Book chapter
Malignant Tumors of Peripheral Nerves
Pediatric Malignancies: Pathology and Imaging, pp.399-414
Springer New York
10/28/2014
DOI: 10.1007/978-1-4939-1729-7_15
Abstract
Malignant peripheral nerve sheath tumors (MPNST) are biologically aggressive spindle cell sarcomas with evidence of nerve sheath differentiation and/or arising from a peripheral nerve or neurofibroma. They show a marked tendency for local recurrence and metastasis. Approximately one half arise within patients with neurofibromatosis 1 (NF1), whereas the remainder is sporadic. Radioimaging studies are unreliable in differentiating MPNST from other sarcomas or from benign nerve sheath tumors. From the microscopic standpoint, the majority of MPNSTs are hypercellular, high grade spindle cell sarcomas with plentiful mitoses, often with some degree of nuclear pleomorphism and/or necrosis, and variable positivity for S100, SOX10, EMA, CD34, Nestin, GFAP, collagen IV, and p53 by immunohistochemistry. Approximately 15 % of all MPNSTs will exhibit some form of divergent differentiation, malignant triton tumor being the most frequent of these variants. Surgical excision with adequate margins is the mainstay of therapy.
Details
- Title: Subtitle
- Malignant Tumors of Peripheral Nerves
- Creators
- Simon Ching-Shun Kao - University of Iowa Health CareDavid M. Parham - Children's Hospital of Los AngelesChristine Fuller - Virginia Commonwealth University
- Resource Type
- Book chapter
- Publication Details
- Pediatric Malignancies: Pathology and Imaging, pp.399-414
- DOI
- 10.1007/978-1-4939-1729-7_15
- Publisher
- Springer New York; New York, NY
- Language
- English
- Date published
- 10/28/2014
- Academic Unit
- Radiology; Stead Family Department of Pediatrics
- Record Identifier
- 9984318809602771
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