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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
Published on: May 17, 2024
Paraneoplastic syndromes affecting the nervous system
Robert B Darnell1, Jerome B Posner
1Howard Hughes Medical Institute and Laboratory of Molecular Neuro-Oncology, The Rockefeller University, New York, NY 10021, USA. darnelr@rockefeller.edu
Abstract:
The paraneoplastic neurologic disorders (PND) are a diverse group of diseases characterized by the presence of neurologic dysfunction in the setting of a remote cancer. PND can affect almost any part of the nervous system, and are most commonly associated with lung cancer (small cell) and gynecologic tumors. Laboratory studies have demonstrated that an autoimmune response links the neurologic disorder and the cancer, and established a model whereby the cancer is believed to initiate the syndrome by expressing a protein antigen normally expressed in the nervous system, leading to anti-tumor immune response followed by autoimmune neurologic symptoms. We review the currently known PND and their pathogenesis.
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