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Journal of Neurosurgery Nov 2016OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) of the eighth cranial nerve (CN) are exceedingly rare. To date the literature has focused on MPNSTs occurring... (Review)
Review
OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) of the eighth cranial nerve (CN) are exceedingly rare. To date the literature has focused on MPNSTs occurring after radiation therapy for presumed benign vestibular schwannomas (VSs), while MPNSTs arising without prior irradiation have received little attention. The objectives of the current study are to characterize the epidemiology, clinical presentation, disease course, and outcome using a large national cancer registry database and a systematic review of the English literature. Additionally, a previously unreported case is presented. METHODS The authors conducted an analysis of the Surveillance, Epidemiology, and End Results (SEER) database, a systematic review of the literature, and present a case report. Data from all patients identified in the SEER database with a diagnosis of MPNST involving the eighth CN, without a history of prior radiation, were analyzed. Additionally, all cases reported in the English literature between January 1980 and March 2015 were reviewed. Finally, 1 previously unreported case is presented. RESULTS The SEER registries identified 30 cases between 1992 and 2012. The average incidence was 0.017 per 1 million persons per year (range 0.000-0.0687 per year). The median age at diagnosis was 55 years, and 16 (53%) were women. Thirteen cases were diagnosed upon autopsy. Of the 17 cases diagnosed while alive, the median follow-up was 118 days, with 3 deaths (18%) observed. When compared with the incidence of benign VS, 1041 VSs present for every 1 MPNST arising from the eighth CN. Including a previously unreported case from the authors' center, a systematic review of the English literature yielded 24 reports. The median age at diagnosis was 44 years, 50% were women, and the median tumor size at diagnosis was 3 cm. Eleven patients (46%) reported isolated audiovestibular complaints typical for VS while 13 (54%) exhibited facial paresis or other signs of a more aggressive process. Treatment included microsurgery alone, microsurgery with adjuvant radiation, or microsurgery with chemoradiation. Sixty-one percent of patients receiving treatment experienced recurrence, 22% of which were diagnosed with drop metastases to the spine. Ultimately, 13 patients (54%) died of progressive disease at a median of 3 months following diagnosis. The ability to achieve gross-total resection was the only feature that was associated with improved disease-specific survival. CONCLUSIONS MPNSTs of the eighth CN are extremely rare and portend a poor prognosis. Nearly half of patients initially present with findings consistent with a benign VS, often making an early diagnosis challenging. In light of these data, early radiological and clinical follow-up should be considered in those who elect nonoperative treatment, particularly in patients with a short duration of symptoms or atypical presentation. These data also provide a baseline rate of malignancy that should be considered when estimating the risk of malignant transformation following stereotactic radiosurgery for VS.
Topics: Adult; Cranial Nerve Neoplasms; Female; Humans; Male; Middle Aged; Nerve Sheath Neoplasms; Vestibulocochlear Nerve; Vestibulocochlear Nerve Diseases
PubMed: 26745487
DOI: 10.3171/2015.7.JNS151056 -
Journal of Neuro-oncology Jan 2022Neurofibromatosis type 2 (NF2) is characterized by often bilateral vestibular schwannomas (VS) that result in progressive hearing loss and compression of nearby... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Neurofibromatosis type 2 (NF2) is characterized by often bilateral vestibular schwannomas (VS) that result in progressive hearing loss and compression of nearby brainstem structures causing cranial nerve palsies. Treatment of these tumors remains challenging, as both surgical removal and expectant management can result in symptom progression. Stereotactic radiosurgery (SRS) has been investigated for the management of NF2-associated VS; however, the role, promises, and pitfalls of this treatment modality remain unclear.
METHODS
Ovid MEDLINE, EMBASE, Web of Science, and Cochrane Reviews were searched for studies assessing SRS outcome in NF2-associated VS only. Primary endpoints included tumor control, serviceable hearing, presence of tinnitus, and cranial nerve V and VII symptoms.
RESULTS
A total of 16 studies (589 patients harboring 750 tumors) were analyzed. Clinical tumor control was achieved in 88% of cases (95% CI 80-95%); salvage surgery was needed in 8% (95% CI 4-13%) of cases. Treatment resulted in a worsening of pre-treatment serviceable hearing (OR = 0.26, p < 0.01), increased facial nerve (OR = 1.62, p < 0.01) and trigeminal nerve (OR = 1.42, p = 0.07) impairment. The incidence of vestibular symptoms and hydrocephalus were not consistently reported and thus could not be assessed.
CONCLUSIONS
The treatment of NF2-associated VS continues to pose a challenge, as current SRS regimens result in impaired hearing and worse cranial nerve comorbidities, despite achieving high tumor control. It remains unclear if these findings have to be regarded as treatment complications or, rather, continued disease progression.
Topics: Hearing Loss; Humans; Neurofibromatosis 2; Neuroma, Acoustic; Radiosurgery; Treatment Outcome
PubMed: 35040021
DOI: 10.1007/s11060-021-03910-8 -
Neurosurgical Focus Sep 2012Various studies report outcomes of vestibular schwannoma (VS) surgery, but few studies have compared outcomes across the various approaches. The authors conducted a... (Review)
Review
OBJECT
Various studies report outcomes of vestibular schwannoma (VS) surgery, but few studies have compared outcomes across the various approaches. The authors conducted a systematic review of the available data on VS surgery, comparing the different approaches and their associated complications.
METHODS
MEDLINE searches were conducted to collect studies that reported information on patients undergoing VS surgery. The authors set inclusion criteria for such studies, including the availability of follow-up data for at least 3 months, inclusion of preoperative and postoperative audiometric data, intraoperative monitoring, and reporting of results using established and standardized metrics. Data were collected on hearing loss, facial nerve dysfunction, persistent postoperative headache, CSF leak, operative mortality, residual tumor, tumor recurrence, cranial nerve (CN) dysfunction involving nerves other than CN VII or VIII, and other neurological complications. The authors reviewed data from 35 studies pertaining to 5064 patients who had undergone VS surgery.
RESULTS
The analyses for hearing loss and facial nerve dysfunction were stratified into the following tumor categories: intracanalicular (IC), size (extrameatal diameter) < 1.5 cm, size 1.5-3.0 cm, and size > 3.0 cm. The middle cranial fossa approach was found to be superior to the retrosigmoid approach for hearing preservation in patients with tumors < 1.5 cm (hearing loss in 43.6% vs 64.3%, p < 0.001). All other size categories showed no significant difference between middle cranial fossa and retrosigmoid approaches with respect to hearing loss. The retrosigmoid approach was associated with significantly less facial nerve dysfunction in patients with IC tumors than the middle cranial fossa method was; however, neither differed significantly from the translabyrinthine corridor (4%, 16.7%, 0%, respectively, p < 0.001). The middle cranial fossa approach differed significantly from the translabyrinthine approach for patients with tumors < 1.5 cm, whereas neither differed from the retrosigmoid approach (3.3%, 11.5%, and 7.2%, respectively, p = 0.001). The retrosigmoid approach involved less facial nerve dysfunction than the middle cranial fossa or translabyrinthine approaches for tumors 1.5-3.0 cm (6.1%, 17.3%, and 15.8%, respectively; p < 0.001). The retrosigmoid approach was also superior to the translabyrinthine approach for tumors > 3.0 cm (30.2% vs 42.5%, respectively, p < 0.001). Postoperative headache was significantly more likely after the retrosigmoid approach than after the translabyrinthine approach, but neither differed significantly from the middle cranial fossa approach (17.3%, 0%, and 8%, respectively; p < 0.001). The incidence of CSF leak was significantly greater after the retrosigmoid approach than after either the middle cranial fossa or translabyrinthine approaches (10.3%, 5.3%, 7.1%; p = 0.001). The incidences of residual tumor, mortality, major non-CN complications, residual tumor, tumor recurrence, and dysfunction of other cranial nerves were not significantly different across the approaches.
CONCLUSIONS
The middle cranial fossa approach seems safest for hearing preservation in patients with smaller tumors. Based on the data, the retrosigmoid approach seems to be the most versatile corridor for facial nerve preservation for most tumor sizes, but it is associated with a higher risk of postoperative pain and CSF fistula. The translabyrinthine approach is associated with complete hearing loss but may be useful for patients with large tumors and poor preoperative hearing.
Topics: Humans; MEDLINE; Neuroma, Acoustic; Neurosurgical Procedures; Postoperative Complications
PubMed: 22937848
DOI: 10.3171/2012.6.FOCUS12163 -
American Journal of Otolaryngology 2021The anti-angiogenic agent bevacizumab is currently the only drug used clinically for neurofibromatosis type 2-related vestibular schwannomas (NF2-VS). Though benefits... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The anti-angiogenic agent bevacizumab is currently the only drug used clinically for neurofibromatosis type 2-related vestibular schwannomas (NF2-VS). Though benefits have been demonstrated in several cases, the standardized dosage remains unclear.
OBJECTIVE
Our meta-analysis was performed to systematically and comprehensively investigate the reliability and toxicity of bevacizumab in the treatment of NF2-VS, with particular emphasis on the impact of dosage.
METHODS
The literature search was conducted for studies providing data on patients treated with bevacizumab for NF2-VS across PubMed, Embase, and Cochrane Library until December 31, 2020. Two reviewers extracted the incidence rate of results independently. Then we calculated and pooled unadjusted incidence rate with 95% CIs for each study. The subgroups analyzed were conducted.
RESULTS
Fourteen citations (prospective or retrospective observational cohort studies) were eligible based on data from a total of 247 patients with NF2 and 332 related VSs. The pooled results showed that the radiographic response rate (RRR) was 30% [95% CI (20%-42%)], the hearing response rate (HRR) was 32% [95% CI (21%-45%)]. The incidence of major complications was: hypertension 29% [95% CI (23%-35%)], proteinuria 30% [95% CI (18%-44%)], menstrual disorders 44% [95% CI (16%-73%)], hemorrhage 14% [95% CI (4%-26%)], grade3/4 events 12% [95% CI (4%-22%)].
CONCLUSIONS
Nearly one-third of NF2-VS patients may benefit significantly from bevacizumab due to hearing improvement and tumor reduction. Menstrual disorders were the most common adverse events. The high-dose regimen didn't show better efficacy, but results varied considerably according to age.
Topics: Adult; Age Factors; Bevacizumab; Dose-Response Relationship, Drug; Female; Hearing; Hemorrhage; Humans; Hypertension; Male; Menstruation Disturbances; Neurofibromatosis 2; Neuroma, Acoustic; Prospective Studies; Proteinuria; Retrospective Studies; Vestibulocochlear Nerve; Young Adult
PubMed: 34214711
DOI: 10.1016/j.amjoto.2021.103148 -
Journal of Radiosurgery and SBRT 2019Vestibular schwannomas (VS) are benign tumors with a slow growth rate. There exists controversy regarding whether patients should receive upfront observation, SRS, FSRT,...
INTRODUCTION
Vestibular schwannomas (VS) are benign tumors with a slow growth rate. There exists controversy regarding whether patients should receive upfront observation, SRS, FSRT, or surgery at the time of diagnosis. For patients declining resection, this systematic review evaluates the risks and benefits between observation and SRS upon diagnosis of VS.
METHODS
Published studies on VS (including acoustic neuromas) were systematically reviewed for clinical series including patients with newly/recently diagnosed unilateral VS. Studies that included patients with previous treatment for the VS or focused on patients with neurofibromatosis (or other genetic conditions) were excluded. Review articles and systematic reviews were excluded but reviewed for relevant references that would otherwise meet search criteria.
RESULTS
Most patients electing observation underwent their first surveillance scan 6 months after initial diagnosis, then annually or every-other-year thereafter. Follow up was similar for patients receiving radiosurgery. The follow up period varied between studies as not all studies published at a specific time point. Observation alone conferred a radiographic tumor control rate of 65% and serviceable hearing in 71.3% at by the end of the follow up period in the reviewed studies, and 34% of patients initially opting for observation went on to ultimately elect for treatment. Initial radiosurgery resulted in a tumor control rate of 97% and serviceable hearing rate of 73.8% at by the end of the follow up period. Radiosurgery resulted in improved tumor control at the end of the follow up period (p < 0.0001), and serviceable hearing did not statistically differ (p = 0.69). There is an early risk of progressive hearing decline on patients who chose initial observation.
CONCLUSION
Based on the available published data, observation after initial diagnosis is appropriate for many patients with VS without symptomatic brainstem compression. However, initial observation does risk early progressive hearing dysfunction and patients with serviceable hearing at diagnosis may benefit from early therapy. Long term follow up is critical, and an algorithm to better risk-stratify patients with newly diagnosed VS is underway.
PubMed: 31641546
DOI: No ID Found -
BioMed Research International 2016. Vestibular schwannomas (VS) are benign tumours of the vestibular nerve and can lead to hearing loss, tinnitus, vertigo, facial palsy, and brainstem compression.... (Meta-Analysis)
Meta-Analysis
. Vestibular schwannomas (VS) are benign tumours of the vestibular nerve and can lead to hearing loss, tinnitus, vertigo, facial palsy, and brainstem compression. Audiovestibular diagnostic tests are essential for detection and treatment planning. . Medline was used to perform a systematic literature review with regard to how audiovestibular test parameters correlate with symptoms, tumour size, and tumour location. . The auditory brainstem response can be used to diagnose retrocochlear lesions caused by VS. Since hearing loss correlates poorly with tumour size, a retrocochlear lesion is probably not the only cause for hearing loss. Also cochlear mechanisms seem to play a role. This can be revealed by abnormal otoacoustic emissions, despite normal ABR and new MRI techniques which have demonstrated endolymphatic hydrops of the inner ear. Caloric and head impulse tests show frequency specific dynamics and vestibular evoked myogenic potentials may help to identify the location of the tumour regarding the involved nerve parts. . In order to preserve audiovestibular function in VS, it is important to stop the growth of the tumour and to avoid degenerative changes in the inner ear. A detailed neurotological workup helps to diagnose VS of all sizes and can also provide useful prognostic information.
Topics: Evoked Potentials, Auditory, Brain Stem; Hearing Loss; Humans; Magnetic Resonance Imaging; Neuroma, Acoustic; Vertigo; Vestibular Function Tests; Vestibule, Labyrinth
PubMed: 27747231
DOI: 10.1155/2016/4980562 -
Acta Neurochirurgica Jun 2017Repeated controlled studies have revealed that stereotactic radiosurgery is better than microsurgery for patients with vestibular schwannoma (VS) <3 cm in need of... (Comparative Study)
Comparative Study Review
OBJECTIVE
Repeated controlled studies have revealed that stereotactic radiosurgery is better than microsurgery for patients with vestibular schwannoma (VS) <3 cm in need of intervention. In this systematic review we aimed to compare results from single-fraction stereotactic radiosurgery (SRS) to fractionated stereotactic radiotherapy (FSRT) for patients with VS.
DATA SOURCES AND ELIGIBILITY CRITERIA
We systematically searched MEDLINE, Web of Science, Embase and Cochrane and screened relevant articles for references. Publications from 1995 through 2014 with a minimum of 50 adult (>18 years) patients with unilateral VS, followed for a median of >5 years, were eligible for inclusion. After screening titles and abstracts of the 1094 identified articles and systematically reviewing 98 of these articles, 19 were included.
INTERVENTION
Patients with unilateral VS treated with radiosurgery were compared to patients treated with fractionated stereotactic radiotherapy.
RESULTS
No randomized controlled trial (RCT) was identified. None of the identified controlled studies comparing SRS with FSRT were eligible according to the inclusion criteria. Nineteen case series on SRS (n = 17) and FSRT (n = 2) were included in the systematic review. Loss of tumor control necessitating a new VS-targeted intervention was found in an average of 5.0% of the patients treated with SRS and in 4.8% treated with FSRT. Mean deterioration ratio for patients with serviceable hearing before treatment was 49% for SRS and 45% for FSRT, respectively. The risk for facial nerve deterioration was 3.6% for SRS and 11.2% for FSRT and for trigeminal nerve deterioration 6.0% for SRS and 8.4% for FSRT. Since these results were obtained from case series, a regular meta-analysis was not attempted.
CONCLUSION
SRS and FSRT are both noninvasive treatment alternatives for patients with VS with low rates of treatment failure in need of rescue therapy. In this selection of patients, the progression-free survival rates were on the order of 92-100% for both treatment options. There is a lack of high-quality studies comparing radiation therapy alternatives for patients with VS. Finally, 19 articles reported long-term tumor control after SRS, while only 2 articles reported long-term FSRT results, making effect estimates more uncertain for FSRT.
Topics: Adult; Aged; Disease-Free Survival; Dose Fractionation, Radiation; Facial Nerve; Female; Humans; Male; Microsurgery; Neuroma, Acoustic; Postoperative Complications; Radiosurgery; Trigeminal Nerve
PubMed: 28409393
DOI: 10.1007/s00701-017-3164-6 -
Journal of Neuro-oncology Sep 2013The aim of this study was to review the literature on quality of life among adult patients with neurofibromatosis 1, neurofibromatosis 2 and schwannomatosis, and to... (Review)
Review
The aim of this study was to review the literature on quality of life among adult patients with neurofibromatosis 1, neurofibromatosis 2 and schwannomatosis, and to identify the specific aspects of quality of life that were studied and reported in this population. We also set out to report predictors of quality of life. Published research reports were included if they described quality of life in this population and met methodological quality according to a list of predefined criteria. Eight studies (7 in NF1, 1 in NF2, 0 in schwannomatosis), conducted between 2001 and 2013, met inclusion criteria. The methodological quality of the eight studies was mostly high according to ratings by predefined criteria. Most studies reported that patients with NF experience decreased quality of life when compared to the general population. Visibility and disease severity were strong predictors of skin-specific quality of life in NF1 patients. However, the majority of findings regarding predictors of quality of life were weak or inconclusive. Given the decreased quality of life in NF patients, it is important to examine more comprehensively the psychosocial factors in this population, especially in patients with NF2 and schwannomatosis. Mind body interventions that address these domains may provide comprehensive and efficacious long term treatment.
Topics: Adult; Humans; Neurilemmoma; Neurofibromatoses; Neurofibromatosis 1; Neurofibromatosis 2; Quality of Life; Review Literature as Topic; Skin Neoplasms
PubMed: 23817811
DOI: 10.1007/s11060-013-1195-2 -
Hearing preservation after stereotactic radiosurgery for vestibular schwannoma: a systematic review.Journal of Clinical Neuroscience :... Jun 2009Radiosurgery has evolved into an effective alternative to microsurgical resection in the treatment of patients with vestibular schwannoma. We performed a systematic... (Review)
Review
Radiosurgery has evolved into an effective alternative to microsurgical resection in the treatment of patients with vestibular schwannoma. We performed a systematic analysis of the literature in English on the radiosurgical treatment of vestibular schwannoma patients. A total of 254 published studies reported assessable and quantifiable outcome data of patients undergoing radiosurgery for vestibular schwannomas. American Association of Otolaryngology-Head and Neck Surgery (AAO-HNS) class A or B and Gardner-Robertson (GR) classification I or II were defined as having preserved hearing. A total of 5825 patients (74 articles) met our inclusion criteria. Practitioners who delivered an average dose of 12.5 Gy as the marginal dose reported having a higher hearing preservation rate (12.5 Gy=59% vs. >12.5 Gy=53%, p=0.0285). Age of the patient was not a significant prognostic factor for hearing preservation rates (<65 years=58% vs. >65 years=62%; p=0.4317). The average overall follow-up was 41.2 months. Our data suggest that an overall hearing preservation rate of about 57% can be expected after radiosurgical treatment, and patients treated with 12.5 Gy were more likely to have preserved hearing.
Topics: Age Factors; Aged; Cochlear Nerve; Dose-Response Relationship, Radiation; Hearing Loss; Humans; Iatrogenic Disease; Middle Aged; Neuroma, Acoustic; Postoperative Complications; Radiosurgery; Vestibular Nerve
PubMed: 19303780
DOI: 10.1016/j.jocn.2008.09.023 -
Acta Neurochirurgica Apr 2023Hybrid peripheral nerve sheath tumors (HPNST) are a newly recognized class of peripheral nerve sheath tumor, composed of at least two areas characteristic of...
PURPOSE
Hybrid peripheral nerve sheath tumors (HPNST) are a newly recognized class of peripheral nerve sheath tumor, composed of at least two areas characteristic of perineurioma, schwannoma, or neurofibroma. The literature consists only of case reports and small series; therefore, we present an illustrative case and an analysis of all reported cases of HPNST with a perineurioma component in the literature.
METHODS
A systematic search of the literature was performed to identify all reported cases of hybrid perineurioma-schwannoma or perineurioma-neurofibroma in the world's literature. Individual cases were analyzed for demographics, clinical features, imaging, and outcomes.
RESULTS
A total of 159 cases were identified across 41 studies. Hybrid tumors tended to present in mid-adulthood (median 38.5 years), predominantly affected females (57%, 89/156), as a painless (63%, 63/100) mass, or swelling. Ten patients (10/74, 14%) had a history of neurofibromatosis 1, and 2 patients a history of neurofibromatosis 2 (2/74, 3%). The majority (78%, 122/157) of cases occurred superficially, most commonly in the lower extremity (25%, 39/157). Perineurioma-schwannoma was the most reported (86%, 137/159) pathologic diagnosis, with 3 cases presenting with malignant features. Two cases reocurred after resection.
CONCLUSION
HPNST tend to occur in mid-adulthood and present as slowly progressive, painless, superficial masses, with a heterogeneous appearance on imaging. These entities pose a unique diagnostic challenge and likely remain under-recognized in the literature and current clinical practice. They pose low risk of recurrence or malignant transformation, and future work regarding the association with neurofibromatosis and genetic profiles is needed.
Topics: Female; Humans; Adult; Nerve Sheath Neoplasms; Neurilemmoma; Neurofibroma; Neurofibromatosis 2; Neurofibromatosis 1
PubMed: 36396843
DOI: 10.1007/s00701-022-05413-5