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Table of Contents
Year : 2019  |  Volume : 2  |  Issue : 2  |  Page : 137-138

Cerebellopontine angle neurothekeoma (nerve sheath myxoma): An overview

Department of Neurology; Department of Medicine, Federal University of Santa Maria, Santa Maria, Rio Grande do Sul, Brazil

Date of Web Publication10-Jan-2020

Correspondence Address:
Mr. Jamir Pitton Rissardo
Rua Roraima, Santa Maria, Rio Grande do Sul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJNO.IJNO_13_19

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How to cite this article:
Rissardo JP, Fornari Caprara AL. Cerebellopontine angle neurothekeoma (nerve sheath myxoma): An overview. Int J Neurooncol 2019;2:137-8

How to cite this URL:
Rissardo JP, Fornari Caprara AL. Cerebellopontine angle neurothekeoma (nerve sheath myxoma): An overview. Int J Neurooncol [serial online] 2019 [cited 2022 Jun 27];2:137-8. Available from: https://www.Internationaljneurooncology.com/text.asp?2019/2/2/137/275530

Dear Editor,

We read the article on “International Journal of Neurooncology” with great interest. Multani et al. reported a case of an adult female who presented with a right cerebellopontine angle (CPA) syndrome. Neuroimaging was suggestive of CPA epidermoid, with the possibility of rosette-forming glioneuronal tumor. On immunohistochemistry, a possible diagnosis of benign nerve sheath myxoma (neurothekeoma) was made.[1]

Neurothekeomas are benign tumors of a likely nerve sheath origin. They were first characterized by Gallager and Helwig in 1980. In this context, other titles to this rare entity were already proposed such as “cutaneous lobular neuromyxoma,” “pacinian neurofibroma,” “perineurial myxoma,” and “plexiform myxoma.”[2]

Only a few cases of patients with CPA neurothekeoma have been reported in the literature. To better evaluate this presentation, we performed a literature search, identified one case after a thorough review of the English-language published literature, and compared it with the Multani et al. report [Table 1].[1],[3] The literature search was performed in Embase, Google Scholar, Lilacs, Medline, Scielo, and ScienceDirect, using a set of terms that included the CPA, neurothekeomas, and nerve sheath myxoma.
Table 1: Case reports of individuals with cerebellopontine angle neurothekeoma

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The cases in [Table 1] have some interesting characteristics. First, the report of Multani et al., Vij et al., and the majority of other cases about neurothekeomas of the central nervous system are from Asia.[1],[2],[3] In this way, we suspect that probably genetic, health behavior, infections, or even a complex multifactorial etiology could contribute to the frequency of reports among these countries. Second, both the reports presented similar findings in cranial computed tomography scan and brain magnetic resonance imaging, which may be a supportive criterion when aggressive management is not possible. Third, only Multani et al. presented a hypothesis before the pathology assessment that was plausible based on the epidemiology and neuroimaging characteristics. Fourth, the follow-up was only provided by Vij et al., and their patient had a full recovery. Moreover, when we analyze other cases in the literature about intracranial neurothekeomas such as those localized in the posterior fossa, middle cranial fossa, and parasellar region; more than 90% had a good prognosis with recurrence occurring in approximately 15%.[1],[2],[3],[4],[5]

  References Top

Multani KM, Balasubramaniam A, Kumar MS, Chavali P. A rare cerebellopontine angle neurothekeoma. Int J Neurooncol 20181;1:53.  Back to cited text no. 1
Bulduk EB, Aslan A, Öcal Ö, Kaymaz AM. Neurothekeoma in the middle cranial fossa as a rare location: Case report and literature review. Neurochirurgie 2016;62:336-8.  Back to cited text no. 2
Vij M, Jaiswal S, Agrawal V, Jaiswal A, Behari S. Nerve sheath myxoma (neurothekeoma) of cerebellopontine angle: Case report of a rare tumor with brief review of literature. Turk Neurosurg 2013;23:113-6.  Back to cited text no. 3
Altmeyer MD, Reed JA. Neurothekeoma Pathology: overview, Etiology, Location and Clinical Features; 2016. Available from: http://emedicine.medscape.com/article/2034472overview. [Last accessed on 2019 Sep 09].  Back to cited text no. 4
Alexandru D, Satyadev R, So W. Neurothekeoma in the posterior fossa: Case report and literature review. Perm J 2012;16:63-4.  Back to cited text no. 5


  [Table 1]


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