title: Giant malignant nerve sheath tumor of lumbosacral plexus with intraspinal extension in a child with neurofibromatosis type 1.
abstract: Malignant peripheral nerve sheath tumors (MPNSTs) are the leading cause of death in young adults and are one of the most frequent non-rhabdomyosarcomatous soft tissue tumors in pediatric age. These tumors usually occur in young adults from a previously recognized neurofibroma, neurofibromatosis type 1 (NF1), with a noted change in size and pain. This child reached the age of 10 without the presence of the more commonly seen manifestations of NF1. Pseudoarthrosis in children has a high rate of association with NF1, and in this case diagnosis of NF1 was supported by development of MPNST in a pre-existing plexiform neurofibroma.
authors.affiliation: Department of Surgery, B.P. Koirala Institute of Health Sciences, Dharan, Nepal.
authors.forename: Akshay
authors.initials: A
authors.lastname: Pratap
authors.forename: Pravin
authors.initials: P
authors.lastname: Nepal
authors.forename: Amit
authors.initials: A
authors.lastname: Agrawal
authors.forename: Mahi Pal
authors.initials: MP
authors.lastname: Singh
authors.forename: Sagar Raj
authors.initials: SR
authors.lastname: Pandey
mesh_terms: D002648:Child; D006801:Humans; D008160:Lumbosacral Plexus / Q000473:pathology* / Q000601:surgery; D008297:Male; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000601:surgery