6,587 research outputs found

    Epidemiology and etiology of osteosarcoma : a compendium of notes abstracted from the literature

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    Osteosarcoma derives from primitive bone-forming mesenchymal cells and is the most common primary bone malignancy. According to the most recent report from the U.S. Cancer Statistics Working Group, the incidence rates and 95% confidence intervals of osteosarcoma for all races and both sexes are 4.0 (3.5-4.6) for the range 0-14 years and 5.0 (4.6-5.6) for the range 0-19 years per year per million persons. Among childhood cancers, osteosarcoma occurs eighth in general incidence and in the following order: leukemia (30%), brain and other nervous system cancers (22.3%), neuroblastoma (7.3%), Wilms tumor (5.6%), Non-Hodgkin lymphoma (4.5%), rhabdomyosarcoma (3.1%), retinoblastoma (2.8%), osteosarcoma (2.4%), Ewing sarcoma (1.4%). The incidence rates of childhood and adolescent osteosarcoma with 95% confidence intervals are: Blacks 6.8 / year /million, Hispanics 6.5 / year / million and Caucasians 4.6 / year /million. Osteosarcoma has a bimodal age distribution, having the first peak during adolescence and the second peak in older adults. The first peak is in the 10 to 20-year-old age group, coinciding with the pubertal growth spurt. This suggests a close relationship between the adolescent growth spurt and osteosarcoma. Osteosarcoma patients whose disease is diagnosed during their growth spurt are generally taller than average, while patients identified in adulthood have average height. The second osteosarcoma peak is in adults older than 65 years of age in which osteosarcoma is more likely to represent a second malignancy, frequently related to Paget’s disease. Approximately 1% of patients with Paget’s disease develop osteosarcoma. Osteosarcoma commonly occurs in the long bones of the extremities near the metaphyseal growth plates. The most common sites are femur (42%, with 75% of tumors in the distal femur), tibia (19%, with 80% of tumors in the proximal tibia), and humerus (10%, with 90% of tumors in the proximal humerus). Other locations of note are the skull or jaw (8%) and pelvis. The overall 5-year survival rate for osteosarcoma is 68%., without significant gender difference. Age of the patient is correlated with survival with the poorest survival for older patients. Complete surgical excision is important to ensure an optimum outcome. Tumor staging, presence of metastases, local recurrence, chemotherapy regimen, anatomic location, size of the tumor, and percentage of tumor cells destroyed after neoadjuvant chemotherapy have variable effects on outcome. The etiology of osteosarcoma is still largely unknown, but there may be a relationship to growth, since it coincides with a period of rapid bone growth in young people. 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. 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 to develop 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

    The etiology of osteosarcoma

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    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 in most of these reports was not available. 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 to develop 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 is apparently independent of the administration of radiotherapy

    The epidemiology of osteosarcoma

    No full text
    Osteosarcoma derives from primitive bone-forming mesenchymal cells and is the most common primary bone malignancy. The incidence rates and 95% confidence intervals of osteosarcoma for all races and both sexes are 4.0 (3.5-4.6) for the range 0-14 years and 5.0 (4.6-5.6) for the range 0-19 years per year per million persons. Among childhood cancers, osteosarcoma occurs eighth in general incidence and in the following order: leukemia (30%), brain and other nervous system cancers (22.3%), neuroblastoma (7.3%), Wilms tumor (5.6%), Non-Hodgkin lymphoma (4.5%), rhabdomyosarcoma (3.1%), retinoblastoma (2.8%), osteosarcoma (2.4%), Ewing sarcoma (1.4%). The incidence rates of childhood and adolescent osteosarcoma with 95% confidence intervals are: Blacks 6.8 / year /million, Hispanics 6.5 / year / million and Caucasians 4.6 / year /million. Osteosarcoma has a bimodal age distribution, having the first peak during adolescence and the second peak in older adults. The first peak is in the 10 to 14-year-old age group, coinciding with the pubertal growth spurt. This suggests a close relationship between the adolescent growth spurt and osteosarcoma. The second osteosarcoma peak is in adults older than 65 years of age in which osteosarcoma is more likely to represent a second malignancy, frequently related to Paget’s disease. The incidence of osteosarcoma has always been considered to be higher in males than in females, at a rate of 5.4 per million persons per year in males versus 4.0 per million in females, with a higher incidence of osteosarcoma in blacks (6.8 per million persons per year) and in Hispanics (6.5 per million) than in whites (4.6 per million). Osteosarcoma commonly occurs in the long bones of the extremities near the metaphyseal growth plates. The most common sites are femur (42%, with 75% of tumors in the distal femur), tibia (19%, with 80% of tumors in the proximal tibia), and humerus (10%, with 90% of tumors in the proximal humerus). Other locations of note are the skull or jaw (8%) and pelvis (8%). Cancer deaths due to bone and joint malignant neoplasms represent 8.9 percent of all childhood and adolescent cancer deaths. Death rates for osteosarcoma have been declining by about 1.3 percent per year. The overall 5-year survival rate for osteosarcoma is 68%, without significant gender difference. Age of the patient is correlated with survival with the poorest survival for older patients. Complete surgical excision is important to ensure an optimum outcome. Tumor staging, presence of metastases, local recurrence, chemotherapy regimen, anatomic location, size of the tumor, and percentage of tumor cells destroyed after neoadjuvant chemotherapy have effects on outcome

    Clinical and pathologic study of two siblings with osteosarcoma

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    Familial occurrence of telangiectatic osteosarcoma is rare. A sister and a brother developed telangectatic osteosarcoma at the age of 11 and 12 respectively. The tumors occurred in the metaphysis and epiphysis of the distal left femour and in the 9th left rib respectively. In their family there was no previous bone cancer or retinoblastoma. Although the local behavior was very aggressive, the clinical courses were relatively indolent in both cases, thanks to the high chemo-sensitive of the telangectatic osteosarcoma. The histologic features were similar, showing anaplastic tumor cells producing osteoid and proliferation of blood cavities. Such malignancies within a family suggests the presence of a genetic factor. We discuss possible explanations for familial aggregation of osteosarcoma, citing genetic factors

    Henri Temianka Correspondence; (jaffe)

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    This collection contains material pertaining to the life, career, and activities of Henri Temianka, violin virtuoso, conductor, music teacher, and author. Materials include correspondence, concert programs and flyers, music scores, photographs, and books.https://digitalcommons.chapman.edu/temianka_correspondence/3789/thumbnail.jp

    Market Value and Patent Citations: A First Look

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    As patent data become more available in machine-readable form, an increasing number of researchers have begun to use measures based on patents and their citations as indicators of technological output and information flow. This paper explores the economic meaning of these citation-based patent measures using the financial market valuation of the firms that own the patents. Using a new and comprehensive dataset containing over 4800 U. S. Manufacturing firms and their patenting activity for the past 30 years, we explore the contributions of R&D spending, patents, and citation-weighted patents to measures of Tobin's Q for the firms. We find that citation-weighted patent stocks are more highly correlated with market value than patent stocks themselves and that this fact is due mainly to the high valuation placed on firms that hold very highly cited patents.

    Shalom N. Jaffe SPSP 2022

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    Shalom N. Jaffe SPSP 2022

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    Jaffe A. J., Froomkin Joseph — Technology and Jobs

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    Daric Jean. Jaffe A. J., Froomkin Joseph — Technology and Jobs. In: Population, 25ᵉ année, n°1, 1970. pp. 216-217
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