title: Spinal cord metastasis of a non-neurofibromatosis type-1 malignant peripheral nerve sheath tumor: an unusual manifestation of a rare tumor.
abstract: Malignant peripheral nerve sheath tumors are rare spindle-cell sarcomas derived from Schwann cells or pluripotent cells of the neural crest. They arise from the spinal roots, peripheral nerves, brachial and lumbosacral plexi, cranial nerves and terminal nerve fibers within soft tissue, intestine, lung and bone. These tumors recur either locally, or metastasize distally. Most of these tumors occur in association with neurofibromatosis type 1. Spinal cord metastasis from malignant nerve sheath tumors associated with neurofibromatosis type 1 is very rare. We describe a rare case of near-total spinal cord metastasis in a patient with malignant nerve sheath tumor in the absence of neurofibromatosis, and highlight the microscopic findings and natural history of this disease process.
authors.affiliation: The Department of Neurology, The University of Chicago School of Medicine and Medical Center, 5841 S. Maryland Street, Chicago, IL 60637, USA.
authors.forename: William S
authors.initials: WS
authors.lastname: Baek
authors.forename: Peter
authors.initials: P
authors.lastname: Pytel
authors.forename: Samir D
authors.initials: SD
authors.lastname: Undevia
authors.forename: Helene
authors.initials: H
authors.lastname: Rubeiz
mesh_terms: D000328:Adult; D001557:Benzenesulfonates / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary* / Q000628:therapy; D008279:Magnetic Resonance Imaging; D018317:Nerve Sheath Neoplasms / Q000473:pathology* / Q000601:surgery; D009455:Neurofibroma / Q000473:pathology* / Q000628:therapy; D009536:Niacinamide / Q000031:analogs & derivatives; D010671:Phenylurea Compounds; D011725:Pyridines / Q000627:therapeutic use; D000077157:Sorafenib; D013120:Spinal Cord Neoplasms / Q000556:secondary* / Q000628:therapy
chemical_list: D001557:Benzenesulfonates; D010671:Phenylurea Compounds; D011725:Pyridines; D009536:Niacinamide; D000077157:Sorafenib