Abstract
Nerve sheath myxoma, a myxoid variant of schwannoma, is a dermal tumor that usually occurs in the upper extremities, head and neck region, or trunk; occasionally, however, it has also been reported to develop in the spinal canal. Here, we describe two cases of intraspinal nerve sheath myxoma. Case 1 was a 74-year-old man with left hypochondrial pain. Gadolinium-enhanced magnetic resonance imaging (MRI) of his spine revealed a well-demarcated intradural extramedullary tumor with peripheral enhancement at the Th8 level. Case 2 was a 58-year-old man with lower back and left buttock pain. Gadolinium-enhanced MRI revealed a well-demarcated intradural extramedullary tumor with peripheral enhancement at the Th12-L1 level. Both cases were clinically diagnosed as schwannoma. Histological studies revealed characteristic myxoid lobules which were separated by fibrous septa or bands of more compact cellular area. The tumor cells were diffusely positive for S-100 and focally positive for Schwann/2E, which reacts with Schwann cells and myelin in the peripheral nervous system. The positive reaction to Schwann/2E confirmed the occurrence of peripheral nerve sheath differentiation. Nerve sheath myxoma should be included in differential diagnosis of spinal canal tumors.
This is a preview of subscription content, log in via an institution to check access.
References
Chamberlain MC, Tredway TL (2011) Adult primary intradural spinal cord tumors: a review. Curr Neurol Neurosci Rep 11:320–328
Yamaguchi H (1980) Studies on the immunohistochemical localization of S-100 and glial fibrillary acidic proteins in the rat nervous system and in human brain tumors. No To Shinkei (in Japanese) 32:1055–1064
Arai H, Hirato J, Nakazato Y (1998) A novel marker of Schwann cells and myelin of the peripheral nervous system. Pathol Int 48:206–214
Nakazato Y, Ishizeki J, Takahashi K, Yamaguchi H, Kamei T, Mori T (1982) Localization of S-100 protein and glial fibrillary acidic protein-related antigen in pleomorphic adenoma of the salivary glands. Lab Invest 46:621–626
Harkin JC, Reed RJ (1969) Tumors of the peripheral nervous system. In: Rosai J (ed) Atlas of tumor pathology, vol. 2nd series Fascicle 3. Armed Forces Institute of Pathology, Washington DC, pp 29–65
Fetsch JF, Laskin WB, Miettinen M (2005) Nerve sheath myxoma: a clinicopathologic and immunohistochemical analysis of 57 morphologically distinctive, S-100 protein- and GFAP-positive, myxoid peripheral nerve sheath tumors with a predilection for the extremities and a high local recurrence rate. Am J Surg Pathol 29:1615–1624
Gallager RL, Helwig EB (1980) Neurothekeoma—a benign cutaneous tumor of neural origin. Am J Clin Pathol 74:759–764
Sheth S, Li X, Binder S, Dry SM (2011) Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis. Mod Pathol 24:343–354
Lee D, Suh YL, Han J, Kim ES (2006) Spinal nerve sheath myxoma (neurothekeoma). Pathol Int 56:144–149
Kaar GF, Bashir SH, N’Dow JM, Best PV, Gomersall LN (1996) Neurothekeoma of the cauda equina. J Neurol Neurosurg Psychiatry 61:530–531
Paulus W, Jellinger K, Perneczky G (1991) Intraspinal neurothekeoma (nerve sheath myxoma). A report of two cases. Am J Clin Pathol 95:511–516
Rickert CH, Schwering EM, Siebers J, Hartmann C, von Deimling A, Paulus W (2007) Chromosomal imbalances and NF2 mutational analysis in a series of 10 spinal nerve sheath myxomas. Histopathology 50:252–257
Abul-Kasim K, Thurnher MM, McKeever P, Sundgren PC (2008) Intradural spinal tumors: current classification and MRI features. Neuroradiology 50:301–314
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Yamato, M., Ikota, H., Hanakita, J. et al. Intradural extramedullary spinal nerve sheath myxoma: a report of two cases. Brain Tumor Pathol 31, 57–61 (2014). https://doi.org/10.1007/s10014-012-0131-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10014-012-0131-z