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Chap 24 - NERVE SHEATH TUMORS

Published online by Cambridge University Press:  01 March 2011

By
Markku Miettinen
Edited by
Markku Miettinen
Markku Miettinen
Affiliation:
Armed Forces Institute of Pathology, Washington DC
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Summary

The benign nerve sheath tumors discussed in this chapter include variants of neurofibroma, schwannoma, and granular cell tumors, and the rare perineurial cell tumors (perineuriomas) and nerve sheath myxomas. Malignant peripheral nerve sheath tumor (MPNST) is the term for malignant tumors arising in a neurofibroma. Nerve sheath tumors occur in both peripheral and cranial nerves. Their classification is discussed following the principles in the Armed Forces Institute of Pathology (AFIP) atlas of tumors of the peripheral nervous system.

Alterations in the neurofibromatosis type 1 (NF1) gene leading to the absence or loss of function of the NF1 protein (neurofibromin) are the basis of the neurofibromatosis 1 syndrome and associated neurofibromas. There is also evidence for somatic NF1 gene mutations' being responsible for sporadic neurofibromas. Analogous to this, changes in the neurofibromatosis type 2 (NF2) gene that lead to the loss of function or absence of NF2 protein (merlin) are the pathogenesis of the NF2 syndrome. This syndrome entails bilateral vestibular schwannomas, meningiomas, and certain gliomas. Corresponding somatic genetic changes are thought to be behind the pathogenesis of sporadic schwannomas.

True neural tumors, such paragangliomas and related tumors, and neuroectodermal tumors, such as malignant melanoma and clear cell sarcoma, are discussed in Chapters 25 and 26. Gastrointestinal autonomic nerve tumor (GANT) is now classified among the gastrointestinal stromal tumors (GISTs) as the ultrastructural variant of a GIST (Chapter 17). Although GANTs and GISTs are not nerve sheath tumors, they have a weak association with NF1 syndrome.

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Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
 , pp. 660 - 723
Publisher: Cambridge University Press
Print publication year: 2010

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