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Review
. 2020 Mar 31;12(4):835.
doi: 10.3390/cancers12040835.

Therapy of Sporadic and NF2-Related Vestibular Schwannoma

Longping Yao  1 , Mohammed Alahmari  1   2 , Yasin Temel  1 , Koos Hovinga  1
Affiliations
Review

Therapy of Sporadic and NF2-Related Vestibular Schwannoma

Longping Yao et al. Cancers (Basel). .

Abstract

Vestibular schwannoma (VS) is a benign primary brain tumor that occurs sporadic or as part of a genetic syndrome. The most common cause is the mutation of the NF2 tumor suppressor gene that is involved in the production of the protein merlin. Merlin plays a role in cell growth and cell adhesion. In patients with NF2, the VSs arise bilaterally and coincide with other brain tumors. In sporadic VS, the tumor is typically unilateral and does not coincide in combination with other tumors. MRI is the standard imaging technique and can be used to assess the size and aspect of the tumor as well as the progression of disease. The preferred management of large VS in both VS types is surgery with or without adjuvant radiation. The management for the medium- or small-sized VS includes wait and scan, radiotherapy and/or surgery. This choice depends on the preference of the patient and institutional protocols. The outcomes of surgical and radiotherapy treatments are improving due to progress in surgical equipment/approaches, advances in radiation delivery techniques and dose optimizations protocols. The main purpose of the management of VS is preserving function as long as possible in combination with tumor control.

Keywords: NF2; function preservation and radiation-induced effects; management; radiotherapy; surgery; vestibular schwannoma.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
MRI imaging of sporadic vestibular schwannoma (VS) in cerebellopontine angle: (a) T2-weighted MRI reveals sporadic VS as hypointense signals and (b) T1-weighted image with contrast demonstrates hyperintensity of sporadic VS (courtesy of Y. Temel).
Figure 2
Figure 2
MRI imaging of bilateral NF2 VS in cerebellopontine angle: (a) T1-weighted MRI reveals bilateral NF2 VS as hypo-intense signals; (b) T2-weighted MRI and (c) T1-weighted MRI with contrast demonstrates hyperintensity of bilateral NF2 VS [53].
Figure 3
Figure 3
An axial T1 MRIs with contrast for left VS (KOOS D), showing tumor shrinkage after the combined approach; A, is the image before surgery; B, after surgery and before GK; C, 6 months after GK; D, 5 years after GK (courtesy of Y. Temel).
See this image and copyright information in PMC

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