Schwannomas and Their Pathogenesis

Hilton, David A.; Hanemann, C. Oliver

doi:10.1111/bpa.12125

Cited by 200 publications

(159 citation statements)

References 200 publications

(242 reference statements)

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“…The tumours are named neurofibromas, which develop on the peripheral nerve sheathes consisting of neoplastic Schwann cells, fibroblasts, perineural cells and mast cells [1], and schwannomas, which designate only the neoplastic proliferation of Schwann cells [2]. Neurofibromas are the core findings in NF1 patients, while schwannomas are features displayed in neurofibromatosis type 2 and schwannomatosis [3].…”

Section: Introductionmentioning

confidence: 99%

Proposing the use of dental pulp stem cells as a suitable biological model of neurofibromatosis type 1

Almeida

Souza

et al. 2014

Childs Nerv Syst

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Section: Introductionmentioning

confidence: 99%

Proposing the use of dental pulp stem cells as a suitable biological model of neurofibromatosis type 1

Almeida

Souza

et al. 2014

Childs Nerv Syst

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“…43 These lesions are usually well circumscribed, especially the schwannomas, and grow between the fascicles of peripheral nerves; schwannomas often undergo cystic and degenerative changes. 28 Most cases are sporadic; however, some are associated with neurofibromatosis (NF) Type 1 and Type 2, 33 schwannomatosis, or Carney's complex, 28 and they may rarely occur following radiation. 45,50 Malignant peripheral nerve sheath tumors (MPNSTs) show variable differentiation toward one of the cellular components of nerve sheaths (Schwann cells, fibroblasts, and perineurial cells).…”

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confidence: 99%

Tumors of the peripheral nervous system: analysis of prognostic factors in a series with long-term follow-up and review of the literature

Montano¹,

D’Alessandris²,

D’Ercole³

et al. 2016

JNS

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abbreviatioNs BPNST = benign PNST; MPNST = malignant PNST; NF = neurofibromatosis; OS = overall survival; PFS = progression-free survival; PNNST = peripheral non-neural sheath tumor; PNST = peripheral nerve sheath tumor; VAS = visual analog scale. patients (with 173 surgically treated tumors) had adequate follow-up data available for analysis. Pain was assessed using a visual analog scale (VAS), and motor and sensory function were assessed by means of the Louisiana State University grading system. They also analyzed the relationship between tumor recurrence and patient sex, patient age, diagnosis of neurofibromatosis (NF), tumor histopathology, tumor size, tumor location, and extent of resection (subtotal vs gross-total resection), using univariate and multivariate analyses. results There was a statistically significant improvement in the mean VAS pain score (preoperative 3.96 ± 2.41 vs postoperative 0.95 ± 1.6, p = 0.0001). Motor strength and sensory function were significantly improved after resection of tumors involving the brachial plexus (p = 0.0457 and p = 0.0043, respectively), tumors involving the upper limb (p = 0.0016 and p = 0.0016, respectively), BPNSTs (p = 0.0011 and p < 0.0001, respectively), and tumors with dimensions less than 5 cm (motor strength: p = 0.0187 and p = 0.0021 for ≤ 3 cm and 3-5 cm tumors, respectively; sensory function: p = 0.0003 and p = 0.0001 for ≤ 3 cm and 3-5 cm tumors, respectively). Sensory function showed a statistically significant improvement also in patients who had undergone resection of tumors involving the lower limb (p = 0.0118). Total resection was associated with statistically significant improvement of motor strength (p = 0.0251) and sensory function (p < 0.0001). In univariate analysis, a history of NF (p = 0.0034), a diagnosis of MPNST or PNNST (p < 0.0001), and subtotal resection (p = 0.0042) were associated with higher risk of tumor recurrence. In multivariate analysis (logistic regression analysis), a history of NF (OR 9.28%, 95% CI 1.62-52.94, p = 0.0121) and a diagnosis of MPNST (OR 0.03%, 95% CI 0.002-0.429, p = 0.0098) or PNNST (OR 0.081%, 95% CI 0.013-0.509, p = 0.0077) emerged as independent prognostic factors for tumor recurrence. coNclusioNs A total resection should be attempted in all cases of peripheral nervous system tumors (irrespective of the supposed diagnosis and tumor dimensions) because it is associated with better prognosis in term of functional outcome and overall survival. Moreover, a total resection predicts a lower risk of tumor recurrence. Patients with a history of NF and tumors with malignant histology remain a challenge both for neurosurgeons and oncologists due to higher recurrence rates and the lack of standardized adjuvant therapies.

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“…Schwannomas, the most common PNST in adults and comprised of abnormal Schwann-like cells, are benign, slow-growing, circumscribed, encapsulated, solitary, and non-infiltrating tumors 2, 19 . NF2 patients develop schwannomas, often multiple, in cranial, spinal, and peripheral nerves, with bilateral vestibular schwannomas a hallmark 2, 19 .…”

Section: Peripheral Nerve Sheath Tumors (Pnst) and Perineural Invamentioning

confidence: 99%

Prospects and progress of oncolytic viruses for treating peripheral nerve sheath tumors

Antoszczyk

Rabkin

2015

Expert Opinion on Orphan Drugs

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Introduction Peripheral nerve sheath tumors (PNSTs) are an assorted group of neoplasms originating from neuroectoderm and growing in peripheral nerves. Malignant transformation leads to a poor prognosis and is often lethal. Current treatment of PNSTs is predominantly surgical, which is often incomplete or accompanied by significant loss of function, in conjunction with radiotherapy and/or chemotherapy, for which the benefits are inconclusive. Oncolytic viruses (OVs) efficiently kill tumor cells while remaining safe for normal tissues, and are a novel antitumor therapy for patients with PNSTs. Areas covered Because of the low efficacy of current treatments, new therapies for PNSTs are needed. Pre-clinically, OVs have demonstrated efficacy in treating PNSTs and perineural tumor invasion, as well as safety. We will discuss the various PNSTs and their preclinical models, and the OVs being tested for their treatment, including oncolytic herpes simplex virus (HSV), adenovirus (Ad), and measles virus (MV). OVs can be ‘armed’ to express therapeutic transgenes or combined with other therapeutics to enhance their activity. Expert opinion Preclinical testing of OVs in PNST models has demonstrated their therapeutic potential and provided support for clinical translation. Clinical studies with other solid tumors have provided evidence that OVs are safe in patients and efficacious. The recent successful completion of a phase III clinical trial of oncolytic HSV paves the way for oncolytic virotherapy to enter clinical practice.

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Schwannomas and Their Pathogenesis

Cited by 200 publications

References 200 publications

Proposing the use of dental pulp stem cells as a suitable biological model of neurofibromatosis type 1

Proposing the use of dental pulp stem cells as a suitable biological model of neurofibromatosis type 1

Tumors of the peripheral nervous system: analysis of prognostic factors in a series with long-term follow-up and review of the literature

Prospects and progress of oncolytic viruses for treating peripheral nerve sheath tumors

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