Abstract
Malignant neoplasms arising in association with peripheral nerves and resembling elements of the peripheral nerve are considered in this category. These tumors may arise in the setting of neurofibromatosis or may be seen sporadically in children. The unequivocal recognition of a high grade sarcoma as malignant peripheral nerve sheath tumor (MPNST) requires that the tumor clearly arises from a peripheral nerve, emerge from a benign peripheral nerve sheath tumor such as neurofibroma or schwannoma, or demonstrate histological, immunohistochemical, and ultrastructural evidence of peripheral nerve tissue differentiation.
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Meis JM, Enzinger FM, Martz KL, Neal JA (1992) Malignant peripheral nerve sheath tumors (malignant schwannomas) in children. Am J Surg Pathol 16(7):694–707
Casanova M, Ferrari A, Spreafico F et al (1999) Malignant peripheral nerve sheath tumors in children: A single-institution twenty-year experience. J Pediatr Hematol Oncol 21(6):509–513
Mautner VF, Friedrich RE, von Deimling A et al (2003) Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma. Neuroradiology 45(9):618–625
Carli M, Ferrari A, Mattke A et al (2005) Pediatric malignant peripheral nerve sheath tumor: The Italian and German soft tissue sarcoma cooperative group. J Clin Oncol 23(33):8422–8430
Friedrich RE, Hartmann M, Mautner VF (2007) Malignant peripheral nerve sheath tumors (MPNST) in NF1-affected children. Anticancer Res 27(4A):1957–1960
Harrisingh MC, Lloyd AC (2004) Ras/Raf/ERK signaling and NF1. Cell Cycle 3(10):1255–1258
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Tihan, T. (2010). Malignant Peripheral Nerve Sheath Tumors. In: Adesina, A., Tihan, T., Fuller, C., Poussaint, T. (eds) Atlas of Pediatric Brain Tumors. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-1062-2_15
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DOI: https://doi.org/10.1007/978-1-4419-1062-2_15
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