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Esophageal subepithelial lesions (ESELs) encompass a variety of diseases, including leiomyoma, granular cell tumors, hemangioma, lipoma, stromal tumors, leiomyosarcoma, schwannoma, neuroendocrine tumors and more. These lesions often present asymptomatically, leading to a generally low clinical diagnosis rate. Common imaging techniques for diagnosing ESELs include conventional endoscopy, spiral computed tomography, and endoscopic ultrasound (EUS). Among these, EUS is currently regarded as one of the most accurate methods for diagnosing ESELs. In recent years, EUS has increasingly been combined with advanced technologies such as artificial intelligence, submucosal saline injection, high-frequency impedance measurement, and enhanced imaging to improve diagnostic accuracy and reduce missed diagnoses. This article reviews the application and recent advancements of EUS in diagnosing esophageal submucosal lesions., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Schwannomas are common peripheral nerve tumors originating from Schwann cells, primarily occurring in the head and neck, limbs, and trunk. Schwannomas occurring in the mesocolon are rare and often have no specific manifestations. Abdominal schwannomas need to be differentiated from common abdominal tumors such as gastrointestinal stromal tumors., Patient Concerns: We report a case of a mesocolic schwannoma in a 59-year-old female presenting with gastrointestinal symptoms of acid reflux. At an outside hospital, gastroscopy, colonoscopy, and abdominal computed tomography scans revealed a soft tissue mass adjacent to the greater curvature of the stomach, leading to a suspicion of a gastric mesenchymal tumor., Diagnoses: Mesocolic schwannoma., Interventions: Laparoscopy was performed at our hospital. Intraoperatively, the tumor was found to be closely related to the transverse colon and was initially diagnosed as a mass originating from the transverse colon. Consequently, a resection of the mass along with the adherent portion of the transverse colon was performed. Postoperative pathology and immunohistochemistry confirmed that the tumor was a schwannoma of the mesentery and did not originate from the transverse colon., Outcomes and Lessons: Schwannomas can be distinguished from gastrointestinal stromal tumors by immunohistochemical staining, and surgical treatment is effective for benign schwannomas., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

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.)

Objective: Trigeminal schwannoma (TS), though a rare and benign tumor, becomes a significant surgical challenge due to its intricate location. This study aims to detail the long-term functional outcomes and tumor control post-surgical resection., Method: We analyzed a multicentric retrospective cohort of 39 patients operated on for a TS in five tertiary centers between January 1993 and July 2022., Results: Six TS (15%) were in the middle fossa (type M), two (5%) in the posterior fossa (type P), and two (5%) were extracranial (type E). Twenty-nine (75%) were Dumbbell shape: Eighteen (47%) were MP type, seven (18%) were MPE type, and four (10%) were ME type. Fifth nerve symptoms were the foremost preoperative complaint: hypesthesia (51%), trigeminal neuralgia (36%), and paresthesia (30%). We report a favorable evolution course for 61% of preexisting deficits (half of patients with preoperative paresthesia and neuralgia improved while only 5% of preoperative hypesthesia improved). Postoperative hypesthesia was the most frequent de novo deficit 14 (74%) and resolved in solely half the cases. Various approaches were used according to tumor type. Gross total resection (GTR), Subtotal resection (STR), and partial resection (PR) were achieved in respectively 33% (N = 13), 10% (N = 4), and 56% (N = 22) of patients. The mean clinical and radiological FU was 63 months (12 - 283 months). GTR led to no sign of recurrence (mean FU: 60 months - range: 12-283 months). For STR or PR (67%): 23 (88%) were assigned to a Wait-&-rescan policy (WS group) which offered stability in 70% (N = 16). Three cases (8%) underwent a complementary GKS (GammaKnife) on the residual lesion (GK group) without tumor change., Conclusion: For large TS, the completeness of resection must consider the potential functional burden of surgery. With giant infiltrating lesions, a strategy of planned subtotal resection, complemented by radiosurgery, either complementary or uppon regrowth, may provide similar oncological outcomes., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

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.)

Schwannomas consist of both high-cellularity regions (Antoni A area) and hypocellular regions (Antoni B area) in histopathological findings. Neurofibromas characteristically consist of CD34 positive spindle cells with thin, wavy, nuclei and wavy collagen bands. Previous reports have described segments of schwannomas with neurofibroma features as hybrid tumors, although hybrid tumors were diagnosed based on partial CD34 positivity in many previous reports. On the other hand, the Antoni B area of some schwannomas was reported to be positive for CD34. Therefore, the definition of a hybrid tumor has not been clear. The objective of this study was to determine whether only CD34 positive findings in schwannomas could be used to define a hybrid tumor. In the analysis of our patient with schwannomatosis caused by a novel LZTR1 germline mutation, part of the tumor had CD34 positive hypocellular regions. These regions contained no thin, wavy, nuclei, indicating an Antoni B area. Laser microdissection was used to investigate the genetic background and differences in molecular mechanisms between CD34 positive and CD34 negative regions. All mutations identified in CD34 positive regions were also found in CD34 negative regions. Our data could not clear the genetic background of Antoni B which was CD34 positive area. We then reviewed the pathologies of 66 sporadic schwannomas. Histopathological examinations of all schwannomas revealed the absence of thin, wavy, nuclei and wavy collagen bands, and no hybrid tumors were found in any of the cases. Ten of 66 patients were randomly selected for CD34 immunostaining and positivity was found in all cases. Our data suggest that it is difficult to distinguish schwannomas by staining for CD34 alone, as Antoni B areas can also be positive for CD34. Therefore, CD34 staining alone should not be used to diagnose hybrid tumors in patients with schwannomas., (© 2024 Japanese Dermatological Association.)

Schwannomas are benign tumors of the peripheral nervous system arising from the transformation of Schwann cells (SCs). On the whole, these tumors are related to alterations of the neurofibromin type 2 gene, coding for the oncosuppressor merlin, a cytoskeleton-associated protein belonging to the ezrin-radixin-moesin family. However, the underlying mechanisms of schwannoma onset and progression are not fully elucidated, whereas one of the challenges might be the environment. In this light, the exposure to electromagnetic field (EMF), generated by the use of common electrical devices, has been defiantly suggested as the cause of SCs transformation even if the evidence was mostly epidemiologic. Indeed, insubstantial mechanisms have been so far identified to explain SCs oncotransformation. Recently, some in vitro evidence pointed out alterations in proliferation and migration abilities in SCs exposed to EMF (0.1 T, 50 Hz, 10 min). Here, we used the same experimental paradigma to discuss the involvement of putative epigenetic mechanisms in SCs adaptation to EMF and to explain the occurrence of hypoxic alterations after the exposure. Our findings indicate a set of environmental-induced changes in SCs, toward a less-physiological state, which may be pathologically relevant for the SCs differentiation and the schwannoma development., (© 2024 The Author(s). Journal of Cellular Physiology published by Wiley Periodicals LLC.)

Purpose: Pathogenic LZTR1 variants cause schwannomatosis and dominant/recessive Noonan syndrome (NS). We aim to establish an association between heterozygous loss-of-function LZTR1 alleles and isolated multiple café-au-lait macules (CaLMs)., Methods: A total of 849 unrelated participants with multiple CaLMs, lacking pathogenic/likely pathogenic NF1 and SPRED1 variants, underwent RASopathy gene panel sequencing. Data on 125 individuals with heterozygous LZTR1 variants were collected for characterizing their clinical features and the associated molecular spectrum. In vitro functional assessment was performed on a representative panel of missense variants and small in-frame deletions., Results: Analysis revealed heterozygous LZTR1 variants in 6.0% (51/849) of participants, exceeding the general population prevalence. LZTR1-related CaLMs varied in number, displayed sharp or irregular borders, and were generally isolated but occasionally associated with features recurring in RASopathies. In 2 families, CaLMs and schwannomas co-occurred. The molecular spectrum mainly consisted of truncating variants, indicating loss-of-function. These variants substantially overlapped with those occurring in schwannomatosis and recessive NS. Functional characterization showed accelerated protein degradation or mislocalization, and failure to downregulate mitogen-activated protein kinase signaling., Conclusion: Our findings expand the phenotypic variability associated with LZTR1 variants, which, in addition to conferring susceptibility to schwannomatosis and causing dominant and recessive NS, occur in individuals with isolated multiple CaLMs., Competing Interests: Conflict of Interest The authors declare no conflicts of interest., (Copyright © 2024 American College of Medical Genetics and Genomics. Published by Elsevier Inc. All rights reserved.)

Purpose: This study aimed to investigate whether multiparametric magnetic resonance imaging (MRI) including dynamic contrast-enhanced (DCE) and diffusion weighted (DW) MRI can differentiate pleomorphic adenoma (PA) from schwannoma in the parapharyngeal space., Methods: Forty-six patients with pathologically proven PAs and 47 schwannomas in the parapharyngeal space were enrolled. All patients underwent conventional MRI, and DW-MRI and DCE-MRI were performed in 30 and 33 patients, respectively. Fisher's exact, Mann-Whitney-U tests and Independent samples t-test were used to compare variables between PAs and schwannomas. Multivariate logistic regression analysis was used to examine the diagnostic performance of MRI parameters., Results: The PAs usually show lobulation sign, posterior displacement of ICA and attached to the parotid gland deep leaf, while bird beak configuration, anterior displacement of ICA and involvement of foramen jugular were more commonly seen in the schwannomas(all p < 0.001). The washout rate of PAs was found to be higher than that of schwannomas (p = 0.035), whereas no significance was found in the other DCE-MRI parameters and in ADCs(p > 0.05). Using a combination of conventional MRI features including lobulation sign, bird beak configuration, direction of internal carotid artery(ICA) displacement and attached to the parotid gland in multivariate logistic regression analysis, sensitivity, specificity, and accuracy in differential diagnosis of PAs and schwannomas were 97.8%, 91.5% and 94.6%, respectively., Conclusion: Conventional MRI can effectively differentiate PAs from schwannomas in the parapharyngeal space with a high diagnostic accuracy. The DCE-MRI and DWI have limited added diagnostic value to conventional MRI in the differential diagnosis., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Giant extradural thoracic schwannomas are very rare tumors in the pediatric age group and often occur together with neurofibromatosis. Giant schwannomas span across more than two vertebral segments and have an extraspinal extension of over 2.5 cm. In this case, we report on a 5-year-old boy with a purely extradural giant schwannoma without accompanying neurofibromatosis., Clinical Presentation: A 5-year-old male patient was admitted to the orthopedics and traumatology outpatient clinic with complaints of difficulty in walking following waist and left leg pain after falling from a chair. Contrast-enhanced spinal MRI and cranial MRI showed an extradural spinal lesion measuring 22 × 18 × 35 mm that pushed the spinal cord to the right at the T10-12 level and extended into the left foramen at the T11-12 level. The patient was operated. The tumor was removed completely by performing bilateral laminoplasty at the T10-11-12 levels. Histopathology result reported schwannoma., Conclusion: Giant schwannomas are slow-growing tumors that rarely occur in childhood. In these patients, spinal traumas can lead to serious neurological deficits. Early diagnosis and successful surgery can prevent permanent neurological damage., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Inroduction: Schwannomas are the most common tumors of the peripheral nervous system. Surgical eradication with excision or enucleation is the treatment for symptomatic and large schwannomas., (This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Case: A 28-year-old man presented for a painful lower extremity mass. Imaging revealed a nonspecific, poorly defined lucent lesion in the left distal tibial cortex with scalloping. The diagnosis of intracortical schwannoma was made after open biopsy revealed positive S-100 immunohistochemical staining and characteristic spindled cells. Definitive management was achieved through curettage and bone grafting. Six months postoperatively, the patient's pain had improved with complete radiographic healing., Conclusion: An intracortical schwannoma is a rare, benign lesion which may have atypical radiological findings and nonspecific presentations. Definitive diagnosis is made with permanent histology and S-100 immunohistochemical staining., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/C485)., (Copyright © 2024 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: Most schwannomas are isolated tumours occurring in otherwise healthy people. However, bilateral vestibular schwannomas (BVS) or multiple non-vestibular schwannomas indicate an underlying genetic predisposition. This is most commonly NF2 -related schwannomatosis (SWN), but when BVS are absent, this can also indicate SMARCB1 -related or LZTR1 -related SWN., Methods: We assessed the variant detection rates for the three major SWN genes ( NF2 , LZTR1 and SMARCB1 ) in 154 people, from 150 families, who had at least one non-vestibular schwannoma, but who did not meet clinical criteria for NF2 -related SWN at the time of genetic testing., Results: We found that 17 (11%) people from 13 families had a germline SMARCB1 variant and 19 (12%) unrelated individuals had a germline LZTR1 variant. 19 people had an NF2 variant, but 18 of these were mosaic and 17 were only detected when 2 tumours were available for testing. The overall detection rate was 25% using blood alone, but increased to 36% when tumour analysis was included. Another 12 people had a germline variant of uncertain significance (VUS)., Conclusions: There were similar proportions of LZTR1 , SMARCB1 or mosaic NF2 . However, since an NF2 variant was detected in tumours from 103 people, it is likely that further cases of mosaicism would be detected if more people had additional tumours available for analysis. In addition, if further evidence becomes available to show that the VUSs are pathogenic, this would significantly increase the proportion of people with a genetic diagnosis. Our results indicate the importance of comprehensive genetic testing and improved variant classification., Competing Interests: Competing interests: GE has received consultancy fees from AstraZeneca, Springworks and Everything Genetic., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Nerve tumors in the retroperitoneal space are a rarity. Radical surgery according to soft tissue tumors can lead to persistent pain and neurological deficits. This study aims to evaluate clinical outcomes of patients treated by a visceral- / neurosurgical approach. 33 patients with a retroperitoneal nerve tumor underwent surgery between 01/2002 and 12/2022 at our department. A visceral surgeon provided access to the retroperitoneal space, followed by micro-neurosurgical tumor preparation under neuromonitoring. Clinical examination and MRI were performed 12 weeks after surgery and further 3 months (WHO grade > 1) or 12 months (WHO grade 1). Further examinations were based on MRI findings and residual symptoms with median follow-up time of 24 months. One patient was treated for two distinct masses resulting in a total of 34 histological findings. Schwannomas (n = 15; 44.1%) and neurofibromas (n = 10; 29.4%) were the most common tumors. Long-term improvements were noted in radicular pain (15/18 patients; 83.3%), motor deficits (7/16 patients; 43.8%), abdominal discomfort and pain (5/7 patients; 71.4%). Recurrences were observed in 3/33 (9,1%) patients. This study represents the largest series of retroperitoneal BPNSTs treated with microsurgical techniques. Prospective multicenter studies are warranted to establish standardized treatment guidelines., (© 2024. The Author(s).)

Background: Large mediastinum tumors invading the thoracic outlet have consistently been a challenge in thoracic surgery. Due to the large size of the tumor and its proximity to many important tissues, appropriate surgical approaches are crucial for a successful surgery., Case Presentations: Here, we present a case of a large neurilemmoma that invaded the thoracic outlet that was resected by a supraclavicular-median sternotomy approach. The case was a 58-year-old woman with a large mass in the right chest cavity that had invaded the thoracic outlet. The preoperative biopsy showed a blood clot with a few fibrous connective tissues covered by a single layer of flat epithelium. There was insufficient evidence to diagnose the mass as a tumor, and imaging examinations suggested a diagnosis of solitary pleural fibroma. For good exposure of the cranial and caudal aspects of the large mass, we devised a median sternotomy combined with a supraclavicular approach and safely achieved complete resection. The patient recovered well and experienced no severe complications or functional restrictions of the upper extremity. The postoperative pathology diagnosis was a neurilemmoma., Conclusions: The supraclavicular-median sternotomy approach could be an optional approach for the complete resection of large mediastinal tumors invading the thoracic outlet., (© 2024. The Author(s).)

Objective: Schwannomas are benign, slow-growing tumors originating from Schwann cells in peripheral nerves, commonly affecting the median and ulnar nerves in the forearm and wrist. Surgical excision is the gold standard treatment. This study presents our treatment strategies and outcomes for large-sized ulnar and median nerve schwannomas at the forearm and wrist level., Methods: From 2012 to 2023, we enrolled 15 patients with schwannomas over 2 cm in size in the median or ulnar nerve at the forearm and wrist. The study included 12 patients with median nerve schwannomas (mean age: 61 years) and 3 with ulnar nerve schwannomas (mean age: 68 years), with a mean follow-up of 26.9 months., Results: After surgery, all patients with median nerve schwannomas experienced mild, transient numbness affecting fewer than two digits, resolving within six months without motor deficits. Ulnar nerve schwannoma excision caused mild numbness in two patients, also resolving within six months, but all three developed ulnar claw hand deformity, which persisted but improved at the last follow-up. Despite this, patients were satisfied with the surgery due to relief from severe tingling pain., Conclusions: Schwannomas of the median, ulnar, and other peripheral nerves should be removed by carefully dissecting the connecting nerve fascicles to avoid injury to healthy ones. Sensory deficits may occur but are unlikely to significantly impact quality of life. However, in motor-dominant nerves like the ulnar nerve, there is a risk of significant motor deficits that could affect hand function, though not completely. Therefore, thorough preoperative discussion and consideration of interfascicular nerve grafting are essential., (© 2024. The Author(s).)

Rectal Schwannomas are exceptionally uncommon tumors that arise from the peripheral nerve plexus. These neoplasms are incidentally discovered as submucosal tumor-like lesions during routine colonoscopy in individuals without symptoms. Surgical resection accompanied by biopsy is considered the standard approach for diagnosis and treatment. We describe here the first successful utilization of submucosal tunneling endoscopic resection (STER) technique for removal of a rare rectal Schwannoma. A 59-year-old asymptomatic female was found to have a 15 × 12 mm submucosal tumor during colonoscopy. Submucosal tunneling endoscopic resection achieved en bloc resection and pathology confirmed Schwannoma. The patient was discharged uneventfully with no adverse events. This study highlights the efficacy and safety of STER for removing rare rectal Schwannomas., 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.

Summary: Schwannomas are benign tumours of peripheral nerves originating in the nerve sheaths. Only 1% are reported in the oral cavity. We report a 24-year-old female who presented with a 10-year history of a mass at the base of the tongue with associated odynodysphagia, referred otalgia and loss of weight. Oral examination revealed a large left sided base of tongue mass extending into the vallecula. An open transcervical suprahyoid resection was performed with a good outcome., (Copyright© Authors.)

Objectives: Describe a single institution's cochlear implant outcomes for patients with inner ear schwannomas (IES) in the setting of various tumor management strategies (observation, surgical resection, or stereotactic radiosurgery [SRS])., Study Design: Single-institution retrospective review., Patients: Patients diagnosed with isolated, sporadic IES who underwent cochlear implantation (CI)., Interventions: CI with or without IES treatment., Main Outcome Measures: Speech perception outcomes, tumor status., Results: Twelve patients with IES underwent CI with a median audiologic and radiologic follow-up of 12 months. Six patients underwent complete resection of the tumor at the time of CI, four underwent tumor observation, and two underwent SRS before CI. At 1 year after CI for all patients, the median consonant-nucleus-consonant (CNC) word score was 55% (interquartile range, 44-73%), and the median AzBio sentence in quiet score was 77% (interquartile range, 68-93%). Overall, those with surgical resection performed similarly to those with tumor observation (CNC 58 versus 61%; AzBio in quiet 74 versus 91%, respectively). Patients who underwent tumor resection before implantation had a wider range of speech performance outcomes compared with patients who underwent tumor observation. Two patients had SRS treatment before CI (10 months previous and same-day as CI) with CNC word scores of 6 and 40%, respectively., Conclusions: Patients with IES who underwent CI demonstrated similar speech performance outcomes (CNC 56% and AzBio 82%), when compared with the general cochlear implant population. Patients who underwent either tumor observation or surgical resection performed well after CI., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Meningioma and schwannoma are common tumours of the nervous system. They occur sporadically or as part of the hereditary NF2-related schwannomatosis syndrome. There is an unmet need for new effective drug treatments for both tumour types. In this paper, we demonstrate overexpression/activation of TAM (TYRO3/AXL/MERTK) receptors (TAMs) and overexpression/release of ligand GAS6 in patient-derived meningioma tumour cells and tissue. For the first time, we reveal the formation of MERTK/TYRO3 heterocomplexes in meningioma and schwannoma tissue. We demonstrate the dependence of AXL and TYRO3 expression on MERTK in both tumour types, as well as interdependency of MERTK and AXL expression in meningioma. We show that MERTK and AXL contribute to increased proliferation and survival of meningioma and schwannoma cells, which we inhibited in vitro using the MERTK/FLT3 inhibitor UNC2025 and the AXL inhibitor BGB324. UNC2025 was effective in both tumour types with superior efficacy over BGB324. Finally, we found that TAMs are expressed by tumour-associated macrophages in meningioma and schwannoma tumours and that UNC2025 strongly depleted macrophages in both tumour types., (© 2024. he Authors.)

Objective: Trigeminal schwannomas are rare benign tumors originating from the Schwann cells of the trigeminal nerve. Despite the common occurrence of trigeminal neuropathy in trigeminal schwannomas, a detailed analysis has not yet been performed because of the rarity of this disease. This study aimed to analyze trigeminal neuropathy in trigeminal schwannoma resection and identify the risk factors for postoperative worsening of trigeminal neuropathy., Methods: A retrospective analysis of 86 surgical cases was performed at our institution between 1975 and 2018. Obtained parameters included age, sex, diagnosis, reoperation, tumor size, tumor location, presence or absence of cysts, surgical approach, degree of tumor removal, and pre/postoperative trigeminal neuropathy. Uni- and multivariate analyses were performed to identify the risk factors for worsening postoperative sensory disturbances., Results: Of 83 patients, 58.1% had preoperative trigeminal neuropathy. Postoperative sensory disturbance occurred in 27.9%, with worsening in two cases and de novo symptoms in 22 cases. Regarding risk factors for worsening postoperative sensory disturbances, older age, smaller tumor size, middle and posterior (MP) type, gross total removal (GTR), and anterior transpetrosal approach were identified in the univariate analysis, while MP type and GTR were identified in the multivariate analysis., Conclusions: This study analyzed trigeminal neuropathy in trigeminal schwannomas in detail and identified tumor location and removal rate as risk factors for worsening postoperative sensory disturbances. Treatment strategies to reduce the risk of trigeminal neuropathy should be considered., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

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).)

Leiomyomas and schwannomas are both types of rare benign soft tissue tumours. Leiomyomas are more commonly found in the lower limbs than in the upper extremities, while schwannomas are rare peripheral nerve sheath tumours that can occur in different anatomical regions. However, they rarely occur in the saphenous nerve. This case study presents a 41-year-old female patient with a solitary mass lesion located deep in the soft tissue of the anteromedial lower extremity. The physical examination revealed a palpable, elastic-hard, mobile and non-tender mass. Magnetic resonance imaging (MRI) showed an oval-shaped subcutaneous mass on contrast-enhanced T1-weighted sections. The initial MRI images suggested a schwannoma, but the tumour was later confirmed to be a leiomyoma after total enucleation. An immunohistochemical study was performed for differential diagnosis. Solitary mass lesions in the lower extremities can be mistaken for various types of tumours and misdiagnosed and require histopathological examination and good radiological imaging for differential diagnosis. Complete surgical excision is usually a safe and effective treatment for leiomyomas., Competing Interests: The authors have no conflict of interest.

Neurofibromatosis type 2 (NF2) is a rare disorder that causes vestibular schwannomas (VS), meningiomas and ependymomas. To date, there is no FDA approved drug-based treatment for NF2. We have previously identified that BET inhibition can selectively reduce growth of the NF2-null schwannoma and Schwann cells in vitro and tumorigenesis in vivo and, separately, reported that inhibition of Focal Adhesion Kinase 1 (FAK1) via crizotinib has antiproliferative effects in NF2-null Schwann cells. The current study was aimed at determining whether combined BET and FAK inhibition can synergize and to identify the mechanisms of action. A panel of normal and NF2-null Schwann and schwannoma cell lines were used to characterize the effects of combined BET and FAK inhibition in vitro and in vivo using pharmacological and genetic approaches. The mechanism of action was explored by chromatin immunoprecipitation, ChIP-PCR, western blotting, and functional approaches. We find that combined BET and FAK inhibition are synergistic and inhibit the proliferation of NF2-null schwannoma and Schwann cell lines in vitro and in vivo, by arresting cells in the G1/S and G2/M phases of the cell cycle. Further, we identify the mechanism of action through the downregulation of FAK1 transcription by BET inhibition, which potentiates inhibition of FAK by 100-fold. Our findings suggest that combined targeting of BET and FAK1 may offer a potential therapeutic option for the treatment of NF2-related schwannomas., (© 2024. The Author(s), under exclusive licence to Springer Nature Limited.)

Background: A schwannoma is a nerve sheath tumor that is formed by Schwann cells. Vestibular schwannomas are thought to account for the majority of intracranial schwannomas. Nonvestibular schwannomas account for about 10%, about half of which are trigeminal schwannomas. Multiple intracranial schwannomas originating from different cranial nerves are extremely rare., Methods: We describe the clinical case of a 42-year-old female patient with vestibular schwannoma and multiple trigeminal schwannomas., Results: That case shows how multiple trigeminal schwannomas were identified intraoperatively during elective surgery for vestibular schwannoma removal, most of which were resected. No new neurological deficits were observed in the patient., Conclusions: The presence of multiple intracranial schwannomas is extremely rare in neurosurgical practice and can change the intraoperative strategy and the course of the surgery., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Abstract: We report a rare case of cellular schwannoma (CS) manifesting as an ulcerated nodular lesion, mimicking spindle cell melanoma on the sole of the foot. CS, a benign variant of schwannoma, typically occurs in deep soft tissues but can rarely present cutaneously. The diagnosis of CS heavily relies on histopathological examination and immunohistochemical staining for specific markers such as SOX10 and S100. In this case, initial clinical suspicion of nodular melanoma was confirmed on biopsy, which revealed a spindle cell neoplasm positive for SOX10 and negative for melanocytic markers. Misdiagnosis of nodular melanoma was averted through complete excision. CS diagnosis demands careful consideration due to its resemblance to other spindle cell neoplasms, especially melanoma. Meticulous histopathological evaluation and immunostaining are important to differentiate CS from similar lesions, ensuring accurate diagnosis and appropriate management. This report contributes valuable insights into the diagnostic challenges and management of CS, particularly in unusual cutaneous presentations., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Abstract: Schwannoma is a benign tumor originating from Schwann cells. It commonly occurs in the head, neck, and extremities, but rarely occurs in the trachea. Tracheal schwannoma is usually asymptomatic. We reported the 18 F-FDG PET/CT findings of a 61-year-old man with bronchoscopically biopsy-proven schwannoma, which presented challenges in differentiation from certain benign tumors and low-grade malignancies in the trachea., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose: Facial nerve schwannomas (FNSs) are rare intracranial tumors, and the optimal management of these tumors remains unclear. We investigated the long-term follow-up results of FNS with good facial nerve function., Methods: At nine medical centers in the Korean Facial Nerve Study Group, 43 patients undergoing observation periods longer than 12 months for FNS with good facial nerve function (House-Brackmann grade ≤ II) were enrolled, and clinical and radiographic data were obtained for these cases., Results: The mean follow-up period was 63 months. In the majority of cases, tumors involved multiple segments (81.4%) and only eight cases were confined to a single site. There were no cases where the tumor was confined to the extratemporal region. Tumor size increased slightly, with an average estimated change of 0.48 mm/year. Twenty (46.5%) of 43 patients showed no change in tumor size. Seven patients (16.3%) showed worsening House-Brackmann (H-B) grade, of which two patients deteriorated from H-B grade I to II, four worsened to grade III, and one deteriorated to grade IV. The remaining 36 patients (83.7%) showed no change in facial nerve function. There was no difference in H-B grade according to tumor size at the time of diagnosis or change in tumor size., Conclusion: We conducted a large-scale observational study of FNS with good facial nerve function. Our study showed that many patients maintained facial nerve function during long-term follow-up. Conservative management with regular examination and imaging can be an appropriate option for managing FNS with good facial nerve function., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The 2021 World Health Organization Classification of Central Nervous System Tumours introduced significant revisions to the categorization of paraspinal and nerve sheath tumours. This updated system encompasses seven tumour types: schwannoma, neurofibroma, perineurioma, hybrid nerve sheath tumours, malignant melanotic nerve sheath tumour, malignant peripheral nerve sheath tumour and cauda equina neuroendocrine tumour. This review provides an image-rich cytologic reference of these tumours, with particular emphasis on intraoperative smear preparations. Knowledge of the key cytological features of these tumours and their differential diagnoses will help guide classification of these challenging entities., (© 2023 John Wiley & Sons Ltd.)

Initially described as a highly specific immunohistochemical marker for carcinomas of mammary origin, trichorhinophalangeal syndrome type 1 (TRPS1) has subsequently been detected in a variety of other non-mammary tumors. In this study, we examined the immunohistochemical expression of TRPS1 in 114 peripheral nerve sheath tumors, including 43 malignant peripheral nerve sheath tumors (MPNSTs), 58 schwannomas, including 9 cellular neurofibromas, and 13 neurofibromas, including 1 atypical neurofibroma. Notably, TRPS1 was expressed in 49% of MPNSTs and was absent in all schwannomas and neurofibromas. All MPNSTs showed TRPS1 labeling in >50% of nuclei, with 95% of cases demonstrating diffuse labeling. Most cases (67%) showed weak TRPS1 immunoreactivity, while a smaller subset showed moderate (24%) or strong (9%) intensity staining. Analysis of publicly available gene expression datasets revealed higher levels of TRPS1 mRNA in MPNSTs with PRC2 inactivation. In keeping with this finding, TRPS1 expression was more commonly observed in MPNSTs with loss of H3K27me3, suggesting a potential relationship between TRPS1 and the PRC2 complex. This study further broadens the spectrum of TRPS1-expressing tumors to include MPNST., Competing Interests: Declaration of competing interest No., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Schwannomas are benign tumors derived from the sheath of Schwann cells. Though it is common to see schwannomas in the head and neck region, auricular schwannomas are rare and only few cases have been reported. There are no distinguishing clinical findings or images; therefore, the histopathological diagnosis is mandatory. We describe a case of auricular schwannoma with clinical pictures and discuss the differential diagnoses according to histopathologic findings., 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.

Laryngeal schwannomas are rare benign neurogenic tumors of the larynx. They cannot be detected in the early stages because disease onset is asymptomatic. This case of laryngeal schwannoma provided a reference for clinical diagnosis and treatment strategy. A 24-year-old man presented with a 3-year history of hoarseness and sore throat for 6 months. Laryngoscopy and computed tomography showed submucosal bulging of the right ventricular fold in the right paraglottic space. The patient underwent tracheotomy first, followed by laryngotomy. The edema of the laryngeal mucosa was significantly alleviated, with normal movement of the bilateral vocal cords on the 10th day after surgery. The tracheal tube was removed at the time of discharge. The hoarseness had disappeared at the end of the first month after surgery. The effective treatment of laryngeal schwannoma involves complete resection with surgery while protecting the integrity of the larynx and restoring laryngeal function. The indication for surgery should be strictly controlled to avoid damage to the mucosa and structures of the larynx., 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.

Tumors develop in an oxidative environment characterized by peroxynitrite production and downstream protein tyrosine (Y) nitration. We showed that tyrosine nitration supports schwannoma cell proliferation and regulates cell metabolism in the inheritable tumor disorder NF2-related Schwannomatosis (NF2-SWN). Here, we identified the chaperone Heat shock protein 90 (Hsp90) as the first nitrated protein that acts as a metabolic switch to promote schwannoma cell proliferation. Doubling the endogenous levels of nitrated Hsp90 in schwannoma cells or supplementing nitrated Hsp90 into normal Schwann cells increased their proliferation. Metabolically, nitration on either Y33 or Y56 conferred Hsp90 distinct functions; nitration at Y33 (Hsp90 NY33 ) down-regulated mitochondrial oxidative phosphorylation, while nitration at Y56 (Hsp90 NY56 ) increased glycolysis by activating the purinergic receptor P2X7 in both schwannoma and normal Schwann cells. Hsp90 NY33 and Hsp90 NY56 showed differential subcellular and spatial distribution corresponding with their metabolic and proliferative functions in schwannoma three-dimensional cell culture models. Collectively, these results underscore the role of tyrosine nitration as a post-translational modification regulating critical cellular processes. Nitrated proteins, particularly nitrated Hsp90, emerge as a novel category of tumor-directed therapeutic targets., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: The rhomboid lip is a neural tissue encountered during cerebellopontine angle surgery, with differing shape and extent among individuals. This study aimed to investigate the variation of rhomboid lips during posterior fossa surgery., Methods: In this retrospective study, we examined posterior cranial fossa surgeries performed using a retrosigmoid approach. Rhomboid lips were classified according to thickness, extent, and appearance, with some subjected to histological analysis. T2-weighted magnetic resonance imaging of rhomboid lips was conducted., Results: Among 304 surgeries, rhomboid lips were observed in 75 patients who underwent schwannoma or meningioma resection, facial spasm-related neurovascular decompression, and other surgeries (37, 2, 32, and 4 patients, respectively). Rhomboid lips were categorized based on apparent thickness: thin membranous type, resembling an arachnoid membrane, and thick parenchymal type. Rhomboid lip extension was classified by position relative to the choroid plexus: nonextension, lateral extension, and jugular foramen (41, 22, and 12 patients, respectively). Veins were observed on the rhomboid lip surface in 37 cases. The rhomboid lip was visible in only 1 case (parenchymal jugular foramen type) on magnetic resonance imaging. Histologically, the rhomboid lip comprised an ependymal cell layer, a glial layer, and connecting tissue. The glial layer thickness determined the rhomboid lip thickness, which was greater in the parenchymal type than in the membrane type. In 42 patients, the rhomboid lip was dissected with no complications observed., Conclusions: Morphological classification of the rhomboid lip and understanding of its anatomical details contribute to safe surgical field development for neurosurgeons., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: The aim of this retrospective study was to compare the efficacy of transcervical (TC), endoscope-assisted transoral (TO), and endoscope-assisted TC for resection of retrostyloid space schwannomas., Methods: The study included patients who underwent complete resection of schwannomas by only one surgical approach. The data we collected included tumor size, estimated blood loss, postoperative complications, and so on. Statistical analysis was performed using one-way analysis of variance and Fisher's exact test., Results: The study collected 85 patients with tumors mostly located at the oropharyngeal level who were followed up 6 months at least. The results showed that endoscope-assisted TO had certain advantages over others. Additionally, the endoscope-assisted TO set the lowest incidence of neurological complications., Conclusion: Our findings demonstrate that for team with rich experience in the skull base surgery, endoscope-assisted TO is a superior option compared to the other two groups for resection of retrostyloid space schwannomas, with the better preservation of neurological function., (© 2024 Wiley Periodicals LLC.)

Abstract: Esophageal schwannoma is very rare. We describe FDG PET/CT findings in a case of benign esophageal schwannoma. Endoscopic ultrasound showed the tumor was located in the muscular layer of the esophagus. FDG PET/CT showed intense FDG uptake with SUV max of 10 of the tumor mimicking malignancy. This case indicates that schwannoma should be included in the differential diagnosis of esophageal FDG-avid lesions., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

This study aimed to compare clinical and pathological features of retroperitoneal classical schwannomas and cellular schwannomas. A total of 64 cases of retroperitoneal classical schwannoma and 48 cases of cellular schwannoma were studied. Histopathological analysis was performed using hematoxylin and eosin staining and immunohistochemistry. Retroperitoneal cellular schwannomas exhibited 100% (48/48) and 75% (36/48) positive expression for glial fibrillary acidic protein (GFAP) and cytokeratins (CK), respectively. Classical schwannomas showed rates of 6.25% (4/64) and 15.63% (10/64), respectively (P < .05). In classic schwannomas, 85.9% (55/64) showed a reticular pattern of positive anti-CD34 staining around tumor margins and subcapsular areas vs 52.1% (25/48) in cellular schwannomas (P < .05). Cellular schwannomas exhibited more mitotic figures than classical schwannoma (P < .05). The recurrence rate of cellular schwannomas was 10.42% (5/48), while that of classical schwannomas was 1.56% (1/64) (P < .05). Retroperitoneal cellular schwannomas commonly express GFAP and CK compared to classical schwannomas, suggesting that cellular schwannoma may originate from unmyelinated Schwann cells, while classical schwannoma may originate from myelinated Schwann cells. Anti-CD34 staining patterns may be used to distinguish between the 2 types. Retroperitoneal cellular schwannomas also show higher mitotic activity and are more prone to recurrence., (© The Author(s) 2024. Published by Oxford University Press on behalf of American Association of Neuropathologists, Inc.)

Schwannomas are benign peripheral nerve sheath tumours with a low risk of malignant transformation. About 25%-40% are in the head and neck region with the posterior third of the tongue being a rather rare site of its growth, and a lesion at this site is understandably difficult to approach and treat. Being benign and encapsulated, surgical excision is in the mainstay of treatment. Symptoms can range from a globus pharyngeus and dysphagia to airway compromise depending on the size and site. Traditionally, a paramedian lip split approach with paramedian mandibulotomy and mandible swing may be used. However, some recent reports of the use of carbon dioxide laser and robotic surgery for tongue base lesions are seen in the literature. Our case report is a unique addition to the management strategies for such tongue base lesions as we employed diode 980 nm laser using a minimally invasive transoral approach with a successful outcome., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Benign esophageal tumors are uncommon, accounting for approximately 2% of esophageal tumors. Esophageal schwannoma is a much rarer solid tumor with few cases reported in the literature. Open surgery is the surgical approach of choice for the treatment of esophageal tumors. With the advent of thoracoscopy, more and more countries are adopting a thoracoscopic approach to treat esophageal tumors, but there is still no clear surgical standard or modality for the treatment of esophageal tumors., Patient Concerns: A 50-year-old woman was admitted to our hospital. Over the past 2 months, her clinical presentation has included progressively worse swallowing disorder and weight loss. Gastroscopy showed an elevated lesion with a smooth surface visible 18 cm out from the incisors. An electron circumferential ultrasound endoscopy showed a hemispherical bulge with a smooth surface 18 to 23 cm from the incisor; the bulge originated from the intrinsic muscular layer and showed a heterogeneous mixed moderate ultrasound with a little blood flow signal and blue-green elastography in 1 of the sections measuring approximately 4 cm × 3 cm. Chest computed tomography (CT) showed a mass-like soft tissue shadow in the upper esophagus measuring approximately 39 mm × 34 mm, with a CT The lumen was compressed and narrowed, and the lumen of the upper part of the lesion was dilated, and the adjacent trachea was compressed and displaced to the right., Interventions: After completion of the examination, assisted by artificial pneumothorax and thoracoscopic resection of esophageal masses were performed., Diagnosis and Outcomes: Postoperative pathology report: Mesenchymal-derived tumor (esophagus), combined with immunohistochemical staining results and morphologic features supported schwannoma. The patient's postoperative course was calm. The patient's postoperative dysphagia subsided., Conclusion: We describe a case of successful treatment of a schwannoma of the upper esophagus using artificial pneumothorax-assisted VATS. The combined use of Sox10 and S100 helps to improve the sensitivity and specificity of schwannoma diagnosis. Damage to the esophageal lining was avoided by mixed thoracoscopic and endoscopic exploration. This approach can also be applied to benign esophageal tumors in the thoracic and subthoracic segments, leading to better minimally invasive results., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objectives: New diagnostic criteria for NF2-related schwannomatosis (NF2) were published in 2022. An updated UK prevalence was generated in accordance with these, with an emphasis on the rate of de novo NF2 (a 50% frequency is widely quoted in genetic counselling). The distribution of variant types among de novo and familial NF2 cases was also assessed., Methods: The UK National NF2 database identifies patients meeting updated NF2 criteria from a highly ascertained population cared for by England's specialised service. Diagnostic prevalence was assessed on 1 February 2023. Molecular analysis of blood and, where possible, tumour specimens for NF2, LZTR1 and SMARCB1 was performed., Results: 1084 living NF2 patients were identified on prevalence day (equivalent to 1 in 61 332). The proportion with NF2 inherited from an affected parent was only 23% in England. If people without a confirmed molecular diagnosis or bilateral vestibular schwannoma are excluded, the frequency of de novo NF2 remains high (72%). Of the identified de novo cases, almost half were mosaic. The most common variant type was nonsense variants, accounting for 173/697 (24.8%) of people with an established variant, but only 18/235 (7.7%) with an inherited NF2 pathogenic variant (p<0.0001). Missense variants had the highest proportion of familial association (56%). The prevalence of LZTR1 -related schwannomatosis and SMARCB1 -related schwannomatosis was 1 in 527 000 and 1 in 1.1M, respectively, 8.4-18.4 times lower than NF2., Conclusions: This work confirms a much higher rate of de novo NF2 than previously reported and highlights the benefits of maintaining patient databases for accurate counselling., Competing Interests: Competing interests: DGE has received consultancy fees from AstraZeneca, Springworks and Everything Genetic Ltd., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: To improve the characteristics of primary thyroid schwannomas (PTS) and to provide reference basis for clinical diagnosis and treatment., Methods: PubMed was searched for case reports of PTS up to December 2022 using the search terms "Thyroid nerve sheath tumor" or "Thyroid schwannoma" or "Thyroid Neurilemmoma", respectively. 34 cases were screened., Results: PTS can occur at any age, nodules averaged 3.9 cm. The most common symptoms were voice change and dysphagia. Fine needle aspiration cytology showing spindle-shaped cells should be considered for schwannoma. Most cases underwent thyroid lobectomy or nodule removal with a good prognosis. Tissue types with both Antoni A and Antoni B features are common. Positive immunohistochemical staining for S-100 protein, CD34 and waveform proteins helped confirm the diagnosis., Conclusions: Positive immunohistochemistry for S-100 and wave proteins helps confirm the diagnosis. Preoperative diagnosis is challenging, but pathology and immunohistochemical staining are the gold standard for diagnosis. The first choice of treatment is surgical resection of the nodules, the prognosis is good., (© 2024. The Author(s).)

Background: Schwannomas in the renal hilum are rare among retroperitoneal tumors. However, the possibility of malignant findings cannot be ruled out, and surgery is often indicated. This case was expected to be difficult to remove laparoscopically because the tumor was sandwiched between the arteriovenous veins of the renal portal. Sometimes, the tumor should be resected with a conservative approach to the kidney to preserve the renal function., Case Presentation: Our patient was a 51-year-old Asian-Japanese man who was referred to our department for a retroperitoneal tumor in the renal hilum. Since malignancy could not be ruled out due to its size (45 × 48 × 55 mm) on imaging, the tumor was excised laparoscopically. Histopathology revealed schwannoma., Conclusions: We herein report a case in which a renal hilar tumor between renal arteriovenous vessels was successfully resected laparoscopically., (© 2024. The Author(s).)

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.)

Purpose: Schwannoma is a rare mesenchymal tumor. In this study, we analyzed clinicopathologically 99 schwannomas.This retrospective study delves into the clinical, pathological, and immunohistochemical dimensions of abdominal schwannomas., Results: A cohort of 99 cases, comprising 4 malignant and 95 benign schwannomas, was meticulously examined. Clinical analysis revealed a notable gender distribution (1:1.7, male to female) and an average age of 58.5 years. The majority of cases were asymptomatic. A cohort of 99 cases, comprising 4 malignant and 95 benign schwannomas, was meticulously examined. Clinical analysis revealed a notable gender distribution (1:1.7, male to female) and an average age of 58.5 years. The majority of cases were asymptomatic. Tumor sizes ranged from 0.5 to 30 cm, with distinct locations in the stomach for most benign cases and the abdomen/small intestine for malignancies. Initial misdiagnoses were frequent. Pathological evaluations revealed distinct features, including Antoni A and B patterns, spindle cells, and lymphatic sheath structures in benign schwannomas. Malignant cases exhibited atypical cells, ulcers, and invasive growth. Immunohistochemical markers, such as S100, SOX10, and vimentin, consistently demonstrated positivity by contributing to accurate diagnoses. Treatment outcomes indicated a poor prognosis in malignant cases, with overall survival ranging from 10 to 41 months. Conversely, benign cases displayed no recurrence or metastasis during follow-up, despite atypical behaviors., Conclusion: This study underscores the rarity of abdominal schwannomas and underscores the need for a comprehensive diagnostic morphology and immunohistochemistry. SOX10 emerges as a crucial and specific marker for accurate diagnosis. Further research is imperative to refine diagnostic protocols and enhance our understanding of the clinical behavior of abdominal schwannomas., (© 2024 The Author(s). Cancer Medicine published by John Wiley & Sons Ltd.)