Giant malignant nerve sheath tumor of lumbosacral plexus with

Pediatric neurosurgery · Jan 2007

Case Reports

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.

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- Akshay Pratap, Pravin Nepal, Amit Agrawal, Mahi Pal Singh, and Sagar Raj Pandey.
- Department of Surgery, B.P. Koirala Institute of Health Sciences, Dharan, Nepal.
- Pediatr Neurosurg. 2007 Jan 1; 43 (5): 410-3.
AbstractMalignant 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.(c) 2007 S. Karger AG, Basel.

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Giant malignant nerve sheath tumor of lumbosacral plexus with intraspinal extension in a child with neurofibromatosis type 1.

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