title: Comparative pathology of nerve sheath tumors in mouse models and humans.
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.
authors.affiliation: Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Anat O
authors.initials: AO
authors.lastname: Stemmer-Rachamimov
authors.forename: David N
authors.initials: DN
authors.lastname: Louis
authors.forename: Gunnlaugur P
authors.initials: GP
authors.lastname: Nielsen
authors.forename: Cristina R
authors.initials: CR
authors.lastname: Antonescu
authors.forename: Alexander D
authors.initials: AD
authors.lastname: Borowsky
authors.forename: Roderick T
authors.initials: RT
authors.lastname: Bronson
authors.forename: Dennis K
authors.initials: DK
authors.lastname: Burns
authors.forename: Pascale
authors.initials: P
authors.lastname: Cervera
authors.forename: Margaret E
authors.initials: ME
authors.lastname: McLaughlin
authors.forename: Guido
authors.initials: G
authors.lastname: Reifenberger
authors.forename: Michael C
authors.initials: MC
authors.lastname: Schmale
authors.forename: Mia
authors.initials: M
authors.lastname: MacCollin
authors.forename: Richard C
authors.initials: RC
authors.lastname: Chao
authors.forename: Karen
authors.initials: K
authors.lastname: Cichowski
authors.forename: Michel
authors.initials: M
authors.lastname: Kalamarides
authors.forename: Shanta M
authors.initials: SM
authors.lastname: Messerli
authors.forename: Andrea I
authors.initials: AI
authors.lastname: McClatchey
authors.forename: Michiko
authors.initials: M
authors.lastname: Niwa-Kawakita
authors.forename: Nancy
authors.initials: N
authors.lastname: Ratner
authors.forename: Karlyne M
authors.initials: KM
authors.lastname: Reilly
authors.forename: Yuan
authors.initials: Y
authors.lastname: Zhu
authors.forename: Marco
authors.initials: M
authors.lastname: Giovannini
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D018317:Nerve Sheath Neoplasms / Q000145:classification / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000145:classification / Q000473:pathology; D016518:Neurofibromatosis 2 / Q000145:classification / Q000473:pathology