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.
Highlights
Vestibular schwannoma (VS) is a benign tumor arising from Schwann cells of the vestibular part of the 8th cranial nerve (CN)
There is a trend towards the approach of planned stereotactic radiosurgery (SRS) after planned subtotal resection (Figure 3)
The analysis showed that none of the patients developed a relapse (100% control rate) or radiation-induced complication
Summary
Vestibular schwannoma (VS) is a benign tumor arising from Schwann cells of the vestibular part of the 8th cranial nerve (CN). The tumor can occur as a sporadic (isolated) lesion or as a part of a genetic syndrome. In 5% of the VS cases the cause is neurofibromatosis type 2 (NF2) and the tumor occurs bilaterally [1,2]. VS in NF2 patients is diagnosed much earlier than in sporadic cases due to autosomal dominant inheritance, which leads to screening of known patients and the presence of other symptoms causing brain tumors such as meningiomas, astrocytomas and ependymomas. The options of VS management mainly depend on the size and symptoms. There is a generally accepted classification for the size of tumor: small (less than 1.5 cm), medium (1.5–2.5 cm) or large (more than 2.5 cm). The management for the medium- or small-sized VS includes wait and scan, radiotherapy and/or surgery. This review will provide an update on the pathophysiology, imaging characteristics and discuss the latest insights in patient management
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