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Comparative Pathology of Nerve Sheath Tumors in Mouse Models and Humans

¹ Departments of Pathology and ² Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts; ³ Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York; ⁴ Medical Pathology Center for Comparative Medicine, University of California at Davis, Davis, California; ⁵ Department of Pathology, Harvard Medical School, Boston, Massachusetts; ⁶ Department of Pathology and ⁷ Center for Developmental Biology, University of Texas Southwestern Medical Center, Dallas, Texas; ⁸ INSERM U434, Fondation Jean Dausset-CEPH, Paris, France; ⁹ Department of Pathology, Brigham and Women’s Hospital, Boston, Massachusetts; ¹⁰ Department of Neuropathology, Heinrich-Heine-University, Duesseldorf, Germany; ¹¹ Division of Marine Biology and Fisheries, Rosenstiel School of Marine and Atmospheric Science, University of Miami, Miami, Florida; ¹² Department of Medicine, Veterans’ Affairs Medical Center and University of California at San Francisco, San Francisco, California; ¹³ Genetics Division, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts; ¹⁴ Massachusetts General Hospital Cancer Center, Harvard Medical School Department of Pathology, Charlestown, Massachusetts; ¹⁵ Department of Cell Biology, Neurobiology, and Anatomy, University of Cincinnati College of Medicine, Cincinnati, Ohio; ¹⁶ Genetic Modifiers of Tumorigenesis Section, Mouse Cancer Genetics Program, National Cancer Institute at Frederick, Frederick, Maryland; and ¹⁷ Division of Molecular Medicine and Genetics, University of Michigan Medical School, Ann Arbor, Michigan

ABSTRACT

Despite the progress made in our understanding of the biology of neurofibromatosis (NF), the long-term clinical outcome for affected patients has not changed significantly in the past decades, and both NF1 and NF2 are still associated with a significant morbidity and a decreased life span. A number of NF1 and NF2 murine models have been generated to aid in the study of NF tumor biology and in the development of targeted therapies for NF patients. A single, universal pathological classification of the lesions generated in these murine models is essential for the validation of the models, for their analysis and comparison with other models, and for their future effective use in preclinical treatment trials. For the formulation of a pathological classification of these lesions, the WHO classification of human tumors was used as a reference. However, it was not adopted for the classification of the GEM lesions because of some important differences between the human and murine lesions. A novel classification scheme for peripheral nerve sheath tumors in murine models was therefore devised.

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