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Schwannomas are benign tumours derived from Schwann cells of the peripheral nerve sheaths. A schwannoma arising in the external auditory canal (EAC) is rare, with few cases reported in the literature. These tumours present a unique challenge and should be considered when patients present with persistent ear symptoms in the absence of common otological pathology. We present a case of a man in his late 60s, referred to the otolaryngology team with unilateral conductive hearing loss and a right-sided EAC mass found on otoscopy. CT and MRI of the head and neck revealed a mass occluding the right EAC, originating from a peripheral cutaneous nerve. Management including biopsy, histopathological analysis and surgical excision in its entirety resulted in a good outcome, leaving a symptom-free patient., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Schwannomas originating from the intravertebral canal rarely extend into the paravertebral region or form large masses. There are few reports on such medical cases, and their clinical diagnosis and management are poorly understood. Here, we report a case of an intraspinal schwannoma with a giant extraspinal mass in a middle-aged Chinese woman and the clinical implications of the symptoms, diagnosis, and treatment of thoracic vertebral schwannoma., Case Presentation: A 59-year-old female patient who presented with sudden lower limb numbness and difficulty in walking was referred to our department. Magnetic resonance imaging (MRI) revealed a tumor compressing the T11-T12 spinal canal and extensively invading the left thoracic cavity to form a giant paravertebral mass. Schwannoma was diagnosed after intraoperative pathological examination, in which tumor cells were immunoreactive to S100 and Sox10 but negative for smooth muscle actin (SMH). Intraspinal mass resection through the posterior approach and extraspinal chest mass resection under video-assisted thoracoscopy through the left lateral approach were performed simultaneously, and instrumentation was used to maintain thoracic spinal stability., Conclusion: In patients with large schwannomas, customized treatment plans are crucial. Tailoring the approach to an individual's specific condition by considering factors such as tumor size and location is essential for optimal outcomes., (© 2024. The Author(s).)

Objective: To evaluate the literature and summarize cochlear implantation (CI) outcomes after intralabyrinthine schwannoma (ILS) excision and tumor observation with CI., Data Sources: OVID Medline, Embase, Web of Science; conception to 2024., Review Methods: A literature review was performed using subject headings, MeSH terms, and keywords. Abstracts and full texts were reviewed by two independent reviewers and adjudicated by a third. Inclusion criteria included studies with ILS and CI with reported audiologic outcomes. Subjects were analyzed into two groups, ILS resection with CI and in situ ILS with CI. Patients with NF2 were included. The main outcome of interest was CI audiometric performance level, with secondary outcomes of CI user status and open-set speech attainment., Results: There were 29 articles with a total of 93 patients who met inclusion criteria. The resection group had 17% low performers, 44% intermediate performers, and 38% high performers. The in situ group had 40% low performers, 32% intermediate performers, 27% high performers. The resection group had 69 patients with 97% maintaining user status and 92% with open-set speech recognition. The observation group had 24 patients, with 87% user rate and 86% achieving open-set speech recognition. There was a greater percentage of NF2 diagnosis seen in the in situ group., Conclusion: There is a paucity of literature on CI and ILS. Patients are managed with both resection of tumor and implantation in situ. Early data are limited, with improvement in hearing outcomes and high user rates in both populations., Level of Evidence: N/A Laryngoscope, 134:3910-3920, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

An 80-year-old male underwent chest computed tomography (CT) due to complaints of right-sided chest pain. A chest wall tumor was identified in the right eighth intercostal space, corresponding to the location of his pain. The patient was subsequently referred to our department for further evaluation and treatment. Utilizing single-port thoracoscopic surgery, the tumor was successfully excised. Intraoperatively, the tumor was found beneath the parietal pleura, was contiguous with to the intercostal nerve. Histopathological analysis confirmed the diagnosis of schwannoma originating from the intercostal nerve. The right-sided chest pain was reduced after operation significantly. No recurrence or relapse of symptoms was observed during follow-up. Although schwannoma of the chest wall in asymptomatic in many cases, in this case, localized pain corresponding to the tumor site was evident. This emphasizes the importance of considering schwannoma in the differential diagnosis of chest pain.

Objectives: To determine the natural history of hearing loss and tumor volume in patients with untreated neurofibromatosis type 2 (NF2)-related schwannomatosis. Moreover, we statistically examined the factors affecting hearing prognosis., Methods: This retrospective cohort study was conducted on 37 ears of 24 patients with NF2-related vestibular schwannomatosis followed up without treatment for more than 1 year. We obtained detailed chronological changes in the PTA and tumor volume in each case over time, and the rate of change per year was obtained. Multivariate analysis was also conducted to investigate factors associated with changes in hearing., Results: The average follow-up period was approximately 9 years, and hearing deteriorated at an average rate of approximately 4 dB/year. The rate of maintaining effective hearing decreased from 30 ears (81%) at the first visit to 19 ears (51%) at the final follow-up. The average rate of change in tumor growth for volume was approximately 686.0 mm 3 /year. This study revealed that most patients with NF2 experienced deterioration in hearing acuity and tumor growth during the natural course. A correlation was observed between an increase in tumor volume and hearing loss (r = 0.686; p < 0.001)., Conclusions: Although the hearing preservation rate in NF2 cases is poor with the current treatment methods, many cases exist in which hearing acuity deteriorates, even during the natural course. Patients with an increased tumor volume during the follow-up period were more likely to experience hearing deterioration. Trial registration number 20140242 (date of registration: 27 October 2014)., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: We identified the potential role of electroacupuncture (EA) as an alternative therapy to traditional Chinese medicine (TCM) in a rare case of postoperative gastroparesis after gastric schwannoma (GS)., Clinical Presentation: A 31-year-old woman presented with impaired gastric emptying after gastrectomy for GS and was diagnosed with postoperative gastroparesis syndrome (PGS). The symptoms were slightly relieved after routine placement of the jejunal tube; however, symptoms such as dietary intolerance and impaired gastric emptying persisted. After the consultation, the patient agreed to undergo EA therapy., Intervention and Results: The patient was able to tolerate oral intake after seven days of EA treatment, and the frequency and amount of food intake increased. The jejunal tube was removed at the outpatient follow-up two weeks after discharge, and the patient resumed a semi-liquid diet and was able to eat small amounts of rice. Reexamination of the upper digestive tract angiography showed that part of the contrast agent passed through the pyloric sinus, which showed improvement., Conclusion: EA stimulation increased tolerance to transoral feeding in patients with postoperative gastroparesis and facilitated the passage of contrast agents through the pyloric sinus. No adverse effects were observed during treatment, and the treatment was well accepted and tolerated by patients. A review article noted the benefits of acupuncture for gastrointestinal disorders but lacked high-quality evidence to support this. 1 Therefore, the therapeutic role of EA needs to be further elucidated to provide high-quality evidence-based medical evidence for its clinical use., (Copyright © 2023. Published by Elsevier Inc.)

Objective: To evaluate long-term hearing outcomes following cochlear implantation in patients with neurofibromatosis type 2 and ipsilateral vestibular schwannoma., Study Design: Retrospective study., Setting: Tertiary general hospital., Methods: Twenty-two patients undergoing cochlear implantation between 2004 and 2018 with at least 1 year of follow-up were included. Patients were categorized as "users" or "nonusers" of their cochlear implant (CI). For users, speech perception (disyllabic words) without lip-reading was assessed in quiet conditions 1-year postimplantation, and annually thereafter. CI users were classified into 2 groups on the basis of speech intelligibility (≥40% or <40%). Demographic data, treatment options, and tumor size were also recorded., Results: One year after implantation, 16 (73%) patients used their CI daily. Twelve of these patients had a speech intelligibility ≥40% (mean: 74 ± 21.9%). Three had a Koos stage IV tumor. At the last visit (mean duration of follow-up: 6 ± 5 years), 12 of these 16 patients were still using their implant daily, and 6 had a speech intelligibility ≥40%. No predictive factors for good performance at 1 year or performance stability were identified., Conclusion: Neurofibromatosis type 2 is a complex disease profoundly affecting patient quality of life, and cochlear implantation should always be considered on a case-by-case basis. In some individuals, cochlear implantation can provide good speech intelligibility for extended periods, even posttreatment or in cases of large tumors., (© 2024 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

NF2-related schwannomatosis (NF2) is a rare autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas and multiple meningiomas. This case report presents the extremely rare occurrence of an anaplastic meningioma in a 12-year-old male with previously undiagnosed NF2. The patient presented with a history of abdominal pain and episodic emesis, gait unsteadiness, right upper and lower extremity weakness, and facial weakness. He had sensorineural hearing loss and wore bilateral hearing aids. MR imaging revealed a sizable left frontoparietal, dural-based meningioma with heterogeneous enhancement with mass effect on the brain and midline shift. Multiple additional CNS lesions were noted including a homogenous lesion at the level of T5 indicative of compression of the spinal cord. The patient underwent a frontotemporoparietal craniotomy for the removal of his large dural-based meningioma, utilizing neuronavigation and transdural ultrasonography for precise en bloc resection of the mass. Histopathology revealed an anaplastic meningioma, WHO grade 3, characterized by brisk mitotic activity, small-cell changes, high Ki-67 proliferation rate, and significant loss of P16. We report an anaplastic meningioma associated with an underlying diagnosis of NF2 for which we describe clinical and histopathological features., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Orbital schwannoma is a rare benign tumor, originating from the Schwann cells of the orbital peripheral nerve sheath. Orbital schwannoma is easily misdiagnosed if the patient shows atypical presentations and atypical appearance on MRI imaging. A 56-year-old male experienced hyposmia for 1 year and was misdiagnosed with cavernous hemangioma pre-operation. This case was treated by surgery through the endoscopic trans-nasal approach. After operation, the patient had no recurrence or complications. Preoperative diagnosis for these cases remains difficult. Combined imaging modalities including computed tomography (CT) and magnetic resonance imaging (MRI) can help in differential diagnosis. Surgery is the main treatment modality for treating orbital schwannoma. Outcomes in most cases are favorable without complications or recurrence., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Hearing loss is a common side effect of many tumor treatments. However, hearing loss can also occur as a direct result of certain tumors of the nervous system, the most common of which are the vestibular schwannomas (VS). These tumors arise from Schwann cells of the vestibulocochlear nerve and their main cause is the loss of function of NF2, with 95 % of cases being sporadic and 5 % being part of the rare neurofibromatosis type 2 (NF2)-related Schwannomatosis. Genetic variations in NF2 do not fully explain the clinical heterogeneity of VS, and interactions between Schwann cells and their microenvironment appear to be critical for tumor development. Preclinical in vitro and in vivo models of VS are needed to develop prognostic biomarkers and targeted therapies. In addition to VS, other tumors can affect hearing. Meningiomas and other masses in the cerebellopontine angle can compress the vestibulocochlear nerve due to their anatomic proximity. Gliomas can disrupt several neurological functions, including hearing; in fact, glioblastoma multiforme, the most aggressive subtype, may exhibit early symptoms of auditory alterations. Besides, treatments for high-grade tumors, including chemotherapy or radiotherapy, as well as incomplete resections, can induce long-term auditory dysfunction. Because hearing loss can have an irreversible and dramatic impact on quality of life, it should be considered in the clinical management plan of patients with tumors, and monitored throughout the course of the disease., (Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)

Oculomotor nerve schwannoma in children not associated with neurofibromatosis is a rare disease, with 26 pediatric cases reported so far. There is no established treatment plan. A 7-year-old girl presented with oculomotor nerve palsy. Surgical reduction of the tumor combined with postoperative gamma knife surgery preserved the oculomotor nerve, improved oculomotor nerve function, and achieved tumor control during the observation period of 20 months. The combination of partial surgical resection and gamma knife surgery as a treatment strategy for oculomotor nerve schwannoma resulted in a good outcome., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

BACKGROUND Aside from the rarity of mobile spinal schwannomas, the coexistence of these tumors with herniated intervertebral disc is also scarce. Furthermore, cauda equina syndrome (CES), as a manifestation of intraspinal schwannomas has been reported rarely. Described here is a case of simultaneous lumbar disc bulge and mobile spinal schwannoma presented with intermittent symptoms of CES. CASE REPORT A 62-year-old man presented with severe but intermittent leg pain for 2 weeks, which later progressed to an episode of lower extremity weakness and difficulty in urination. Magnetic resonance imaging revealed an intraspinal tumor that moved in position relative to the L1-2 disc bulge on scans 6 h apart, with associated spontaneous regression in symptoms. The tumor was found to be a mobile spinal schwannoma, originated from a nerve root. A standard microdissection technique was used to remove the tumor through a spinous process-sparing unilateral approach, with complete laminectomy of L1. Use of intraoperative ultrasound facilitated the accurate tumor localization. Postoperatively, the patient no longer had symptoms. CONCLUSIONS This report presents a combination of a common spinal pathology, intervertebral disc herniation, alongside a rare condition, mobile spinal schwannoma, whose uncommon clinical manifestations, such as CES can cause irreversible neurological deficits. Surgeons need to remain vigilant of potential atypical scenarios when treating patients. Surgical treatment challenges regarding the mobility of tumors, such as accurate localization, should be addressed using intraoperative imaging to avoid wrong-level surgery. To mitigate the irreversible neurological complications, patients should receive comprehensive information for alarming signs of CES.

Concurrent occurrence of schwannoma and meningiomas are rare, and are found especially in association with neurofibromatosis type 2 (NF2). Occurrence of mixed tumor without the aforementioned conditions is extremely rare. We present three cases of mixed tumor in different locations, including two with NF2 and one without NF2. We analyse the relationship of mixed tumor with NF2 and its clinical implications. Presence of mixed schwannoma-meningioma should prompt screening for NF2. Thus aids in early diagnosis of unsuspected NF2 cases. We observed that irrespective of different locations, cases with NF2 showed frequent recurrence of schwannoma as compared to case who did not fit in the existing clinical criteria for NF2. Collision tumor and thereby NF2 mutations indicates the prognosis and recurrence of the tumor, thereby guides in deciding the management., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: Cerebellopontine angle (CPA) tumors are a common cause of secondary trigeminal neuralgia (TN), characterized by their concealed location, slow progression, and difficulty in early detection. This study aims to explore the clinicopathological characteristics of patients with secondary TN due to CPA tumors to enhance understanding and management of secondary TN., Methods: A retrospective analysis was conducted on clinical data and pathological results of 116 patients with CPA tumor-related TN treated at Xiangya Hospital of Central South University from January 1, 2017 to December 31, 2022. The study analyzed the relationship of tumor pathological types with clinical manifestations, tumor location, surgical methods, and treatment outcomes., Results: Among the cases, 95.7% (111/116) were benign tumors, 3.4% (4/116) were malignant tumors, and 0.9% (1/116) were borderline tumors. Benign tumors were predominantly acoustic neuromas, meningiomas, and schwannomas. Among the patients, 46.6% (54/116) presented with isolated TN, while 53.4% (62/116) exhibited other associated symptoms depending on factors such as tumor growth location and rate. The complete resection rate in this group was over 90%, with 41.4% (48/116) of patients undergoing concurrent microvascular decompression after tumor resection, predominantly for schwannomas. The overall effective rate of surgical treatment reached 93.9%, with schwannomas showing higher efficacy rates compared with acoustic neuromas and meningiomas ( P <0.05). The recurrence rate of acoustic neuromas was significantly higher than that of meningiomas and schwannomas ( P <0.05)., Conclusions: CPA tumors are a major cause of secondary TN, predominantly benign, with occasional underdiagnosed malignant tumors. Early diagnosis and treatment significantly impact prognosis. Different tumor types vary in clinical symptoms, surgical approaches, and treatment efficacy. Surgical strategies should balance tumor resection extent and neural function preservation, with microvascular decompression as necessary.

Benign mediastinal tumor is usually asymptomatic and exhibits uncomplicated clinical course. Posterior mediastinal schwannoma is common, but a huge benign tumor causing acute respiratory failure due to mass effect is unusual. We present a patient who suffered from acute respiratory failure due to huge mediastinal mass effect and improved after en bloc surgical resection. A 56-year-old woman had no history of systemic disease, but experienced general discomfort and malaise for several months. She was referred to our emergency department after developing sudden respiratory failure. Intubation was performed with ventilator support and she was admitted to the intensive care unit. Chest radiograph and computed tomography showed a huge mass over the left pleural cavity causing left lung, heart, and mediastinal compression. After en bloc resection, she was weaned off the ventilator successfully and was discharged at 24 days after the operation. Postoperative outpatient follow-up showed no symptoms. Mediastinal ancient schwannoma is a rare posterior mediastinal benign tumor. However, mass effect might lead to lethal complications. En bloc resection is necessary for curative treatment., (© 2024. The Author(s).)

Background and Objective: The incidence of intralabyrinthine schwannomas is increasing, and a growing attention is given to the detrimental effects on hearing function. On the contrary, the vestibular profile of intralabyrinthine vestibular schwannomas (VSs) is still not well understood. We aimed to investigate and report the observed relationships between the intralabyrinthine location of the schwannomas and objective and subjective vestibular profile of the patients., Methods: Retrospective cohort study of 20 consecutive individuals with sporadic intralabyrinthine schwannomas and grouped according to the intralabyrinthine location of the schwannomas. Vestibular testing consisted of the video head impulse test of all three semicircular canals, the caloric test, cervical and ocular vestibular evoked myogenic potentials, and the dizziness handicap inventory. A nonparametric unpaired t test was performed to compare groups, and Fisher's exact test was used for categorical data., Results: The median video head impulse test gains (lateral, anterior, posterior) were 0.40, 0.50, and 0.75 for intravestibular schwannomas and 0.93, 1.52, and 0.91 for intracochlear schwannomas ( p = 0.0001, p = 0.009, p = 0.33), respectively. Caloric unilateral weakness had a median of 100% for intravestibular schwannomas and 14% for intracochlear schwannomas ( p = 0.0001). The mean dizziness handicap inventory was 21 for intravestibular schwannomas and 1 for cochlear schwannomas ( p = 0.02). There were no significant differences in vestibular evoked myogenic potentials according to intralabyrinthine location., Conclusion: By both objective and subjective measures, intralabyrinthine schwannomas with an intravestibular component has significantly worse vestibular function than schwannomas with purely cochlear involvement., Competing Interests: Disclosure and funding: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Background: Facial paralysis secondary to neurofibromatosis type 2 (NF2) presents the reconstructive surgeon with unique challenges because of its pathognomonic feature of bilateral acoustic neuromas, involvement of multiple cranial nerves, use of antineoplastic agents, and management. Facial reanimation literature on managing this patient population is scant., Methods: A comprehensive literature review was performed. All patients with NF2-related facial paralysis who presented in the past 13 years were reviewed retrospectively for type and degree of paralysis, NF2 sequelae, number of cranial nerves involved, interventional modalities, and surgical notes., Results: Twelve patients with NF2-related facial paralysis were identified. All patients presented after resection of vestibular schwannoma. Mean duration of weakness before surgical intervention was 8 months. On presentation, one patient had bilateral facial weakness, 11 had multiple cranial nerve involvement, and seven were treated with antineoplastic agents. Two patients underwent gracilis free functional muscle transfer, five underwent masseteric-to-facial nerve transfer (of whom two were dually innervated with a crossfacial nerve graft), and one patient underwent depressor anguli oris myectomy. Trigeminal schwannomas did not affect reconstructive outcomes if trigeminal nerve motor function on clinical examination was normal. In addition, antineoplastic agents such as bevacizumab and temsirolimus did not affect outcomes if stopped in the perioperative period., Conclusions: Effectively managing patients with NF2-related facial paralysis necessitates understanding the progressive and systemic nature of the disease, bilateral facial nerve and multiple cranial nerve involvement, and common antineoplastic treatments. Neither antineoplastic agents nor trigeminal nerve schwannomas associated with normal examination affected outcomes., Clinical Question/level of Evidence: Therapeutic, V., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Objectives: To characterize facial nerve (FN) schwannomas (FNSs) and FN hemangiomas (FNHs) and their clinical features and management strategies, and to describe the results of cable nerve grafting after FN sectioning during tumor removal., Methods: This retrospective study included 84 FNS cases and 42 FNH cases managed between July 1989 and July 2020 at a quaternary referral center for skull base pathology. Clinical details, locations, management, and results of cable nerve grafting at 1 year and during an average period of 3.12 years were evaluated. Sural nerve interpositioning was performed for patients who experienced FN paralysis for less than 1 year and underwent nerve sectioning during tumor removal., Results: FNSs more often involved multiple segments compared with FNHs. The cerebellopontine angle and the mastoid segments were involved in 16 (19.1%) and 34 (40.5%) FNS cases, respectively; however, the cerebellopontine angle and the mastoid segments were involved in 0 and 7 (16.7%) FNH cases, respectively. Sectioned nerves of 99 patients (78.6%) were restored using interposition cable grafting. At the last follow-up evaluation, 56.3% of FNSs and 60.7% of FNHs attained House-Brackmann (HB) grade III. Lower preoperative HB grades were associated with poorer postoperative outcomes. For FNSs, the mean HB grades were 4.13 at 1 year postoperatively and 3.75 at the last follow-up evaluation ( p = 0.001); however, for FNHs, the mean HB grades were 4.04 postoperatively and 3.75 at the last follow-up evaluation. Therefore, extradural coaptation yielded better outcomes., Conclusion: FNSs can occur along any part of the FN along its course, and FNHs are concentrated around the area of geniculate ganglion. The results of cable inter positioning grafts are better in patients with preoperative FN-HB-III or less when compared with higher grades. The outcome of the interpositioning continues to improve even after 1 year in extradural coaptation., Competing Interests: Conflict of interest: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Purpose: Neurofibromatosis type 2 (NF2) is intractable because of multiple tumors involving the nervous system and is clinically diverse and genotype-dependent. Stereotactic radiosurgery (SRS) for NF2-associated schwannomas remains controversial. We aimed to investigate the association between radiosurgical outcomes and mutation types in NF2-associated schwannomas., Methods: This single-institute retrospective study included consecutive NF2 patients with intracranial schwannomas treated with SRS. The patients' types of germline mutations ("Truncating," "Large deletion," "Splice site," "Missense," and "Mosaic") and Halliday's genetic severity scores were examined, and the associations with progression-free rate (PFR) and overall survival (OS) were analyzed., Results: The study enrolled 14 patients with NF2 with 22 associated intracranial schwannomas (median follow-up, 102 months).　The PFRs in the entire cohort were 95% at 5 years and 90% at 10-20 years. The PFRs tended to be worse in patients with truncating mutation exons 2-13 than in those with other mutation types (91% at 5 years and 82% at 10-20 years vs. 100% at 10-20 years, P = 0.140). The OSs were 89% for patients aged 40 years and 74% for those aged 60 years in the entire cohort and significantly lower in genetic severity group 3 than in the other groups (100% vs. 50% for those aged 35 years; P = 0.016)., Conclusion: SRS achieved excellent PFR for NF2-associated intracranial schwannomas in the mild (group 2A) and moderate (group 2B) groups. SRS necessitates careful consideration for the severe group (group 3), especially in cases with NF2 truncating mutation exons 2-13., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: To identify the characteristics of pain syndrome in patients with schwannomas depending on genetic predisposition., Material and Methods: The study included 46 patients with peripheral, spinal and intracranial schwannomas, corresponding to the schwannomatosis phenotype according to the 2022 clinical criteria. All patients underwent sequencing of the LZRT1 , Nf2 and SMARCB1 and a copy number study in the NF2 ., Results: The most severe widespread pain was observed in patients with pathogenic LZRT1 variants, while patients with mosaic variants may not even have local tumor-related pain. Patients with SMARCB1 variants may have no pain or have localized pain that responds well to surgical treatment., Conclusion: Further studies of the molecular features of schwannomatosis and driver mutations in the pathogenesis of pain are necessary to improve the effectiveness of pain therapy in this group of patients. Schwannomatosis is a disease from the group of neurofibromatosis, manifested by the development of multiple schwannomas. Neuropathic pain is one of the main symptoms characteristic of peripheral schwannomas, however, the severity and prevalence of the pain syndrome does not always correlate with the location of the tumors. According to modern concepts, the key factors influencing the characteristics of the pain syndrome are the target gene and the type of pathogenic variant. The most severe widespread pain is observed in patients with pathogenic variants in the LZRT1 gene, while patients with mosaic variants may not even have local pain associated with tumors. Patients with variants in SMARCB1 may have no pain or localized pain that responds well to surgical treatment.