Section 24. Neoplastic Disorders >
Part 1. Principles of Oncology >
Chapter 443. Cancer Genetics and BiologyDavid Malkin
Topics Discussed: cancer genetics; childhood cancer; genetics and dysmorphology; medical oncology; oncogenes; tumor suppressor genes.
Sections: The Retinoblastoma Susceptibility Gene (RB1): Paradigm Tumor Suppressor, Wilms Tumor: A Complex Tumor Suppressor Model, Tumors of the Peripheral Nervous System: Neuroblastoma, Cytogenetic Translocationsthe Role of Fusion Proteins in Ewing Sarcoma and Primitive Neuroectodermal Tumors, Cytogenetic Translocations or Chromosomal Loss in Rhabdomyosarcoma, Osteosarcoma: Complex Genetic Mechanisms, Cancer Predisposition Syndromes, Molecular and Clinical Surveillance for Cancer Predisposition, References.
Excerpt:"Cancer is the most common cause of disease-related death in children beyond
the newborn period. The unique biological features, the cell of
origin, and the response to therapy of childhood cancers make them
intriguing models with which to study and understand the process
of human carcinogenesis. Tumors of childhood commonly reflect the
embryonic precursor of the cell types in which they arise. The majority
occur sporadically, and their etiology remains unclear. Obvious
environmental influences to cancer initiation are not generally
apparent. Some cancers, such as neuroblastoma, appear to arise even
before birth, whereas a significant number of others present with
a striking family history of cancer or coincide with congenital abnormalities,
suggesting an important role for inherited causal genetic factors. Disorders
such as xeroderma pigmentosum,1 which places children at
increased risk for developing skin cancers, or Beckwith-Wiedemann
syndrome,2 which is commonly complicated by the development
of embryonal tumors, including Wilms tumor of the kidney, rhabdomyosarcoma,
or hepatoblastoma, are examples of inherited disorders that confer
an increased risk developing cancer. Cancer predisposition syndromes in
which nonmalignant phenotypic features are not observed include hereditary
retinoblastoma,3 Li-Fraumeni syndrome,4 and familial
polyposis,5 while others such as von Hippel Lindau disease6 are
associated with the coincident presentation of both benign and..."
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