title: Malignant peripheral nerve sheath tumor: pathology and genetics.
abstract: Malignant peripheral nerve sheath tumors are soft tissue neoplasms that show differentiation toward cells of the nerve sheath. They often arise from peripheral nerves or preexisting benign nerve sheath tumors and are generally high-grade neoplasms, which behave aggressively with high incidence of distant metastases. Malignant peripheral nerve sheath tumor can be histologically diverse and is difficult to diagnose because of its morphological overlap with a variety of other sarcomas and its lack of specific immunohistochemical markers or genetic profile. We review the pathology of malignant peripheral nerve sheath tumor, with reference to etiology, molecular genetics, and clinical factors.
authors.affiliation: Department of Histopathology, Sarcoma Unit, Royal Marsden NHS Foundation Trust, 203 Fulham Road, London SW3 6JJ, UK. Electronic address: khin.thway@rmh.nhs.uk.
authors.forename: Khin
authors.initials: K
authors.lastname: Thway
authors.affiliation: Department of Histopathology, Sarcoma Unit, Royal Marsden NHS Foundation Trust, 203 Fulham Road, London SW3 6JJ, UK.
authors.forename: Cyril
authors.initials: C
authors.lastname: Fisher
mesh_terms: D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism*; D003937:Diagnosis, Differential; D006801:Humans; D018317:Nerve Sheath Neoplasms / Q000235:genetics / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000235:genetics / Q000473:pathology*; D018319:Neurofibrosarcoma / Q000235:genetics / Q000473:pathology*; D010524:Peripheral Nervous System Neoplasms / Q000235:genetics / Q000473:pathology*; D012509:Sarcoma / Q000235:genetics / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000235:genetics / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor
keywords: Malignant peripheral nerve sheath tumor; Neurofibromatosis type 1; Neurofibrosarcoma; Sarcoma; Triton tumor