to conventional therapy. A genetic predisposition to osteosarcoma is found in patients with hereditary retinoblastoma, characterized by mutation of the retinoblastoma gene RB1 on chromosome 13q14. The Rothmund-Thomson syndrome is an autosomal recessive disorder with a heterogeneous clinical profile. Patients may have a few or multiple clinical features including skin rash, small stature, skeletal dysplasias, sparse or absent scalp hair, eyebrows or eyelashes, juvenile cataracts, and gastrointestinal disturbance including chronic emesis and diarrhea; its molecular basis is the mutation in the RECQL4 gene in a subset of cases. The Li-Fraumeni syndrome is an autosomal dominant disorder characterized by a high risk of developing osteosarcoma and has been found in up to 3% of children with osteosarcoma. It is associated with a germline mutation of the p53, a suppressor gene. The following three criteria must be met for a diagnosis of Li-Fraumeni syndrome: (1) A proband diagnosed with sarcoma when younger than 45 years; (2) A first-degree relative with any cancer diagnosed when younger than 45 years; (3) Another first- or second-degree relative of the same genetic lineage with any cancer diagnosed when younger than 45 years or sarcoma diagnosed at any age. A second recessive p53 oncogene on chromosome 17p13.1 may also play a role in the development and progression of osteosarcoma. Osteosarcoma has also been associated with solitary or multiple osteochondroma, solitary enchondroma or enchondromatosis (Ollier's disease), multiple hereditary exostoses, fibrous dysplasia, chronic osteomyelitis, sites of bone infarcts, sites of metallic prostheses and sites of prior internal fixation. Ionizing radiation is a well-documented etiologic factor. Osteosarcoma has also been associated with the use of intravenous radium and Thorotrast. Exposure to alkylating agents may also contribute to its development ,and it is apparently independent of the administration of radiotherapy.
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PMID:The etiology of osteosarcoma. 2021 84

Benign and malignant tumors in the temporomandibular joint (TMJ) are rare. However, when a patient presents with clinical findings such as altered occlusion or facial asymmetry, a morphologic alteration in the condyle should be ruled out. The differential diagnosis for benign hyperplastic bony lesions in the TMJ should include condylar hyperplasia, osteochondroma, osteoma, chondroma, and osteoblastoma. If malignant features are present, chondrosarcoma and osteosarcoma should be considered. For the differential diagnosis, imaging is the most noninvasive method to evaluate the integrity of the TMJ. Imaging can be classified as morphologic or functional according to the information provided. The current scientific data have shown that panoramic images have 97% sensitivity and 45% specificity for identifying hyperplastic conditions in the TMJ. The sensitivity and specificity of medical computed tomography (CT) and cone-beam CT is 70% and 100%, and 80% and 100%, respectively, for the detection of bony abnormalities. To differentiate benign and malignant bony tumors, magnetic resonance imaging has a sensitivity and specificity of 44% and 95%, respectively. The corresponding percentages for single positron emission CT are 91% and 94%, for single positron emission CT/CT are 100% and 100%, for positron emission tomography are 88% and 72%, and for positron emission tomography/CT are 100% and 97%. The combination of morphologic and functional (single positron emission CT and positron emission tomography) modalities appears to improve the sensitivity and specificity to assess a hyperplastic condyle, facilitating treatment planning and providing a better prognosis for the patient.
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PMID:Imaging modalities to access bony tumors and hyperplasic reactions of the temporomandibular joint. 2045 15

Parosteal osteosarcoma and low-grade central osteosarcoma are two types of low-grade osteosarcoma that show similar clinical behaviors, histological features, and genetic background (ie, amplified sequences of 12q13-15, including MDM2 and CDK4). Low-grade osteosarcoma is often confused with benign lesions, and ancillary techniques to enhance diagnostic accuracy have been awaited. This study explores the use of MDM2 and CDK4 immunohistochemistry for the histological diagnosis of low-grade osteosarcoma. We studied 23 cases of low-grade osteosarcoma from 21 patients (parosteal osteosarcoma (n=14), low-grade central osteosarcoma (n=9)) and 40 cases of benign histological mimics (myositis ossificans (n=11), fibrous dysplasia (n=14), osteochondroma (n=6), desmoplastic fibroma (n=1), florid reactive periostitis (n=4), Nora's lesion (n=3), and turret exostosis (n=1)). Low-grade osteosarcoma labeled for MDM2 in 16 cases (70%) and for CDK4 in 20 cases (87%). All low-grade osteosarcomas expressed one or both markers (100%), with 13 cases (57%) expressing both. Staining pattern was diffuse in most cases, and the majority expressed moderate or strong intensity for either antibody. MDM2/CDK4 immunostaining was shown irrespective of low-grade osteosarcoma histological subtype. In contrast, only 1 Nora's lesion out of the 40 miscellaneous benign processes showed immunoreactivity for MDM2 or CDK4. The combination of these two markers thus shows 100% sensitivity and 97.5% specificity for the diagnosis of low-grade osteosarcoma. MDM2 and CDK4 immunostains therefore reliably distinguish low-grade osteosarcoma from benign histological mimics, and their combination may serve as a useful adjunct in this difficult differential diagnosis.
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PMID:Immunohistochemical analysis of MDM2 and CDK4 distinguishes low-grade osteosarcoma from benign mimics. 2060 38

Extraskeletal osteochondroma near the hip is rare and its pathological diagnosis is based on radiological and histopathological examination. It is vital that such a diagnosis be considered when a discrete, ossified mass is localized in soft tissues, even at atypical sites. Differential diagnoses include myositis ossificans, a lipomatous lesion, a pseudomalignant osseous tumour, an ossifying fibromyxoid tumour, an extraskeletal chondroma with endochondral ossification, synovial (osteo) chondromatosis, tumoural calcinosis, a synovial sarcoma and an extraskeletal osteosarcoma. Clinical awareness of this benign entity is important as no malignant transformation or metastasis has been reported. Marginal excision with histopathological identification is the treatment of choice.
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PMID:Extraskeletal osteochondroma near the hip: a pediatric case. 2080 44

We conducted a retrospective case-series review to identify the various diagnoses of neoplasms of the nasal cavity and paranasal sinuses in a pediatric population. Our study group was made up of 54 children-23 boys and 31 girls, aged 8 months to 16 years (mean: 9 yr). All patients had been diagnosed with a tumor of the nasal cavity or paranasal sinuses between Jan. 1, 1955, and Dec. 31, 1999, at one of four university-based, tertiary care referral centers. We compiled data on tumoral characteristics (location, size, and histopathology), morbidity and mortality, and rates of recurrence. Lesions included adnexal neoplasm, ameloblastic fibro-odontoma, basal cell carcinoma, benign fibrous histiocytoma, blue nevus, chondrosarcoma, compound nevus, epithelioma adenoides cysticum, esthesioneuroblastoma, Ewing sarcoma, fibrosarcoma, giant cell granuloma, granulocytic sarcoma, hemangioma, hemangiopericytoma, Langerhans cell histiocytosis, lymphangioma, lymphoma, melanoma, neuroblastoma, neurofibroma, ossifying osteofibroma, osteochondroma, osteosarcoma, port wine stain, rhabdomyosarcoma, Spitz nevus, and xanthogranuloma. To the best of our knowledge, this is the largest such study of its kind to date. We believe that the large size of this study and the data on disease incidence will allow clinicians to be better informed of the differential diagnosis of neoplasms of the nasal cavity and paranasal sinuses in the pediatric population.
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PMID:Differential diagnosis of pediatric tumors of the nasal cavity and paranasal sinuses: a 45-year multi-institutional review. 2108 77

Bizarre parosteal osteochondromatous proliferation (BPOP) also eponymically called "Nora's lesion", is a rare benign reactive bone lesion first reported in 1983. BPOP occurs classically on the bones of the hands and feet and long bones. This lesion can easily be confused, both clinically and microscopically, with other benign and malignant lesions of bone, including osteochondroma, parosteal osteosarcoma, myositis ossificans and reactive periostitis. BPOP has been reported to have a high rate of recurrence. Only 3 cases of BPOP of the head and neck have been reported in the literature, of which one involved the maxilla. We present a rare case of BPOP involving the mandible in a 10 year old African American male. Microscopically, a fibro-cartilaginous cap giving rise to a proliferation of variably mineralized osteophytic finger-like projections of bone was seen. Multiple trabeculae of "blue bone" were noted as well as numerous atypical appearing chondrocytes. The lesion recurred within 4 months following the initial excision but has not recurred to date after the second local excision. To the best of our knowledge, this is the first report of BPOP arising in the mandible. In addition, we discuss the clinical and microscopic features, differential diagnosis, and prognosis of this rare entity. We present a case of BPOP of the mandible and believe this is the first report of such a case in the mandible.
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PMID:Bizarre parosteal osteochondromatous proliferation (Nora's Lesion) of the mandible. a rare bony lesion. 2209 73

Veterinary medicine is most often a mysterious world for the human doctors. However, animals are important for human medicine thanks to the numerous biological similarities. Primary bone tumors are not uncommon in veterinary medicine and especially in small domestic animals as dogs and cats. As in human medicine, osteosarcoma is the most common one and especially in the long bones extremities. In the malignant bone tumor family, chondrosarcoma, fibrosarcoma and hemangiosarcoma are following. Benign bone tumors as osteoma, osteochondroma and bone cysts do exist but are rare and of little clinical significance. Diagnostic modalities used depend widely on the owner willing to treat his animal. Radiographs and bone biopsy are the standard to make a diagnosis but CT, nuclear medicine and MRI are more an more used. As amputation is treatment number one in appendicular bone tumor in veterinary medicine, this explains on the one hand why more recent imaging modalities are not always necessary and on the other hand, that prognostic on large animals is so poor that it is not much studied. Chemotherapy is sometimes associated with the surgery procedure, depending on the aggressivity of the tumor. Although, the strakes differs a lot between veterinary and human medicine, biological behavior are almost the same and should led to a beneficial team work between all.
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PMID:Imaging of primary bone tumors in veterinary medicine: which differences? 2219 93


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