A literature review on surgery for cervical vagal schwannomas

Cervical vagal schwannoma is a benign, slow-growing mass, often asymptomatic, with a very low lifetime risk of malignant transformation in general population, but diagnosis is still a challenge. Surgical resection is the treatment of choice even if its close relationship with nerve fibres, from which it arises, threats vagal nerve preservation. We present a case report and a systematic review of literature. All studies on surgical resection of cervical vagal schwannoma have been reviewed. Papers matching the inclusion criteria (topic on surgical removal of cervical vagal schwannoma, English language, full text available) were selected. Fifty-three patients with vagal neck schwannoma submitted to surgery were identified among 22 studies selected. Female/male ratio was 1.5 and median age 44 years. Median diameter was 5 cm (range 2 to 10). Most schwannoma were asymptomatic (68.2%) and received an intracapsular excision (64.9%). Postoperative symptoms were reported in 22.6% of patients. Cervical vagal schwannoma is a benign pathology requiring surgical excision, but frequently postoperative complications can affect patients lifelong, so, surgical indications should be based carefully on the balance between risks and benefits

Vestibular schwannoma and ipsilateral endolymphatic hydrops: an unusual association

Vestibular schwannoma and endolymphatic hydrops are two conditions that may present with similar audio-vestibular symptoms. The association of the two in the same patient represents an unusual nding that may lead clinicians to errors and delays in diagnosis and clinical management of affected subjects. We discuss the case of a patient with an intrameatal vestibular schwannoma reporting symptoms suggestive for ipsilateral endolymphatic hydrops. The patient presented with uctuating hearing loss, tinnitus, and acute rotatory vertigo episodes, and underwent a full audiological evaluation and imaging of the brain with contrast-enhanced Magnetic Resonance Imaging. Clinical audio-vestibular and radiological examination con rmed the presence of coexisting vestibular schwannoma and endolymphatic hydrops. Hydrops was treated pharmacologically; vestibular schwannoma was monitored over time with a wait and scan protocol through conventional MRI. The association of vestibular schwannoma and endolymphatic hydrops is rare, but represents a possible nding in clinical practice. It is therefore recommended investigating the presence of inner ear disorders in patients with vestibular schwannoma and, similarly, to exclude the presence of this condition in patients with symptoms typical of inner ear disorders

Ancient Schwannoma of the Cauda Equina: our experience and review of the literature

Ancient schwannomas (AS) are exceedingly rare variant of common schwannomas (CS). Only two cases involving the cauda equina region have been previously reported in literature. AS are typically associated with a higher histological degree of degenerative changes (Antoni B areas). It is of peculiar importance, according to our opinion, to outline that, because of their extremely slow growth (which explains the increase of the degenerative changes in respect to the CS) and their typical soft consistency in respect to their standard counterparts, AS usually imply an even better prognosis

Surgical treatment of sporadic vestibular schwannoma in a series of 1006 patients. Trattamento chirurgico degli schwannomi vestibolari: risultati su una serie di 1006 pazienti

La gestione dello schwannoma vestibolare (SV) sporadico si è gradualmente evoluta negli ultimi decenni. Lo scopo di questo studio è di analizzare l’evoluzione negli esiti chirurgici dell’exeresi di queste lesioni, realizzata da un team neurotologico tra il 1990 e il 2006, attraverso differenti approcci. È stata eseguita una revisione retrospettica monocentrica dei dati clinici di 1006 pazienti. Al fine di valutare eventuali modifiche e progressi, il periodo di 17 anni è stato diviso in tre periodi, ciascuno comprendente rispettivamente 268 SV (1990- 1996), 299 SV (1997-2001), e 439 SV (2002-2006). Il follow-up medio è stato di 5,9 ± 2,4 anni. Complessivamente l’asportazione totale è stata ottenuta nel 99,4% dei casi. Il tasso di mortalità è stato dello 0,3%, la meningite e la perdita di liquido cefalo rachidiano (LCR) sono stati osservati nel 1,2% e il 9% dei casi, rispettivamente. La frequenza della perdita di LCR è diminuita dal 11,6% al 7,1% tra il primo e dell’ultimo periodo (p < 0,01) e la revisione chirurgica dal 3,4% al 0,9% (p < 0,05). Il nervo facciale è stato anatomicamente conservato nel 97,7% dei casi. Ad un anno, una buona funzione del nervo facciale è stata osservata nel 85,1% dei pazienti (I e II grado HouseBrackmann), con una variazione tra il primo e l’ultimo periodo che andava dal 78,4% al 87,6% (p < 0,05). Ad un anno post-operatorio la conservazione dell’udito è stata ottenuta nel 61,6% dei pazienti, passando dal 50,9% del primo periodo, al 69,0% del periodo piú recente (p < 0,05) (classe A + B + C dalla classificazione AAO-HNS). L’udito utile (classe A + B) è stato conservato nel 33,5% dei casi complessivamente, con percentuali comprese tra il 21,8% e 42% nel primo e nell’ultimo periodo rispettivamente (p < 0,01). Gli esiti chirurgici dell’asportazion dello schwannoma vestibolare sporadico sono migliorati negli anni per quanto riguarda i risultati funzionali del nervo facciale, la conservazione dell’udito, le perdite di liquido cefalorachidiano, principalmente grazie all’esperienza del team neurotologico. I risultati funzionali dopo la rimozione microchirurgica completa SV di grandi dimensioni dipendono dall’ esperienza maturata sulle lesioni di piccole dimensioniThe management of sporadic vestibular schwannoma (VS) has evolved in the last decades. The aim of this study was to analyse the evolution in surgical outcomes of VSs operated by a neurotological team between 1990 and 2006 by different approaches. A monocentric retrospective review of medical charts of 1006 patients was performed. In order to assess eventual changes and progress, the 17-years period was divided in three periods, each one comprehending 268 VS (1990-1996), 299 VS (1997-2001), and 439 VS (2002-2006). Mean follow-up was 5.9 ± 2.4 years. Overall, complete VS removal was achieved in 99.4% of cases. Mortality rate was 0.3%, meningitis and CSF leaks were observed in 1.2 % and 9 % of the cases, respectively. CSF leakage decreased from 11.6% to 7.1% between the first and last period (p < 0.01) as well as revision surgery from 3.4 % to 0.9 % (p < 0.05). Facial nerve was anatomically preserved in 97.7% of cases. At one year, a good facial nerve function was observed in 85.1% of patients (grade I and II of House-Brackmann grading scale), which ranged between the first and last period from 78.4% to 87.6% (p <0.05). At one year, hearing preservation was obtained in 61.6% of patients, which increased from the first period to the last one from 50.9% to 69.0% (p < 0.05) (class A+B+C from the AAO-HNS classification). Useful hearing (class A+B) was observed in 33.5% of cases overall, with 21.8% and 42% in the first and last period, respectively (p < 0.01). Surgical outcomes of sporadic vestibular schwannoma have improved concerning facial nerve function outcomes, hearing preservation and cerebrospinal fluid (CSF) leaks, mainly due to the neuro-otological team's experience. Functional results after complete microsurgical removal of large VS depend on experience gained on small VS removal

The changing landscape of vestibular schwannoma diagnosis and management: A cross-sectional study.

ObjectivesTo assess the current state of the diagnosis and management of vestibular schwannoma (VS) as well as treatment trends, and to evaluate the role of treatment setting and various specialists in treatment plan.MethodsPatients diagnosed with VS completed a voluntary and anonymous survey. The questionnaires were distributed through Acoustic Neuroma Association website, Facebook page, and e-mail newsletters from January to March 2017.ResultsIn total, 789 VS patients completed the survey. Of those, 414 (52%) underwent surgery; 224 (28%) underwent radiotherapy; and 121 (15%) were observed. General otolaryngologists diagnosed 62% of responders, followed by primary care (11%) and neurotologists (10%). Patients who underwent surgery were significantly younger and had larger tumors compared to those treated with radiation or observation. The ratio of patients having nonsurgical versus surgical resection changed from 1:2 to 1:1 for the periods of 1979 through 2006 versus 2007 through 2017, respectively. Neurosurgeons (40%) and neurotologists (38%) were the most influential in treatment discussion. Neurotologists (P < 0.001) and general otolaryngologists (P = 0.04) were more influential than neurosurgeons for the decision process in patients with smaller tumors. Patients treated at academic versus nonacademic private institutions reported similar tumor sizes (P = 0.27), treatment decisions (P = 0.09), and decision satisfaction (P = 0.78).ConclusionThere is a continuing trend toward nonsurgical management, with approximately half of the patients opting for nonsurgical management. In this cohort, the patients commonly presented with otologic symptoms and otolaryngologists made the most diagnoses. Neurotologists and neurosurgeons were the most influential in treatment discussion.Level of evidenceNA Laryngoscope, 130:482-486, 2020

Dosimetric Analysis of Neural and Vascular Structures in Skull Base Tumors Treated with Stereotactic Radiosurgery.

Objective To examine the relationship between the prescribed target dose and the dose to healthy neurovascular structures in patients with vestibular schwannomas treated with stereotactic radiosurgery (SRS). Study Design Case series with chart review. Setting SRS center from 2011 to 2013. Subjects Twenty patients with vestibular schwannomas treated at the center from 2011 to 2013. Methods Twenty patients with vestibular schwannomas were included. The average radiation dose delivered to healthy neurovascular structures (eg, carotid artery, basilar artery, facial nerve, trigeminal nerve, and cochlea) was analyzed. Results Twenty patients with vestibular schwannomas who were treated with fused computed tomography/magnetic resonance imaging-guided SRS were included in the study. The prescribed dose ranged from 10.58 to 17.40 Gy over 1 to 3 hypofractions to cover 95% of the target tumor volume. The mean dose to the carotid artery was 5.66 Gy (95% confidence interval [CI], 4.53-6.80 Gy), anterior inferior cerebellar artery was 8.70 Gy (95% CI, 4.54-12.86 Gy), intratemporal facial nerve was 3.76 Gy (95% CI, 3.04-4.08 Gy), trigeminal nerve was 5.21 Gy (95% CI, 3.31-7.11 Gy), and the cochlea was 8.70 Gy (95% CI, 7.81-9.59 Gy). Conclusions SRS for certain vestibular schwannomas can expose the anterior inferior cerebellar artery (AICA) and carotid artery to radiation doses that can potentially initiate atherosclerotic processes. The higher doses to the AICA and carotid artery correlated with increasing tumor volume. The dose delivered to other structures such as the cochlea and intratemporal facial nerve appears to be lower and much less likely to cause immediate complications when shielded

Simultaneous Contralateral Vestibular Schwannoma and Middle Ear Paraganglioma Tumor

To the best of our knowledge, only 2 cases of a simultaneous contralateral vestibular schwannoma (VS) and middle ear paraganglioma (MEP) have previously been reported in literature. We report the third case observed in a 43-year-old male, who presented with an 11-year history of right-sided hearing loss and a 1-year history of left-sided pulsatile tinnitus. A magnetic resonance imaging (MRI) showed a VS on the right side and computer tomography (CT) identified a Fisch type A1 paraganglioma on the left side. The VS was treated using a translabyrinthine approach and the MEP was kept under radiological observation for 1 year. Due to the growth of the MEP (Fisch type A2), it was treated with excision via a retroauricular approach. Our case was very challenging because there was a different and important pathology on each side, both carrying a risk of deafness as a consequence of the disease and/or the treatments

Schwannoma of the tongue in a child

A schwannoma or neurilemmoma is a benign, slow growing, usually solitary and encapsulated tumour originating from Schwann cells of the nerve sheath. Approximately 25-40% of all schwannomas are seen in the soft tissues of the head and neck, often originate from the acoustic nerve. Intraoral schwannomas are rare and account for 1% of schwannomas of the head and neck region. We report the case of a 10-year-old boy diagnosed with a schwannoma of the tongue. The purpose of this report is to emphasize the possibility of diagnosing schwannoma among all other lingual lesions in children. The disease itself was diagnosed histologically after complete surgical excision. Five years after surgical treatment, the patient is without signs of recurrence. This paper highlights the importance of a multidisciplinary approach in the diagnosis and surgical treatment of this very rare entity

Patient Decision Making in Vestibular Schwannoma: A Survey of the Acoustic Neuroma Association.

Objective To assess the decision-making process of patients with vestibular schwannoma (VS). Study Design Patients with VS completed a voluntary survey over a 3-month period. Setting Surveys were distributed online through email, Facebook, and member website. Subjects and Methods All patients had a diagnosis of VS and were members of the Acoustic Neuroma Association (ANA). A total of 789 patients completed the online survey. Results Of the 789 participants, 474 (60%) cited physician recommendation as a significant influential factor in deciding treatment. In our sample, 629 (80%) saw multiple VS specialists and 410 (52%) sought second opinions within the same specialty. Of those who received multiple consults, 242 (59%) of patients reported receiving different opinions regarding treatment. Those undergoing observation spent significantly less time with the physician (41 minutes) compared to surgery (68 minutes) and radiation (60 minutes) patients ( P < .001). A total of 32 (4%) patients stated the physician alone made the decision for treatment, and 29 (4%) felt they did not understand all possible treatment options before final decision was made. Of the 414 patients who underwent surgery, 66 (16%) felt they were pressured by the surgeon to choose surgical treatment. Conclusion Deciding on a proper VS treatment for patients can be complicated and dependent on numerous clinical and individual factors. It is clear that many patients find it important to seek second opinions from other specialties. Moreover, second opinions within the same specialty are common, and the number of neurotologists consulted correlated with higher decision satisfaction

Benign retroperitoneal schwannoma presenting as colitis: A case report

We report a case of a patient presenting with clinical , radiological and endoscopic features of colitis due to a compressive left para-aortic mass. Total open surgical excision was performed, which resulted in complete resolution of colitis. Histopathology and immunohistochemistry revealed benign retroperitoneal schwannoma. These neural sheath tumors rarely occur in the retroperitoneum. They are usually asymptomatic but as they enlarge they may compress adjacent structures, which leads to a wide spectrum of nonspecific symptoms, including lumbar pain, headache, secondary hypertension, abdominal pain and renal colicky pain. CT and MR findings show characteristic features, but none are specific. Schwannoma can be isolated sporadic lesions, or associated with schwannomatosis or neurofibromatosis type II (NF2). Although they vary in biological and clinical behavior, their presence is, in nearly every case, due to alterations or absence of the NF2 gene, which is involved in the growth regulation of Schwann cells. Both conditions were excluded by thorough mutation analysis. Diagnosis is based on histopathological examination and immunohistochemistry. Total excision is therapeutic and has a good prognosis. Schwannomatosis and NF2 should be excluded through clinical diagnostic criteria. Genetic testing of NF2 is probably not justified in the presence of a solitary retroperitoneal schwannoma