Publications by authors named "Scott A Rutherford"

 Skull base meningiomas (SBMs) are often subtotally resected and there is a paucity of evidence regarding the long-term rates of postoperative tumor progression. We aimed to investigate the factors that influence tumor recurrence in patients with an extended period of follow-up.  Surgically resected tumors with long-term radiological follow-up were included for analysis.

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Objective: Skull base chordoma and chondrosarcoma are distinct sarcomas of the skull base but share significant therapeutic challenges due to their proximity to critical neurovascular structures, making surgical resection difficult. We sought to establish factors associated with outcome predictors in a national cohort of patients.

Methods And Analysis: Data for all patients referred with a diagnosis of skull base chordoma or chondrosarcoma from April 2017 to December 2022 were obtained.

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Skull base chordomas and chondrosarcomas are distinct types of rare, locally aggressive mesenchymal tumors that share key principles of imaging investigation and multidisciplinary care. Maximal safe surgical resection is the treatment choice for each, often via an expanded endoscopic endonasal approach, with or without multilayer skull base repair. Postoperative adjuvant radiation therapy is frequently administered, usually with particle therapy such as proton beam therapy (PBT).

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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 -related schwannomatosis (SWN), but when BVS are absent, this can also indicate -related or -related SWN.

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Background: Nonauditory symptoms can be a prominent feature in patients with sporadic vestibular schwannoma (VS), but the cause of these symptoms is unknown. Inflammation is hypothesized to play a key role in the growth and symptomatic presentation of sporadic VS, and in this study, we investigated through translocator protein (TSPO) positron emission tomography (PET) whether inflammation occurred within the "normal appearing" brain of such patients and its association with tumor growth.

Methods: Dynamic PET datasets from 15 patients with sporadic VS (8 static and 7 growing) who had been previously imaged using the TSPO tracer [C]()-PK11195 were included.

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Article Synopsis
  • The study investigates the potential long-term effects of radiation treatment, particularly focusing on the rare occurrence of radiation-induced vestibular schwannomas (VSs) among patients who received radiation that covered the posterior fossa.* -
  • A review of eight cases revealed that patients had a median age of 15 years at the time of radiation and were diagnosed with VS a median of 51 years later, with some tumor characteristics noted, such as adherence to the facial nerve.* -
  • The findings suggest that while the occurrence of radiation-induced VS is rare, more research is needed to understand its causative relationship with radiation exposure and to improve management strategies for affected patients.*
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Purpose: There is no guidance surrounding postoperative venous thromboembolism (VTE) prophylaxis using pharmacological agents (chemoprophylaxis) in patients undergoing skull base surgery. The aim of this study was to compare VTE and intracranial haematoma rates after skull base surgery in patients treated with/without chemoprophylaxis.

Methods: Review of prospective quaternary centre database including adults undergoing first-time skull base surgery (2009-2020).

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Objective: Translabyrinthine excision of a vestibular schwannoma is associated with acute vestibular failure. Preoperative intratympanic gentamicin (ITG) injections can improve objective balance function after surgery but its clinical benefits remain to be established.

Methods: Adult patients undergoing translabyrinthine removal of a vestibular schwannoma between January 2014 and February 2018 underwent preoperative vestibular function testing.

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Objective: This study aimed to assess degree of audiovestibular handicap in patients with vestibular schwannoma.

Methods: Audiovestibular handicap was assessed using the Hearing Handicap Inventory, Tinnitus Handicap Inventory and Dizziness Handicap Inventory. Patients completed questionnaires at presentation and at least one year following treatment with microsurgery, stereotactic radiosurgery or observation.

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Background: The occurrence of hyperostotic bilateral spheno-orbital meningiomas (BSOMs) is very rare. Patients present with bilateral symptoms and require bilateral treatment. This series describes 6 patients presenting to 2 UK neurosurgical units and includes a literature review.

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Article Synopsis
  • NF2-schwannomatosis is the leading genetic syndrome linked to meningioma, causing significant health issues due to the presence of multiple tumors like schwannomas and ependymomas.
  • Managing meningiomas in NF2-schwannomatosis is complex, requiring careful consideration of treatment options and their risks, focusing on conservative management until patients become symptomatic.
  • Effective treatment involves surgery for symptomatic tumors and collaboration with specialized teams, while approaches like radiotherapy and medications (e.g., bevacizumab) have specific roles and limitations in this context.
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Objective: Skull base meningiomas (SBMs) involving the cavernous sinus encase the internal carotid artery (ICA) and may lead to stenosis of the vessel. Although ischemic stroke has been reported in the literature, there are to the authors' knowledge no reported studies quantifying the risk of stroke in these patients. The authors aimed to determine the frequency of arterial stenosis in patients with SBMs that encase the cavernous ICA and to estimate the risk of ischemic stroke in these patients.

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Article Synopsis
  • The study examines the safety of radiation treatment for benign tumors in patients with NF2-related schwannomatosis, finding an increased risk of subsequent malignancies or malignant progression in those who received radiation compared to untreated patients.
  • Out of 1345 NF2 patients studied, 266 received radiation, leading to a malignancy rate of approximately 6%, as opposed to less than 1% in those not treated with radiation.
  • The findings suggest that NF2 patients should not receive radiation as the first treatment option for benign tumors due to the associated risks and should be informed about the significant risk of developing new malignancies.
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Objective: Preoperative differentiation of facial nerve schwannoma (FNS) from vestibular schwannoma (VS) can be challenging, and failure to differentiate between these two pathologies can result in potentially avoidable facial nerve injury. This study presents the combined experience of two high-volume centers in the management of intraoperatively diagnosed FNSs. The authors highlight clinical and imaging features that can distinguish FNS from VS and provide an algorithm to help manage intraoperatively diagnosed FNS.

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Background: There is evidence that macrophage infiltration in the tumor microenvironment promotes vestibular schwannoma (VS) growth. Efficacy of bevacizumab in NF2-associated VS demonstrates the value of therapies targeting the microvascular tumor microenvironment, and tumor-associated macrophages (TAMs) may represent another druggable target.

Objective: To characterize the relationship between growth, TAM infiltration, and circulating monocyte chemokines in a large cohort of patients with VS.

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Vestibular schwannomas are benign nerve sheath tumours that arise on the vestibulocochlear nerves. Vestibular schwannomas are known to occur in the context of tumour predisposition syndromes NF2-related and LZTR1-related schwannomatosis. However, the majority of vestibular schwannomas present sporadically without identification of germline pathogenic variants.

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The authors present the case of a 24-year-old female with neurofibromatosis type 2. Growth of the left vestibular schwannoma and progressive hearing loss prompted the decision to proceed to translabyrinthine resection with cochlear nerve preservation and cochlear implant insertion. Complete resection with preservation of the facial and cochlear nerves was achieved.

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Background: Bilateral vestibular schwannomas (VS) are pathognomonic of neurofibromatosis type 2 (NF2), but the diagnostic criteria also include unilateral VS (UVS) in combination with multiple meningiomas (MM) and other schwannomas, as well as MM without VS.

Objective: To investigate the diagnostic value of these criteria and establish the presence of other genetic conditions in patients presenting in this manner.

Methods: The Manchester International NF2 database was accessed to obtain information on patients presenting with a UVS and MM or ≥2 nonintradermal schwannomas (NIDS).

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 The study aimed to determine long-term outcomes in patients with intraoperative electrical conduction block in an anatomically intact facial nerve (FN).  Single center retrospective review of prospectively collected database of all vestibular schwannoma surgeries between January 1, 2008 and August 25, 2015. Operative notes were reviewed and patients with anatomically intact FNs, but complete conduction block at the end of surgery were included for analysis.

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Background: Limited data exist on the disease course of neurofibromatosis type 2 (NF2) to guide clinical trial design.

Methods: A prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990 and 2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred, and inheritance type.

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Clear cell meningioma (CCM) is a rare variant of meningioma. In recent years, an association between cranial and spinal CCMs and germline loss of function mutations in the SMARCE1 gene (SWI/SNF chromatin remodeling complex subunit gene) has been discovered. We report a family with an incidental large spinal clear cell meningioma in a young adult following reflex screening for a germline loss of function pathogenic variant (PV) in the SMARCE1 gene.

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 Vestibular schwannomas (VS) are histologically benign tumors arising from cranial nerve VIII. Far from a homogenous proliferation of Schwann cells, mounting evidence has highlighted the complex nature of the inflammatory microenvironment in these tumors.  A review of the literature pertaining to inflammation, inflammatory molecular pathways, and immune-related therapeutic targets in VS was performed.

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Objective: Inflammation and angiogenesis may play a role in the growth of sporadic and neurofibromatosis type 2 (NF2)-related vestibular schwannoma (VS). The similarities in microvascular and inflammatory microenvironment have not been investigated. The authors sought to compare the tumor microenvironment (TME) in sporadic and NF2-related VSs using a combined imaging and tissue analysis approach.

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Article Synopsis
  • The study evaluates the occurrence of mosaicism in de novo neurofibromatosis 2 (NF2) among patients without known prior family history, testing over 1,000 individuals for NF2 variants using DNA from lymphocytes and tumors.
  • The findings show that 22% of patients exhibited proven or presumed mosaic NF2 variants, with the overall probable mosaicism rate estimated at nearly 60%, varying significantly with age.
  • The research concludes that NF2 likely has the highest mosaicism rate in nonlethal de novo dominant diseases, suggesting minimal risks to offspring that may be related to the variant frequencies found in the parents' blood.
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