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Schwannomas – So-Called Benign Nervous System Tumors
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Schwannomas – So-Called Benign Nervous System Tumors

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Molecular Cancer Biology".

Deadline for manuscript submissions: closed (31 January 2021) | Viewed by 21812

Special Issue Editors

Dr. Carsten Hagemann

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Guest Editor
Tumorbiology Laboratory, Department of Neurosurgery, University of Würzburg, Josef-Schneider-Str. 11, D-97080 Würzburg, Germany
Prof. Dr. Cordula Matthies

E-Mail Website
Guest Editor
Department of Neurosurgery, University Hospital of Würzburg, Josef-Schneider-Straße 11, Würzburg, Germany
Interests: function; audiology; hearing disorders; deafness; neuro-oncology; deep brain stimulation; head and neck surgery; speech intelligibility; spine surgery; brain injury; movement disorders

Special Issue Information

Dear Colleagues,

Schwannomas are so-called benign tumors of the nervous system, originating from the Schwann cell sheaths of cranial or peripheral nerves. In accordance with their benign nature, they do not cause metastasis, except if they transform into malignant nerve sheath tumors MPNST, and usually grow slowly, causing insidiously progressive symptoms along with increasing nerve compression. Different to expectations though, if not diagnosed early on, they do destroy their nerves of origin and, frequently, lesion-neighbouring nerves.

Most frequently, schwannomas occur as vestibular schwannomas (formerly called acoustic neuromas), tumors of the vestibulo-cochlear nerves, and in up to 8% of cases, they present bilaterally due to the genetic constellation of neurofbromatosis type 2 (NF2). They cause loss of vestibular function and carry a high risk of deafness and facial palsy by natural course as well as by treatment. They may also lead to trigeminal pain, swallowing deficit, and vocal cord palsies. Further, schwannomas of the spinal roots, nerve plexus, and peripheral nerves cause considerable sensory and motor deficits and also pain. As NF2 is one of the most well-defined genetic diseases, it is a highly interesting model of investigating and correlating genetic, tumor biological, and clinical features.

The mechanisms of nerve border invasion by schwannomas is an ongoing topic of discussion and laboratory investigations, as is the question why some of these tumors remain dormant and some start excessive proliferation; these characteristics relate to the extent of “malignant” behaviour towards neural structures and to underlying genetic disease varying largely from patient to patient. The NF2 mutation causes deficiency of Merlin and of LATS1 and 2, thereby disturbing the equilibrium between ERK and Rac cascades with elevated MAPK signaling and dysregulation of the HIPPO pathway, all causing tumor cell proliferation as well as resistance to radiation, chemotherapy, and DNA-damaging agents.

The Special Issue ”Schwannomas” of Cancers shall give an introduction to one of the most frequent tumors of the nervous system, convey current knowledge of its biological behaviour, typical clinical presentation, and available as well as evolving treatment options with regard to tumor cure and nerve function.

Dr. Carsten Hagemann
Prof. Dr. Cordula Matthies
Guest Editors

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Published Papers (7 papers)

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13 pages, 4400 KiB  
Article
Risk Stratification for Immediate Postoperative Hearing Loss by Preoperative BAER (Brainstem Auditory Evoked Response) and Audiometry in NF2-Associated Vestibular Schwannomas
by Isabel Gugel, Florian Grimm, Philip Hartjen, Maria Breun, Julian Zipfel, Marina Liebsch, Hubert Löwenheim, Ulrike Ernemann, Lan Kluwe, Victor-Felix Mautner, Marcos Tatagiba and Martin Ulrich Schuhmann
Cancers 2021, 13(6), 1384; https://doi.org/10.3390/cancers13061384 - 18 Mar 2021
Cited by 1 | Viewed by 2282
Abstract
Both brainstem auditory evoked potentials (BAEP) and audiometry play a crucial role in neuro-oncological treatment decisions in Neurofibromatosis Type 2 associated (NF2) vestibular schwannoma (VS) as hearing preservation is the major goal. In this study, we investigated the risk of immediate postoperative hearing [...] Read more.
Both brainstem auditory evoked potentials (BAEP) and audiometry play a crucial role in neuro-oncological treatment decisions in Neurofibromatosis Type 2 associated (NF2) vestibular schwannoma (VS) as hearing preservation is the major goal. In this study, we investigated the risk of immediate postoperative hearing deterioration (>15 dB and/or 15% loss in pure-tone average [PTA]/ speech discrimination score [SDS] in a cohort of 100 operated VS (ears) in 72 NF2 patients by retrospective analysis of pre- and postoperative hearing data (PTA, SDS, American Association of Otolaryngology–Head and Neck Surgery [AAO-HNS], and brainstem auditory evoked potential [BAEP] class) taking into account relevant influencing factors, particularly preoperative audiometry and BAEP status and the extent of resection. Immediately after surgery, the hearing was preserved in 73% of ears and approximately ~60% of ears kept their hearing classes. Preoperative BAEP (p = 0.015) and resection amount (p = 0.048) significantly influenced postoperative hearing outcome. The prediction model for postoperative hearing deterioration/loss between preoperative BAEP and AAO-HNS class showed increased risk by increasing BAEP class. Twenty-one tumors/ears were identified with large BAEP and AAO-HNS class discrepancies (≥2 points) and were associated with a high (48–100%) risk of deafness after surgery in ears with preoperative available hearing. Overall, the results were heterogeneous but the better both BAEP and audiometry class before surgery, the higher the chance of hearing maintenance afterwards. Large resection amounts (e.g., 100% risk in near-total resections) exhibit a significant (p < 0.05) higher risk compared to smaller amounts (e.g., 10/20% in laser-coagulated/partially resected tumors). Our results emphasized the indispensable role of both hearing monitoring in form of audiometry and neurophysiology (BAEP) in the pre-and perioperative monitoring of NF2-associated VS. Both BAEP and audiometry are good prognostic markers for the postoperative hearing outcome. The extent of resection should be strictly guided by and adjusted to the intraoperative neurophysiological monitoring. Full article
(This article belongs to the Special Issue Schwannomas – So-Called Benign Nervous System Tumors)
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16 pages, 10833 KiB  
Article
Surgical Outcome of Trigeminal Schwannomas
by Amir Kaywan Aftahy, Maximilian Groll, Melanie Barz, Arthur Wagner, Nicole Lange, Vicki Marie Butenschön, Claire Delbridge, Denise Bernhardt, Bernhard Meyer, Chiara Negwer and Jens Gempt
Cancers 2021, 13(6), 1310; https://doi.org/10.3390/cancers13061310 - 15 Mar 2021
Cited by 13 | Viewed by 5959
Abstract
(1) Background: As resection of trigeminal schwannomas is challenging, due to anatomical involvement of the anterior, middle and posterior fossa, the appropriate approach is important. We report our experience with surgical resection of trigeminal schwannomas by simple and classic skull-base approaches. (2) Methods: [...] Read more.
(1) Background: As resection of trigeminal schwannomas is challenging, due to anatomical involvement of the anterior, middle and posterior fossa, the appropriate approach is important. We report our experience with surgical resection of trigeminal schwannomas by simple and classic skull-base approaches. (2) Methods: We performed a retrospective single-center study including patients who underwent surgery for trigeminal schwannoma tumors between June 2007 and May 2020, concentrating on surgical technique, extent of resection, postoperative outcome and complications. (3) Results: We included 13 patients (median age 57.5 with range of 36-83 years, 53.8% (7/13) female. The most common preoperative clinical presentations were facial pain in six (46.2%), hypoacusis in four (30.8%), trigeminal nerve hypesthesia in 11 (V1 46.2% (6/13), V2 (61.5% (8/13), V3 46.2% (6/13)) and headache in three (23.1%) patients. In three cases, the tumor was resected in a two-stage technique. The intradural subtemporal approach was performed in six cases, the extradural technique in two cases, the retrosigmoid approach in five cases, a Kawase approach in two cases and the transnasal endoscopic approach in one case. The gross total resection rate was 76.9% (10/13) and the median follow-up time 24.0 (0–136) months. Three (23.1%) patients developed postoperative anesthesia in at least one branch of the trigeminal nerve. Trigeminal motor function was preserved in 11 (84.6%) patients. Ten of the 11 patients (76.9%) who reported decreased gustation, cerebellar ataxia, visual impairment, or headache improved postoperatively. Two (15.4%) patients exhibited minimal facial palsy (House and Brackmann II–III), which resolved during the follow-up. The total adverse event rate requiring surgical intervention during follow-up was 7.7%. Surgery-related mortality was 0%. (4) Conclusions: Trigeminal schwannomas are rare benign lesions with intra- and extracranial extension. Considering the low operative morbidity and satisfying functional outcome, gross total resection of trigeminal schwannomas is achievable by classic, but also individually tailored approaches. More invasive or combined techniques were not needed with meticulous surgical planning. Full article
(This article belongs to the Special Issue Schwannomas – So-Called Benign Nervous System Tumors)
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11 pages, 775 KiB  
Article
Long-Term Outcomes of Stereotactic Radiosurgery for Trigeminal, Facial, and Jugular Foramen Schwannoma in Comparison with Vestibular Schwannoma
by Yuki Shinya, Hirotaka Hasegawa, Masahiro Shin, Takehiro Sugiyama, Mariko Kawashima, Atsuto Katano, Akinori Kashio, Kenji Kondo and Nobuhito Saito
Cancers 2021, 13(5), 1140; https://doi.org/10.3390/cancers13051140 - 7 Mar 2021
Cited by 12 | Viewed by 2388
Abstract
Stereotactic radiosurgery (SRS) is known to provide excellent tumor control with functional preservation for vestibular schwannomas (VS), but its efficacy in the other major intracranial schwannomas including trigeminal (TS), facial (FS), and jugular foramen schwannomas (JFS) has not been established yet due to [...] Read more.
Stereotactic radiosurgery (SRS) is known to provide excellent tumor control with functional preservation for vestibular schwannomas (VS), but its efficacy in the other major intracranial schwannomas including trigeminal (TS), facial (FS), and jugular foramen schwannomas (JFS) has not been established yet due to their rarity. We retrospectively analyzed data of 514 consecutive patients who had intracranial schwannomas (460 VS, 22 TS, 7 FS, and 25 JFS) and underwent SRS. The 5- and 10-year tumor control rates were 97% and 94% for VS, 100% and 100% for TS, 80% and 80% for FS, and 100% and 80% for JFS. Radiation-induced complications included one hydrocephalus for TS (4.5%), no cases for FS (0%), and one hydrocephalus and one lower palsy for JFS (8.0%). Through matched cohort analysis between patients with VS and each of the non-VS, we found no statistical difference in tumor control and radiation-induced adverse events. SRS seems to provide long-term tumor control with functional preservation for TS, FS and JFS and the efficacies are similar to VS. Full article
(This article belongs to the Special Issue Schwannomas – So-Called Benign Nervous System Tumors)
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11 pages, 1101 KiB  
Article
Surgical Management of Sporadic Peripheral Nerve Schwannomas in Adults: Indications and Outcome in a Single Center Cohort
by Julian Zipfel, Meizer Al-Hariri, Isabel Gugel, Alexander Grimm, Volker Steger, Ruth Ladurner, Michael Krimmel, Marcos Tatagiba and Martin U. Schuhmann
Cancers 2021, 13(5), 1017; https://doi.org/10.3390/cancers13051017 - 1 Mar 2021
Cited by 8 | Viewed by 2657
Abstract
Most sporadic peripheral nerve sheath tumors in adults are schwannomas. These tumors usually present with significant pain but can also cause neurological deficits. Symptomatology is diverse, and successful surgical interventions demand interdisciplinarity. We retrospectively reviewed 414 patients treated between 2006 and 2017 for [...] Read more.
Most sporadic peripheral nerve sheath tumors in adults are schwannomas. These tumors usually present with significant pain but can also cause neurological deficits. Symptomatology is diverse, and successful surgical interventions demand interdisciplinarity. We retrospectively reviewed 414 patients treated between 2006 and 2017 for peripheral nerve sheath tumors. We analyzed clinical signs, symptoms, histology, and neurological function in the cohort of adult patients with schwannomas without a neurocutaneous syndrome. In 144 patients, 147 surgical interventions were performed. Mean follow-up was 3.1 years. The indication for surgery was pain (66.0%), neurological deficits (23.8%), significant tumor growth (8.8%), and suspected malignancy (1.4%). Complete tumor resection was achieved on 136/147 occasions (92.5%). The most common location of the tumors was intraspinal (49.0%), within the cervical neurovascular bundles (19.7%), and lower extremities (10.9%). Pain and neurological deficits improved significantly (p ≤ 0.003) after 131/147 interventions (89.1%). One patient had a persistent decrease in motor function after surgery. Complete resection was possible in 67% of recurrent tumors, compared to 94% of primary tumors. There was a significantly lower chance of complete resection for schwannomas of the cervical neurovascular bundle as compared to other locations. The surgical outcome of sporadic schwannoma surgery within the peripheral nervous system is very favorable in experienced peripheral nerve surgery centers. Surgery is safe and effective and needs a multidisciplinary setting. Early surgical resection in adult patients with peripheral nerve sheath tumors with significant growth, pain, neurological deficit, or suspected malignancy is thus recommended. Full article
(This article belongs to the Special Issue Schwannomas – So-Called Benign Nervous System Tumors)
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15 pages, 3004 KiB  
Article
Macrophage and Lymphocyte Infiltration Is Associated with Volumetric Tumor Size but Not with Volumetric Growth in the Tübingen Schwannoma Cohort
by Vítor Moura Gonçalves, Elisa-Maria Suhm, Vanessa Ries, Marco Skardelly, Ghazaleh Tabatabai, Marcos Tatagiba, Jens Schittenhelm and Felix Behling
Cancers 2021, 13(3), 466; https://doi.org/10.3390/cancers13030466 - 26 Jan 2021
Cited by 10 | Viewed by 2301 | Correction
Abstract
Most patients with vestibular schwannomas can be cured with microsurgical resection, or tumor growth can be stabilized by radiotherapy in certain cases. Recurrence is rare but usually difficult to treat. Treatment alternatives to local therapies are not established. There is growing evidence of [...] Read more.
Most patients with vestibular schwannomas can be cured with microsurgical resection, or tumor growth can be stabilized by radiotherapy in certain cases. Recurrence is rare but usually difficult to treat. Treatment alternatives to local therapies are not established. There is growing evidence of the role of inflammatory processes in schwannomas, which may be exploitable by targeted innovative therapies. To further define the impact of inflammation with tumor growth in vestibular schwannoma, we performed immunohistochemical analyses of CD3, CD8, CD68 and CD163 to assess lymphocyte and macrophage infiltration in 923 tumor tissue samples of surgically resected vestibular schwannomas. An inflammatory score was compared with tumor size and volumetric growth. We observed a significantly larger preoperative tumor size with increased expression rates of CD3, CD8, CD68 and CD163 (p < 0.0001, p < 0.0001, p = 0.0015 and p < 0.0001, respectively), but no differences in percentual volumetric tumor growth. When all four markers were combined as an inflammatory score, tumors with high inflammatory infiltration showed slower percentual growth in a multivariate analysis, including MIB1 expression (p = 0.0249). We conclude that inflammatory cell infiltration increases with larger tumor size but is associated with slower percentual volumetric tumor growth. Full article
(This article belongs to the Special Issue Schwannomas – So-Called Benign Nervous System Tumors)
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12 pages, 586 KiB  
Article
Age at Onset and Presenting Symptoms of Neurofibromatosis Type 2 as Prognostic Factors for Clinical Course of Vestibular Schwannomas
by Isabel Gugel, Florian Grimm, Julian Zipfel, Christian Teuber, Ulrike Ernemann, Lan Kluwe, Marcos Tatagiba, Victor-Felix Mautner and Martin Ulrich Schuhmann
Cancers 2020, 12(9), 2355; https://doi.org/10.3390/cancers12092355 - 20 Aug 2020
Cited by 6 | Viewed by 2882
Abstract
The presenting symptoms of the tumor suppressor gene syndrome neurofibromatosis type 2 (NF2) are often non-specific and unrelated to the disease hallmark bilateral vestibular schwannomas (VS). However, age at onset and presenting symptoms may have predictive values for the clinical course of VS. [...] Read more.
The presenting symptoms of the tumor suppressor gene syndrome neurofibromatosis type 2 (NF2) are often non-specific and unrelated to the disease hallmark bilateral vestibular schwannomas (VS). However, age at onset and presenting symptoms may have predictive values for the clinical course of VS. In this retrospective single-center study, we addressed this issue by reviewing 106 patients with 194 VS. Presenting symptoms attributable to VS commonly occur in 87% of adults and 31% of children. Age at onset significantly correlates with tumor volumes at presentation (p = 0.034). In addition, age at onset significantly correlates with pure-tone average (p = 0.0001), speech discrimination scores (p = 0.001), age at beginning of hearing loss (p = 0.0001), age at deafness (p = 0.0001), and age at first surgery (p = 0.0001). Patients presenting with VS related symptoms had significantly (p < 0.05) worse hearing values at presentation and after surgery. These patients also exhibited higher growth rates and tumor volumes compared to patients with non-VS related presenting symptoms, but this difference did not reach the significance level of p < 0.05. Due to the late appearance of these symptoms, the time of beginning hearing loss, surgery and deafness is significantly delayed (p < 0.05) compared to patients not presenting with VS. In summary, age at onset and type of presenting symptom provide excellent prognostic parameters for predicting VS- and hearing-related clinical course. Full article
(This article belongs to the Special Issue Schwannomas – So-Called Benign Nervous System Tumors)
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7 pages, 1826 KiB  
Correction
Correction: Gonçalves et al. Macrophage and Lymphocyte Infiltration Is Associated with Volumetric Tumor Size but Not with Volumetric Growth in the Tübingen Schwannoma Cohort. Cancers 2021, 13, 466
by Vítor Moura Gonçalves, Elisa-Maria Suhm, Vanessa Ries, Marco Skardelly, Ghazaleh Tabatabai, Marcos Tatagiba, Jens Schittenhelm and Felix Behling
Cancers 2023, 15(2), 377; https://doi.org/10.3390/cancers15020377 - 6 Jan 2023
Viewed by 1133
Abstract
The authors would like to make a correction to their published paper [...] Full article
(This article belongs to the Special Issue Schwannomas – So-Called Benign Nervous System Tumors)
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