Vestibulární schwannom (VS) je intrakraniální benigní tumor vycházející ze Schwannových buněk vestibulární porce vestibulokochleárního nervu. Tento nádor tvoří 85 % tumorů mostomozečkového koutu. Vyrůstá v tzv. přechodové zóně vnitřního zvukovodu, odkud roste směrem k mozkovému kmeni a mozečku. Z toho lze vyvodit posloupnost příznaků, které se u pacienta rozvinou. Mezi jeho nejčastější projevy patří jednostranná porucha sluchu, ušní šelesty a poruchy rovnováhy. Ve většině případů je jeho růst pomalý. V naprosté většině případů (95 %) se jedná o sporadický typ nádoru, ve zbylých 5 % se pak jedná o dědičnou, autozomálně dominantní formu nádoru, která se vyskytuje především u neurofibromatózy 2. typu (NF2).

Vestibular schwannoma (VS) is a benign intracranial tumor derived from myelinating Schwann cells of the vestibular division of the vestibulocochlear nerve. Vestibular schwannomas account for approximately 85 % of cerebellopontine angle tumors. It grows in the so-called transition zone of the internal auditory canal, from which it extends toward the brainstem and cerebellum. From this knowledge, the sequence of symptoms the patient develops can be deduced. The most common manifestations include unilateral hearing loss, tinnitus, and balance disorders. In most cases, VS growth is slow. The vast majority of VS (95 %) occur as sporadic tumors, with the remaining 5 % occurring as part of an inherited, autosomal dominant form of VS, mainly found in patients with neurofibromatosis type 2 (NF2).

• MeSH
• centrální poruchy sluchu MeSH
• diagnostické techniky otologické MeSH
• lidé MeSH
• magnetická rezonanční tomografie MeSH
• mikrochirurgie metody   MeSH
• nervus facialis MeSH
• radiochirurgie MeSH
• rehabilitace MeSH
• sluchové kmenové evokované potenciály MeSH
• vestibulární schwannom * diagnóza   komplikace   terapie   MeSH
• Check Tag
• lidé MeSH

The incidence of sporadic vestibular schwannoma has significantly increased over the past few decades. However, there is no method currently available to accurately predict the risk of subsequent tumor growth. The difference in the management of five patient groups has been evaluated: wait and scan, conversion to microsurgery, conversion to stereoradiotherapy, sterioradiotherapy, and microsurgery. In total, 463 patients with vestibular schwannoma have been consulted in our department from 2010 through 2016. Of the 250 patients initially indicated for observation, 32.4% were later indicated for active treatment. Younger patients were more frequently indicated for surgery (mean age 48 years) compared to older patients, who were more often indicated for stereoradiotherapy (mean age 62 years). Tumor growth was observed more often in patients under 60 years of age and in patients with tumors greater than 10 mm. In elderly patients, including those with larger tumors, a conservative approach is the optimal solution. If tumor growth occurs in the wait-and-scan strategy, it is still possible to continue with a conservative approach in some situations. The duration of follow-up scans is still a matter of debate, as tumors can begin to grow after 5 years from the initial diagnosis.

• Publikační typ
• časopisecké články MeSH

Vestibular schwannoma is the most common benign neoplasm of the cerebellopontine angle. Its first symptoms include hearing loss, tinnitus, and vestibular symptoms, followed by cerebellar and brainstem symptoms, along with palsy of the adjacent cranial nerves. However, the clinical picture has unpredictable dynamics and currently, there are no reliable predictors of tumor behavior. Hence, it is desirable to have a fast routine method for analysis of vestibular schwannoma tissues at the molecular level. The major objective of this study was to verify whether a technique using in-sample specific protein digestion with trypsin would have the potential to provide a proteomic characterization of these pathological tissues. The achieved results showed that the use of this approach with subsequent liquid chromatography-tandem mass spectrometry (LC-MS/MS) analysis of released peptides allowed a fast identification of a considerable number of proteins in two differential parts of vestibular schwannoma tissue as well as in tissues of control healthy samples. Furthermore, mathematical analysis of MS data was able to discriminate between pathological vestibular schwannoma tissues and healthy tissues. Thus, in-sample protein digestion combined with LC-MS/MS separation and identification of released specific peptides followed by mathematical analysis appears to have the potential for routine characterization of vestibular schwannomas at the molecular level. Data are available via ProteomeXchange with identifier PXD045261.

• MeSH
• chromatografie kapalinová metody   MeSH
• lidé MeSH
• peptidové fragmenty * analýza   chemie   metabolismus   MeSH
• peptidy metabolismus   MeSH
• proteolýza MeSH
• proteomika metody   MeSH
• tandemová hmotnostní spektrometrie metody   MeSH
• trypsin chemie   MeSH
• vestibulární schwannom * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

INTRODUCTION: Currently, it is possible to preserve the auditory nerve in a large number of cases, but the preservation of the hearing itself is unpredictable. Apart from wait and scan strategy and stereoradiotherapy, hearing after vestibular schwannoma surgery is considered to remain stable even in long-term follow-up. MATERIALS AND METHODS: Twenty-eight patients had preserved hearing after retrosigmoid suboccipital microsurgery of the vestibular schwannoma between 2008 and 2014. A standard audiological protocol was performed together with an magnetic resonance imaging evaluation of the fluid content of the inner ear. RESULTS: The mean difference in pure-tone average between the direct and final postsurgical examination was 12.758 dB ( p = 2.5E - 06). The word recognition score deteriorated by 17.45% ( p = 0.03516). The mean American Academy of Otolaryngology-Head and Neck Surgery score on the second examination was 2.5, and that on the second examination was 3.111 ( p = 0.00483). There was no significant deterioration in the healthy ear.The signal intensity ratio in the basal turn of the cochlea increased by an average of 0.13 points ( p < 0.05).Patients with persistent tumor or nodular enhancement in the internal acoustic meatus deteriorated significantly in hearing according to the American Academy of Otolaryngology-Head and Neck Surgery scale compared with patients without any finding in the meatus ( p = 0.01299). CONCLUSIONS: There is a discrete but gradual deterioration of the hearing in the postoperative period. Hearing impairment is more pronounced in patients with a nodular process in the internal acoustic meatus, regardless of whether it is growth active. After surgery, the pathological content of the inner ear normalizes (evaluated on T2 magnetic resonance imaging sequences).

• MeSH
• kochlea MeSH
• lidé MeSH
• nedoslýchavost * etiologie   MeSH
• retrospektivní studie MeSH
• sluch MeSH
• vestibulární schwannom * chirurgie   MeSH
• vnitřní ucho * chirurgie   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

Vestibular schwannoma is the most common benign neoplasm of the cerebellopontine angle. It arises from Schwann cells of the vestibular nerve. The first symptoms of vestibular schwannoma include hearing loss, tinnitus, and vestibular symptoms. In the event of further growth, cerebellar and brainstem symptoms, along with palsy of the adjacent cranial nerves, may be present. Although hearing impairment is present in 95% of patients diagnosed with vestibular schwannoma, most tumors do not progress in size or have low growth rates. However, the clinical picture has unpredictable dynamics, and there are currently no reliable predictors of the tumor's behavior. The etiology of the hearing loss in patients with vestibular schwannoma is unclear. Given the presence of hearing loss in patients with non-growing tumors, a purely mechanistic approach is insufficient. A possible explanation for this may be that the function of the auditory system may be affected by the paracrine activity of the tumor. Moreover, initiation of the development and growth progression of vestibular schwannomas is not yet clearly understood. Biallelic loss of the NF2 gene does not explain the occurrence in all patients; therefore, detection of gene expression abnormalities in cases of progressive growth is required. As in other areas of cancer research, the tumor microenvironment is coming to the forefront, also in vestibular schwannomas. In the paradigm of the tumor microenvironment, the stroma of the tumor actively influences the tumor's behavior. However, research in the area of vestibular schwannomas is at an early stage. Thus, knowledge of the molecular mechanisms of tumorigenesis and interactions between cells present within the tumor is crucial for the diagnosis, prediction of tumor behavior, and targeted therapeutic interventions. In this review, we provide an overview of the current knowledge in the field of molecular biology and tumor microenvironment of vestibular schwannomas, as well as their relationship to tumor growth and hearing loss.

• Publikační typ
• časopisecké články MeSH
• přehledy MeSH