An unusual case of Nerve sheath myxoma with schwannomatous and perineural differentiation
Keywords:
Benign tumour, Nerve sheath myxoma, S100 protein, EMA
Abstract
Nerve sheath myxoma (NSM) is a rare benign tumour of peripheral nerves. It is seen in middle aged adults with slight female predilection and presents as a slow growing painless mass. The commonest sites are extremities, scalp, back and neck. Microscopically it has a typical morphological appearance. It is multilobulated and is composed of spindle and stellate shaped cells set in an abundant myxoid stroma. The cells show strong positivity for S-100 protein and are EMA negative or it stains only few perineural cells, indicating its close relationship with schwannoma or neurofibroma. But we report a rare case of nerve sheath myxoma in a 40 year old woman which showed typical microscopic features of NSM. However it showed an unusual co-expression of S100 and EMA indicating a bidirectional schwannomatous-perineural differentiation. The clinicopathological features, various differential diagnosis, its histogenesis and brief review of literature are discussed below.References
1) Spadari F, Guzzi G, Bombeccari GP, Mariani U, Gianatti A, Ruffoni D et al. Nerve sheath myxoma of the tongue. Acta Dermato venerol Croat. 2014; 22(1):52-6.
2) Zamecnik M, Sedlacek T. Nerve sheath myxoma with bidirectional schwannomatous and perineural differentiation. Cesko-slovenskapatologie. 2010;46(3):73-6.
3) Reed RJ, Paulitzer DR. Tumors of neural tissue. In: Elder DE, Editor. Lever’s Histopathology of the skin. 10th ed. Philadelphia: Lippincott Williams & Williams; 2009, p 1126-27.
4) Fathaddin A, Fatani R. Palmar nerve sheath myxoma: A case report. Oman Med Jr. 2012;27(3):212-6.
5) Gehrke JC, Hamson KR, Havey AD. Dermal nerve sheath myxoma of the hallux: a case report. Foot Ankle Int. 1994; 15(12):666-8.
6) Malkoc M, Ormeci T, Keskinbora M, Yilmaz A, Korkmaz O, Tanik CB. Nerve sheath myxoma of the dorsal paravertebral space. Int J Surg Case Rep. 2014;5(11):858-60.
7) Bhat A, Narasimha A, CV, VK S. Nerve sheath myxoma: report of a rare case. Journal of clinical and diagnostic research. 2015;9(4):7-9.
8) Hamodat M. Nerve sheath myxoma presenting as finger nodule in 39 year old female. The internet journal of pathology. 2009:10(1).
9) Sheth S, Li X, Binder S, Dry SM. Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis. Mod Pathol. 2011;24(3):343-54.
10) Suh YL, Song KY, Kim JM. Nerve sheath myxoma (Neurothekeoma)- a case report. J Korean Med Sci.1992;7(1):85-9.
2) Zamecnik M, Sedlacek T. Nerve sheath myxoma with bidirectional schwannomatous and perineural differentiation. Cesko-slovenskapatologie. 2010;46(3):73-6.
3) Reed RJ, Paulitzer DR. Tumors of neural tissue. In: Elder DE, Editor. Lever’s Histopathology of the skin. 10th ed. Philadelphia: Lippincott Williams & Williams; 2009, p 1126-27.
4) Fathaddin A, Fatani R. Palmar nerve sheath myxoma: A case report. Oman Med Jr. 2012;27(3):212-6.
5) Gehrke JC, Hamson KR, Havey AD. Dermal nerve sheath myxoma of the hallux: a case report. Foot Ankle Int. 1994; 15(12):666-8.
6) Malkoc M, Ormeci T, Keskinbora M, Yilmaz A, Korkmaz O, Tanik CB. Nerve sheath myxoma of the dorsal paravertebral space. Int J Surg Case Rep. 2014;5(11):858-60.
7) Bhat A, Narasimha A, CV, VK S. Nerve sheath myxoma: report of a rare case. Journal of clinical and diagnostic research. 2015;9(4):7-9.
8) Hamodat M. Nerve sheath myxoma presenting as finger nodule in 39 year old female. The internet journal of pathology. 2009:10(1).
9) Sheth S, Li X, Binder S, Dry SM. Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis. Mod Pathol. 2011;24(3):343-54.
10) Suh YL, Song KY, Kim JM. Nerve sheath myxoma (Neurothekeoma)- a case report. J Korean Med Sci.1992;7(1):85-9.
Published
2017-01-03
Section
Case Report
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