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Background: Neurofibromatosis type 2 (NF2) is a rare autosomal dominant syndrome with a predisposition to the development of central nervous system tumors, ophthalmic manifestations, and dermatological lesions. The latter are present in 70-95% of patients and can precede the evolution of other tumors. However, they are not included in the diagnostic criteria and are frequently undervalued during follow-up., Methods: An observational cross-sectional study characterizing cutaneous lesions in a cohort of NF2 patients was carried out. Dermatological examinations were performed, and lesions were classified into neural cutaneous tumors (superficial, SNCT, and deep, DNCT), hyperpigmented patches (HyperP), and hypopigmented patches (HypoP). The Dermatology Life Quality Index (DLQI) and EQ-5D questionnaires were applied to evaluate the impact on quality of life., Results: Nineteen patients with a mean age of 36 years were included. Sixteen (84%) patients had cutaneous lesions, mostly developed 10 or more years before the diagnosis. SNCT, DNCT, and HyperP showed similar frequencies (58%). HypoP were observed in only one patient. HyperP developed, on average, earlier than NCT (9.6 vs. 16.5 SNCT, 17.0 DNCT; years). The excised lesions had different histological patterns, including neurofibromas, schwannomas, and a hybrid tumor. Most patients reported a low impact of cutaneous manifestations on the quality of life (DLQI 0 or 1)., Conclusions: Cutaneous lesions are frequent in NF2 and may precede the diagnosis by several years. Their identification is important to establish the diagnosis earlier and potentially reduce morbidity and mortality., (© 2024 the International Society of Dermatology.)

Schwannomas are benign tumors originating from Schwann cells, with seminal vesicle schwannomas being exceedingly rare. This report describes a 54-year-old man with an incidental discovery of a right-sided seminal vesicle mass during a routine ultrasound examination. Further imaging, including MRI and contrast-enhanced CT scans, revealed a well-defined, encapsulated mass with heterogeneous signal intensity suggestive of schwannoma. Histopathological examination confirmed schwannoma, marked by positive S-100 and NSE expression and a Ki67 index of 5%. The patient underwent successful laparoscopic resection without complications and remained asymptomatic for over a month. This case underscores the importance of integrating MRI and histopathological findings to accurately diagnose seminal vesicle schwannomas and guide appropriate surgical management, highlighting the need for increased clinician awareness of this rare tumor., (Copyright: © Neurosciences.)

Background: Spinal schwannomas presenting with an intraspinal hematoma or subarachnoid hemorrhage are extremely rare, and patients often have severe spinal cord compression symptoms. However, the mechanism underlying the bleeding remains unclear., Case Presentation: We present the case of a 53-year-old Chinese female diagnosed with a T12 schwannoma accompanied by an intratumoral hematoma. The patient suddenly experienced unbearable pain in the lower limbs. An emergency operation was necessary, and during surgery, we resected a tumor and evacuated a hematoma. We found a spinal nerve root fracture, intratumoral congestion, tumor capsule rupture, and bleeding. Pathological analysis indicated a schwannoma., Conclusion: Injury to the nerve roots and vessels during motion, nerve root torsion, twisting, venous obstruction, tumor congestion, swelling, and capsule rupture are important processes in spinal schwannoma hemorrhage. Early diagnosis and proactive surgery are key points for treatment., Competing Interests: Declarations. Ethics approval and consent to participate: No ethical approval was sought for this report as this is not a research study. As this case report involved private information disclosure, informed consent was obtained from the reported patient. Consent for publication: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests: The authors declare that they have no conflicts of interest., (© 2024. The Author(s).)

Objectives: Tumors involving the vagus nerve are often clinically silent. We offer a case series with different clinical presentations and distinctive post-surgical sequelae that highlight some of the challenges associated with managing cervical vagal nerve tumors., Methods: Single-institution, retrospective review of patients with tumors involving the vagus nerve. We describe clinical presentations and postoperative sequelae of five patients who underwent surgical management of vagal nerve pathology with atypical presentation or subsequent clinical course., Results: Here, we present five patients treated at our institution for vagal tumors. In four of the five patients, the presenting symptoms resolved after surgery. Two patients presented with intractable neurogenic cough, and another two presented with autonomic symptoms, one with syncope/palpitations and the other with intractable sweating. The final patient presented with a rapidly enlarging vagal paraganglioma and developed intractable cough after resection. We present two patients with novel approach to vagal paragangliomas that underwent ligation of feeding blood supply without removing the tumor, resulting in resolution of an intractable cough in one patient and resolution of severe nighttime sweating in the other., Conclusion: Management of tumors associated with the cervical vagus nerve that present with symptoms or rapid growth poses a clinical dilemma. Consideration of the tumor origin with either enucleation of schwannomas or ligation of feeding vessels may preserve function while addressing the presenting symptoms., 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.

Purpose: In patients with inner ear schwannomas (IES), reports on hearing rehabilitation with cochlear implants (CI) have increased over the past decade, most of which are case reports or small case series. The aim of this study is to systematically review the reported hearing results with CI in patients with IES considering the different audiologic outcome measures used in different countries., Methods: According to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline, a search of published literature was conducted. We included patients with IES (primary or with secondary extension from the internal auditory canal (IAC) to the inner ear, sporadic or NF2 related) undergoing cochlear implantation with or without tumour removal. The audiological results were divided into the categories "monosyllables", "disyllables", "multisyllabic words or numbers", and "sentences"., Results: Predefined audiological outcome measures were available from 110 patients and 111 ears in 27 reports. The mean recognition scores for monosyllabic words with CI were 55% (SD: 24), for bisyllabic words 61% (SD: 36), for multisyllabic words and numbers 87% (SD: 25), and 71% (SD: 30) for sentences. Results from for multisyllabic words and numbers in general showed a tendency towards a ceiling effect. Possible risk factors for performance below average were higher complexity tumours (inner ear plus IAC/CPA), NF2, CI without tumour removal ("CI through tumour"), and sequential cochlear implantation after tumour removal (staged surgery)., Conclusion: Hearing loss in patients with inner ear schwannomas can be successfully rehabilitated with CI with above average speech performance in most cases. Cochlear implantation thus represents a valuable option for hearing rehabilitation also in patients with IES while at the same time maintaining the possibility of MRI follow-up. Further studies should investigate possible risk factors for poor performance. Audiological tests and outcome parameters should be reported in detail and ideally be harmonized to allow better comparison between languages., Competing Interests: Declarations Conflict of interest The institutions of SKP and TR receive research grants from Cochlear Ltd., Sydney, Australia, and MED-EL, Innsbruck, Austria. This work was supported by intramural funds from Martin Luther University Halle-Wittenberg only., (© 2024. The Author(s).)

Rationale: Charcot-Marie-Tooth (CMT) disease, a hereditary motor and sensory neuropathy, presents with progressive chronic sensory and distal motor polyneuropathy. While sensorineural hearing loss and vestibular impairment have been documented in CMT patients, concurrent middle ear cholesteatoma and persistent direction-changing positional nystagmus have not., Patient Concerns: This study details a 22-year-old man with CMT1 exhibiting these symptoms., Diagnoses: A 22-year-old man with CMT1 (PMP22, c.319+1G>T mutation) presented with rotatory vertigo, left-sided hearing loss, and aural fullness. Examination revealed middle ear effusion and a whitish mass behind the left tympanic membrane. Audiometry showed mixed hearing loss on the left side. Imaging indicated middle ear cholesteatoma and facial nerve hypertrophy., Interventions: Surgical removal of the cholesteatoma revealed a dehiscent hypertrophied facial nerve., Outcomes: Postoperative follow-up showed improved hearing and no recurrence., Lessons: This case highlights 3 key points: facial nerve hypertrophy in CMT requiring differentiation from schwannoma, the first reported instance of middle ear cholesteatoma in a CMT patient, and vertigo due to acute otitis media complicated by serous labyrinthitis, manifesting as direction-changing positional nystagmus. These findings underscore the need for thorough diagnosis and management in such presentations. This is the first report of concomitant middle ear cholesteatoma in a CMT patient, illustrating the complexity of diagnosis and treatment., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

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.)

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.)

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.)

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.)

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.

We present a rare case of a schwannoma which is localized on the posterior pharyngeal wall. It caused severe difficulty in swallowing and breathing in a 3-year-old child. Schwannomas of the posterior pharyngeal wall have been rarely reported in children. To our knowledge, only 7 cases of posterior pharyngeal wall schwannoma have been previously reported in the literature, but none of them is associated with a child. The tumor was removed with plasma radiofrequency excision via a transoral route. Computed tomography or magnetic resonance imaging examination showed no recurrence of the tumor up to 2 years. To our knowledge, for the first time, a case of posterior pharyngeal wall schwannoma has been reported in a 3-year-old child, and the schwannoma was removed via plasma radiofrequency transoral excision. This case study illustrates that schwannoma may occur in children, and it is safe to treat this disease through plasma radiofrequency transoral excision route., 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.

Intracochlear schwannomas (ICS) are very rare benign tumours of the inner ear. We present histopathological proof of the extremely rare bilateral occurrence of intracochlear schwannomas with negative blood genetic testing for neurofibromatosis type 2 (NF2). Bilateral schwannomas are typically associated with the condition NF2 and this case is presumed to have either mosaicism for NF2 or sporadic development of bilateral tumours. For progressive bilateral tumour growth and associated profound hearing loss, surgical intervention via partial cochleoectomy, tumour removal, preservation of the modiolus, and simultaneous cochlear implantation with lateral wall electrode carrier with basal double electrode contacts was performed. The right side was operated on first with a 14-month gap between each side. The hearing in aided speech recognition for consonant-nucleus-consonant (CNC) phonemes in quiet improved from 57% to 83% 12 months after bilateral cochlear implantation (CI). Bilateral intracochlear schwannomas in non-NF2 patients are extremely rare but should be considered in cases of progressive bilateral hearing loss. Successful tumour removal and cochlear implantation utilizing a lateral wall electrode is possible and can achieve good hearing outcomes., (© 2023. The Author(s).)

Rationale: Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous syndrome that causes multiple central and peripheral nerve sheath tumors. People with NF1 have a 10% chance of developing malignant peripheral nerve sheath tumors (MPNSTs). Here we report a unique instance of a malignant schwannoma that has remained free of metastasis since its initial removal a decade ago. The malign schwannoma has been infrequently documented in the literature, and remarkably, no instances of such an extensive postoperative time without metastases have ever been described., Patient Concerns: A 46-year-old male patient with NF had multiple neurofibromas in different parts of his body, underwent surgery about 10 years ago (2013), and was diagnosed histopathologically as MPNST., Diagnoses: He was admitted to our institution with a recurrent mass in the posterior third of the proximal thigh and severe pain radiating to the left lower extremity, which presented as sciatic pain (2021). A magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography examination revealed that the tumor was likely malignant., Interventions: Surgical excision was performed., Outcome: A 10-year follow-up revealed no metastases or neurologic impairment., Lessons: When articles about benign schwannomas are placed in a separate category, little is written about NF-1-related malignant schwannomas of the sciatic nerve. MPNSTs are high-grade, aggressive sarcomas with a high risk of local recurrence (40%-65%) and metastasis to other body parts. Therefore, among the various benign peripheral nerve sheath tumors in NF-1 patients, the diagnosis of MPNST is crucial.Orthopedic surgeons should be aware that neurofibromas in NF-1 have a significant risk of developing MPNSTs. This study reports the successful treatment of a giant malignant sciatic nerve schwannoma with a long follow-up period without metastasis., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Cervical prolapsed intervertebral disc is one of the common conditions causing cervical myeloradiculopathy. Anterior Cervical Discectomy and Fusion (ACDF) is the standard line of management for the same. Intradural neurogenic origin tumors are relatively rare and can present with features of myeloradiculopathy. Radiological imaging plays important role in diagnosis of such pathologies., Case Report: We report a patient with C5-6 cervical disc prolapse that presented with radiculopathy symptoms in the right upper limb, which was refractory to conservative care. He underwent a C5-6 ACDF and reported complete relief from symptoms at 4 weeks. He developed deteriorating symptoms over the next 10 weeks and presented at 14 weeks follow-up with severe myeloradiculopathy symptoms on the left upper limb with upper limb weakness. A fresh MRI identified an intradural extramedullary tumor with cystic changes at the index surgery level. This was treated with tumor excision and histopathology confirmed a diagnosis of schwannoma. Simultaneous presence of cord signal changes with disc herniation obscured the cystic schwannoma which became apparent later on contrast enhanced MRI imaging., Conclusion: Careful review of preoperative imaging and contrast MRI study may help in diagnosing cystic schwannomas with concomitant cervical disc herniations that have cord signal changes., (© 2023. The Author(s), under exclusive licence to International Spinal Cord Society.)

Pelvic schwannomas are rare tumors that may occur either sporadically or in the context of schwannomatosis. We retrospectively reviewed the charts of patients harboring a pelvic schwannoma under conservative management or operated at our reference center between 2016 and 2023. All patients were operated by a multidisciplinary team, combining a vascular surgeon and a neurosurgeon. Twenty-four patients harboring 33 pelvic tumors were included in the cohort, including 12 patients with sporadic lesions, 2 patients with NF2-related schwannomatosis, and 10 patients with NF2-independent schwannomatosis. Multi-nodular tumors were more frequent in schwannomatosis compared to sporadic cases (p = 0.005). The mean age at diagnosis was 41 years old. Schwannomas were located on branches of the sciatic nerve (23/33, 70%), the femoral nerve (6/33, 18%), and the obturator nerve (4/33, 12%). Over the course of the study, 16 patients were operated, including 11 sporadic cases. The indication for surgery was pain (12/16, 75%) or tumor growth (4/16, 25%). Complete resection was achieved in 14 of 16 patients (87%). The mean post-operative follow-up was 37 months (range: 2-168 months). At last-follow-up, complete pain relief was achieved in all 12 patients with pre-operative pain. Post-operative morbidity included 3 long-term localized numbness and one MRC class 4 motor deficit in a multi-nodular tumor in a schwannomatosis patient. Despite its limited size, our series suggests that nerve-sparing resection of pelvic schwannomas offers satisfying rates of functional outcome both in sporadic and schwannomatosis cases, except for multi-nodular tumors., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Biliary Schwannoma is an uncommon tumor in the digestive system and extremely rare in the bile duct. We report 48 year-old male with an incidentally found in the CT, a tumor in the hepatic hilum (3.2 cm), abutting the first portion of the duodenum, without common bile duct obstruction or lymphadenopathy. The patient was asymptomatic and had normal tumor markers. The endoscopic ultrasound (EUS) demonstrated contact between the tumor, common bile duct and right hepatic artery and the biopsy suggested of mesenchymal tumor. Tumor board recommended excision of the tumor, due to the risk of malignancy and biliary obstruction. The tumor was resected through laparoscopic approach, with a Roux-en-Y hepatico-jejunostomy. The histological report showed a biliary Schwannoma (positivity for S100 and focal GFAP), which infiltrated the external wall of the bile duct, without affecting the cystic duct, surgical margins were free of neoplasia.

Objective: To observe the evolution and outcomes of postoperative trigeminal neuropathy following surgery of tumor involving the trigeminal nerve., Methods: A prospective observational study was conducted between October 2018 and February 2019 involving 25 patients with tumors confirmed to involve the trigeminal nerve during surgery by senior author. Pre- and postoperative trigeminal nerve function status and clinical data were recorded., Results: This study included 18 cases of meningioma and seven of trigeminal schwannoma. Among the meningioma cases, 55.6% of the patients reported facial sensory dysfunction before surgery, 33.3% presented ocular discomfort, and 5.6% had masticatory muscle atrophy. Postoperatively, all patients experienced facial paresthesia, 94.4% complained of eye dryness, and one (5.56%) exhibited keratitis. Additionally, one patient (5.56%) showed new-onset masticatory weakness. During follow-up, 50.0% of patients reported improvement in facial paresthesia, and one (5.56%) experienced deterioration. Eye dryness resolved in 35.3% of patients, and keratitis remission was observed in one patient. However, one patient (5.56%) developed neurotrophic keratitis. Overall, 55.6% of patients displayed mild masticatory weakness without muscle atrophy. In the cases of schwannoma, 28.6% of patients had facial paresthesia before surgery, 42.9% showed ocular discomfort, and one (14.3%) complained of masticatory dysfunction. Postoperatively, 85.7% of patients reported facial paresthesia and eye dryness, with one patient (16.7%) experiencing keratitis. During follow-up, 66.7% of patients demonstrated improvement in facial paresthesia, 28.6% showed eye dryness remission, and one patient (16.7%) recovered from keratitis. However, one patient (16.7%) developed new-onset neurotrophic keratitis. One patient (16.7%) experienced relief of masticatory dysfunction, but 42.9% reported mild deterioration. Another patient (14.3%) had facial anesthesia that had not improved., Conclusion: Postoperative trigeminal neuropathy is a common complication with a high incidence rate and poor recovery outcomes after surgery for tumors involving the trigeminal nerve. When trigeminal nerve damage is unavoidable, it is essential to provide a multidisciplinary and careful follow-up, along with active management strategy, to mitigate the more severe effects of postoperative trigeminal neuropathy., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Malignant peripheral nerve sheath tumors (MPNSTs) arising from the trigeminal nerves are extremely rare (only 45 cases, including the present case, have been published) and have been reported to develop de novo from the peripheral nerve sheath and are not transformed from a schwannoma or neurofibroma. Here, we report a case of MPNSTs of the trigeminal nerve caused by the malignant transformation of a trigeminal schwannoma, with a particular focus on genetic considerations. After undergoing a near-total resection of a histologically typical benign schwannoma, the patient presented with regrowth of the tumor 10 years after the primary excision. Histopathologic and immunochemical examinations confirmed the recurrent tumor to be an MPNST. Comprehensive genomic analyses (FoundationOne panel-based gene assay) showed that only the recurrent MPNST sample, not the initial diagnosis of schwannoma, harbored genetic mutations, including NF1-p.R2637* and TP53-p.Y234H, candidate gene mutations associated with malignant transformation. Moreover, the results of reverse transcription polymerase chain reaction showed that the fusion of SH3PXD2A and HTRA1, which has been reported as one of the responsible genetic aberrations of schwannoma, was detected in the recurrent tumor. Taken together, we could illustrate the accumulation process of gene abnormalities for developing MPNSTs from normal cells via schwannomas., (© 2023. The Author(s), under exclusive licence to The Japan Society of Brain Tumor Pathology.)

Background: Hybrid nerve sheath tumors (HNST) contain elements of more than one established sub-type of nerve sheath tumor and have been recently recognized in the 2016 WHO classification of central nervous system tumors. While common in the peripheral nerves and extracranial branches of cranial nerves, only one case has been previously documented of an intracranial HNST arising from a cranial nerve., Case Description: We describe a large, multi-compartmental intracranial hybrid nerve sheath tumor arising from the trigeminal nerve in a 22-year-old lady who presented with clinical and radiological features suggestive of a right cerebellopontine angle mass. Histopathological examination following retrosigmoid excision of the tumor revealed histological and immunohistochemical features of a schwannoma and a perineurioma., Conclusions: HNSTs are likely to be underreported in the intracranial region. The clinical course of these tumors and the reason for their occurrence in this location are not known.

Purpose of Review: Several neoplastic lesions may originate from facial nerves, including facial nerve schwannomas. These neoplastic lesions can cause various symptoms, such as facial nerve paralysis, and decrease the quality of life of patients. Therefore, knowledge of how to manage these diseases is important for otologists. However, the incidence of these diseases is extremely low, and universal management methods have not yet been established. This review summarizes recent advances in knowledge regarding these neoplastic lesions, especially facial nerve schwannomas., Recent Findings: Recent advances and the accumulation of knowledge regarding these benign facial nerve lesions have provided several preferable treatments and management methods, especially for facial nerve schwannomas. However, this still depends on the patient's symptoms and tumor localization., Summary: This review presents the optimal treatment protocol and differential diagnosis of benign facial nerve lesions. This may be useful for pretreatment differentiation and treatment decision-making., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Post-styloid parapharyngeal space tumours (PPS) have a notorious course owing to their anatomy and proximity to neurovascular bundles. Nerve injuries are usual outcomes in schwannomas. Our case is the first documented complication of contralateral hemiplegia occurring in the postoperative period in a benign PPS tumour., Case Report: A 24-year-old presented with a swelling on the left lateral aspect of the neck, diagnosed as a PPS schwannoma. He underwent transcervical excision with mandibulotomy with extracapsular dissection of the tumour. Contralateral hemiplegia, as a dreaded complication, was encountered. He was managed conservatively according to ASPECTS stroke guidelines by the critical care team. On a regular follow-up, he noticed an improvement in the lower limb with subsequent upper limb power., Conclusion: Perioperative stroke is a dreaded complication involving PPS in large benign tumours. To prevent unforeseen circumstances, necessary preoperative patient counselling and immense intraoperative care should be taken while dissecting the major vessels., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Cavernous sinus tumours comprise 0.1-0.2% of all intracranial tumours, and are most commonly meningiomas or schwannomas. Central nervous system and cranial nerve granular cell tumours (GCTs) are extremely rare. We report the tenth case of a GCT arising from a cranial nerve, and the second case reported in a cavernous sinus location, and review the literature., Clinical Presentation: A 67-year-old man presented with right sided trigeminal neuralgia. Imaging findings suggested a trigeminal schwannoma and he was treated with CyberKnife radiosurgery. Over a period of 41 months follow up, there was a progression in both symptoms and imaging findings, requiring debulking surgery. Histopathology identified a GCT., Conclusions: This is the first case of a cranial nerve GCT treated with stereotactic radiosurgery. Trigeminal nerve GCTs are a rare differential in cases of presumed schwannomas.

Purpose: We present a prospective case series that aimed to report the functional (voice and swallowing) outcomes of delayed laryngeal reinnervation following vagal interruption by resection of vagal paraganglioma and schwannoma., Materials and Methods: A dedicated, anonymized database was established in 2012 with a minimum eighteen-month follow up set for this report. Internationally validated self- and observer-reported measures were recorded preoperatively and at six, 12 and, 18 months together with demographics, diagnoses, and operative details., Results: A total of eight patients with a median age of 46 (37-54) underwent excision of vagal paraganglioma (five) and schwannoma (three) with few mild complications. Three underwent selective and five non selective reinnervation. Seven out of eight patients underwent synchronous injection medialization. The voice handicap index (VHI-30) improved from a baseline median 83 (range 52-102) to 7.5 (5-58) at 18 months; maximum phonation time improved from median 8 (range 5-15) to 10.5 (8.5-11); voice grade ("G" in grade, roughness, breathiness, asthenia, and strain [GRBAS] scoring) improved from median three (severe impairment, range 0-3) to one (mild impairment, 0-2); Eating Assessment Tool (EAT-10) score improved from median 12 (range 3.5-27) preoperatively to one (0-16); and reflux symptom index (RSI) improved from median 25 (range 17-36) to 7 (0-36). One patient exhibited no discernible reinnervation, while the remainder exhibited good cord bulk and tone, though without purposive abduction., Conclusion: Delayed laryngeal reinnervation for high vagal paralysis is a safe technique associated with good voice and swallowing outcomes by 12-18 months. Potential confounders in this small series and the absence of a control arm both limit conclusions, but this study suggests that further prospective, controlled studies, and/or case registration are merited., (Copyright © 2021 The Voice Foundation. Published by Elsevier Inc. All rights reserved.)

Orbital schwannoma is an exceptionally rare cause of ptosis. Diagnosis may be elusive given its slow rate of growth and its various presentations depending on localization. Herein, we report the case of a 50-year-old male who presented to our clinic with a complaint of unilateral, recurrent ptosis of the left eye. He underwent levator palpebrae resection, which was unsuccessful at improving his ptosis. He later represented with acute-onset diplopia for which magnetic resonance imaging was obtained. Magnetic resonance imaging showed a lesion in the superior orbit with secondary bony dehiscence of the orbital roof. Through a vertical lid-split incision, the lesion was removed, and the frontal lobe was observed protruding through the defect in the orbital roof. This case highlights the importance of diagnostic skepticism in the face of recurrent ptosis and emphasizes the utility of the vertical lid-split approach for anterior lesions of the superior orbit., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Background: Microsurgical resection of vestibular schwannoma (VS) is highly challenging, especially because surgical treatment nowadays is mainly reserved for larger (Koos grade 3 and 4) tumors., Objective: To assess the performance of three-dimensional exoscope use in VS resection in comparison with the operative microscope., Methods: Duration of surgery and clinical and radiological results were collected for 13 consecutive exoscopic schwannoma surgeries. Results were compared with 26 preceding microsurgical resections after acknowledging similar surgical complexity between groups by assessment of tumor size (maximum diameter and Koos grade), the presence of meatal extension or cystic components, and preoperative hearing and facial nerve function., Results: Total duration of surgery was comparable between microscopically and exoscopically operated patients (264 minutes ± 92 vs 231 minutes ± 84, respectively; P = .276). However, operative time gradually decreased in consecutive exoscopic cases and in a multiple regression model predicting duration of surgery, and exoscope use was associated with a reduction of 58.5 minutes (95% CI -106.3 to -10.6; P = .018). Tumor size was identified as the main determinant of duration of surgery (regression coefficient = 5.50, 95% CI 3.20-7.80) along meatal extension and the presence of cystic components. No differences in postoperative hearing preservation and facial nerve function were noted between the exoscope and the microscope., Conclusion: Resection of VS using a foot switch-operated three-dimensional exoscope is safe and leads to comparable clinical and radiological results as resection with the operative microscope., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

We report a case of a man in his early 50 s who was admitted to our hospital for anorexia. An imaging examination led to a preoperative diagnosis of a gastrointestinal stromal tumor and gallbladder stones. He underwent treatment by laparoscopic cholecystectomy and distal partial gastrectomy with lymph node dissection. The final histopathological diagnosis was gastric schwannoma and tubular adenoma of the gallbladder. Gastric schwannoma accounts for only 0.2% of all gastric tumors, and tubular adenoma constitutes only 2.2% of gallbladder tumors. This report describes the diagnosis and treatment process for this combination of tumors, thus providing a reference for similar cases.

Rationale: Ultrasound-guided brachial plexus block is a common anesthetic procedure used in upper extremity surgery. However, it may not be a suitable option for some patients., Patient Concerns: A 17-year-old woman with the left palmar schwannoma scheduled for surgical treatment received ultrasound-guided brachial plexus block. The anesthesia modalities of the disease were discussed., Diagnoses: Based on the patient's complaints and clinical appearance, provisional diagnosis of neurofibroma was considered., Interventions: In this case, we present a case of ultrasound-guided axillary brachial plexus block used for upper extremity surgery in this patient. It was not easily and painlessly reduced in the surgery, although the visual analogue scale score was 0 and no motor movements of the left arm and palm were observed. The pain was relieved by intravenous injection of 50 mcg remifentanil., Outcomes: Immunohistochemically labeled pathological examination confirmed the mass to be a schwannoma. There was no need to apply additional analgesia after surgery, although the patient felt numbness in the left thumb for 3 days follow up., Lessons: Even if there is painless when skin-cutting after implementation of brachial plexus block, the patient is painful when pulls the nerve around the tumor during excision. It is necessary to give an analgesic drug or anesthetize a single terminal nerve as a supplement for brachial plexus block in patients with schwannoma., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Foster Kennedy syndrome refers to a finding of optic atrophy in one eye from direct compression of the optic nerve by a mass lesion and contralateral papilledema in the nonatrophic optic nerve caused by an increased intracranial pressure. When the fundoscopy finding is not due to a direct compressive mass, the term pseudo-Foster Kennedy syndrome is used; this can be caused by any process or processes that result in optic atrophy in one eye and optic disc edema in the other. Identifying Foster Kennedy syndrome in a patient calls for expedient neuroimaging looking for an intracranial mass lesion. In this article, we present the case of a patient presenting with vision loss and Foster Kennedy syndrome who was found to have a large trigeminal cystic schwannoma. While several other accompanying symptoms were not evident from the patient complaint, a careful history and physical examination revealed additional localizing clues: unilateral sensory changes in the face and pterygoid and masseter atrophy, unilateral cranial nerve VI palsy, and episodes of intense déjà vu sensation, which were presumed to represent temporal lobe-onset focal aware seizures. Trigeminal schwannomas are a rare entity, and they are even more rarely cystic. This case highlights an unusual scenario where a slow expansion of the tumor ultimately resulted in vision loss and presentation of the patient to medical attention., (© 2022 American Academy of Neurology.)

The present paper discusses the diagnostic challenges we faced in a 60-year-old woman with a history of schizophrenia, presenting with left unilateral facial pain for the past three months. Based on the elaborate clinical examination and diagnostic nerve blocks, the patient was diagnosed with trigeminal neuralgia (TN) and non-surgical therapy commenced. Further investigations with magnetic resonance imaging (MRI) and ultrasound-guided fine needle aspiration cytology (FNAC) revealed the presence of an extracranial schwannoma involving a branch of the maxillary nerve. The patient was symptomatically relieved after surgical excision of the benign tumor under general anesthesia. Hence, we emphasize the need for special care and attention in psychiatric patients presenting with orofacial pain., (© 2022 Special Care Dentistry Association and Wiley Periodicals LLC.)

Background: Neurofibromatosis type 2 (NF2) is rare genetic disorder mainly characterized by the development of central nervous system lesions, but peripheral nerve pathology may also cause high morbidity including pain, motor, and sensory loss., Objective: To describe the tumor burden of patients with peripheral nerve pathology in NF2 including peripheral neuropathies and schwannomas and the results of surgery in the latter group., Methods: We conducted a retrospective chart review of all patients with NF2 followed up at our NF2 Reference Center to include all patients suffering from peripheral nerve pathology. Tumor detection relied on focal MRIs based on symptoms., Results: Thirty-four patients harboring 105 peripheral nerve schwannomas and 1 perineurioma were included. Schwannomas were mainly located in major nerves (n = 74, 71%) compared with subcutaneous (n = 23, 22%) and intramuscular (n = 8, 7%) cases. Most schwannomas (81/90-90%) were classical discrete tumors while multinodular cases represented only 9 cases (10%). During follow-up, 63 (60%) tumors were operated in 24 patients, including 39 schwannomas of major nerves. A complete resection was achieved in most of the cases (52/63, 83%) with a complete relief of preoperative pain in most patients (57/60, 95%). Persistent motor deficits (5/39, 13%) were mostly encountered in patients operated from multinodular schwannomas (4/5, 80%). Six patients had an associated peripheral neuropathy with 5 cases of pseudo-Charcot-Marie-Tooth-associated amyotrophy., Conclusion: Surgery remains a safe and effective method of treating peripheral nerve schwannoma-associated pain in NF2, with the exception of rare multinodular tumors. Special attention should be drawn to patients harboring severely debilitating neuropathies and perineuriomas., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)