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Sarcomas

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Molecular Pathology in Clinical Practice

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Abstract

Oncologic molecular pathology focuses on identifying and understanding molecular and genetic alterations underlying the development and progression of neoplastic processes. Mesenchymal malignancies may be classified into two pathogenetic types: sarcomas with complex genetic alterations and sarcomas with specific recurrent chromosomal translocations. The first type includes the majority of high-grade, pleomorphic mesenchymal malignancies that are characterized by complex chromosomal abnormalities, for example, malignant fibrous histiocytoma (MFH), osteogenic sarcoma, and embryonal rhabdomyosarcoma. In the second type, the sarcomas are translocation specific, that is, harboring a recurrent chromosomal translocation leading to an in-frame fusion of coding sequences from each of the two rearranged genes. The translocation results in the production of a chimeric transcript encoding a fusion protein with oncogenic activity. Histologically, the translocation-specific sarcomas are generally a monomorphic proliferation of neoplastic cells. This pathogenetic classification appears biologically relevant and is best illustrated by the sarcoma types observed in Li-Fraumeni syndrome and therapy-related malignancies.

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Author information

Authors and Affiliations

  1. Department of Pathology and Attending Pathologist, Bologna University School of Medicine, Bologna

    Giovanni Tallini MD (Professor and Director)

  2. Diagnostic Molecular Pathology Laboratory, Ospedale Bellaria, Bologna, Italy

    Giovanni Tallini MD (Professor and Director)

  3. Molecular Diagnostics Laboratory, Department of Pathology, Yale University Schnool of Medicine, New Haven, CT, 06520, USA

    Pei Hui MD, PhD (Assistant Professor and Attending Pathologist, Medical Director)

Authors
  1. Giovanni Tallini MD
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  2. Pei Hui MD, PhD
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Editor information

Editors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University and New York Presbyterian Hospital, New York, NY, 10021, USA

    Debra G. B. Leonard MD, PhD (Professor and Vice Chair for Laboratory Medicine) (Professor and Vice Chair for Laboratory Medicine)

  2. Hematology, Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA

    Adam Bagg MD (Director) (Director)

  3. Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, GA, 30322, USA

    Angela M. Caliendo MD, PhD (Vice Chair and Associate Professor) (Vice Chair and Associate Professor)

  4. Northwestern University Feinberg School of Medicine, Evanston, IL, 60201

    Karen L. Kaul MD, PhD (Professor) (Professor)

  5. Department of Pathology and Laboratory Medicine, Evanston Northwestern Healthcare, Evanston, IL, 60201, USA

    Karen L. Kaul MD, PhD (Professor) (Professor)

  6. Molecular Pathology Laboratory, Department of Pathology and Laboratory Medicine, University of Pennsylvania Health System, Philadelphia, PA, 19104, USA

    Vivianna M. Van Deerlin MD, PhD (Director) (Director)

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Tallini, G., Hui, P. (2007). Sarcomas. In: Leonard, D.G.B., Bagg, A., Caliendo, A.M., Kaul, K.L., Van Deerlin, V.M. (eds) Molecular Pathology in Clinical Practice. Springer, New York, NY. https://doi.org/10.1007/978-0-387-33227-7_26

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  • DOI: https://doi.org/10.1007/978-0-387-33227-7_26

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-33226-0

  • Online ISBN: 978-0-387-33227-7

  • eBook Packages: MedicineMedicine (R0)

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