Giant malignant nerve sheath tumor of lumbosacral plexus with intraspinal extension in a child with neurofibromatosis type 1.
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.
Pratap
Akshay
A
Department of Surgery, B.P. Koirala Institute of Health Sciences, Dharan, Nepal.
Nepal
Pravin
P
Agrawal
Amit
A
Singh
Mahi Pal
MP
Pandey
Sagar Raj
SR
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