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World Neurosurgery Jun 2022Glomus jugulare tumors (GJTs) are benign paragangliomas of the jugular foramen. Traditional management of these tumors involves surgical resection; however, considering... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Glomus jugulare tumors (GJTs) are benign paragangliomas of the jugular foramen. Traditional management of these tumors involves surgical resection; however, considering the proximity of these tumors to important neurovasculature, stereotactic radiosurgery (SRS) may be an appropriate noninvasive treatment to consider. The aim of this meta-analysis was to evaluate SRS as a treatment option for GJTs.
METHODS
An online search using PubMed, Web of Science, Scopus, and Cochrane databases was performed in March 2019 for articles on radiosurgery treatment of GJTs. The screening process followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
RESULTS
The final analysis comprised 23 studies including 460 patients. Average rates of tinnitus, hearing loss, and lower cranial nerve deficit as presenting symptoms were 56% (95% confidence interval [CI], 46%-66%), 56% (95% CI, 44%-68%), and 42% (95% CI, 31%-54%), respectively. Overall clinical status improvement rate after treatment was 47% (95% CI, 37%-57%). Rates of tinnitus, hearing loss, and lower cranial nerve improvement after treatment were 54% (95% CI, 44%-63%), 28% (95% CI, 19%-40%), and 22% (95% CI, 11%-39%), respectively. The mean follow-up time across studies was 47 months (range, 4-268 months). The aggregate tumor control rate at the time of follow-up was 95% (95% CI, 93%-97%).
CONCLUSIONS
The tumor control rate of 95% and 47% symptomatic improvement suggest that SRS may be a suitable treatment modality for these hypervascular skull base tumors. Future studies are warranted to further evaluate the potential role of SRS in management of GJTs.
Topics: Follow-Up Studies; Glomus Jugulare Tumor; Humans; Paraganglioma; Radiosurgery; Retrospective Studies; Tinnitus; Treatment Outcome
PubMed: 35189418
DOI: 10.1016/j.wneu.2022.02.043 -
World Neurosurgery Feb 2023Tympanojugular paragangliomas (TJPs) are slow-growing tumors arising within the middle ear or jugular foramen. The development of modified skull base approaches and the... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Tympanojugular paragangliomas (TJPs) are slow-growing tumors arising within the middle ear or jugular foramen. The development of modified skull base approaches and the increasing use of stereotactic radiosurgery have provided more modern techniques in the management of TJPs. Several factors dictating approach selection, and resulting clinical outcomes have been inconsistently described.
METHODS
A systematic review of the literature describing modern management of complex TJPs was performed and summarized. A random-effects meta-analysis was performed to describe the rate of tumor control, complications, and symptom improvement in patients undergoing radiosurgery or surgical resection.
RESULTS
Nineteen studies were identified with a total of 852 TJP patients. A minority (153 patients) underwent radiosurgery while 699 underwent surgery. On meta-analysis, there was a 3.5% (95% confidence interval [CI]: 0.5%-6.4%) tumor growth rate following radiosurgery and 3.9% (95% confidence interval [CI]: 1.8%-6.0%) recurrence rate in surgical resection, with no significant moderator effect between the 2 groups (P = 0.9046). Complication rate for radiosurgery was 7.6% (95% CI: 2.8%-12.4%), differing significantly from surgical complication rates of 29.6% (95% CI: 17.1-42.0%, P = 0.0418).
CONCLUSIONS
Stereotactic radiosurgery and surgical resection for TJPs have similar rates of tumor recurrence. Radiation is associated with less risk and lower morbidity, yet there is comparably modest reduction of the tumor size. In sum, the data suggest that radiosurgery is a reasonable management option for patients with minimal symptoms who are high risk for surgery. Microsurgical resection should be reserved for patients with lower cranial neuropathies or those who have failed radiation treatment.
Topics: Humans; Glomus Jugulare Tumor; Neoplasm Recurrence, Local; Paraganglioma; Cranial Nerve Diseases; Radiosurgery; Treatment Outcome; Retrospective Studies
PubMed: 36400356
DOI: 10.1016/j.wneu.2022.11.037 -
Current Oncology (Toronto, Ont.) Jul 2022Craniovertebral junction (CVJ) schwannomas are rare, with surgery and stereotactic radiosurgery (SRS) being effective yet challenging options. We systematically reviewed... (Review)
Review
BACKGROUND
Craniovertebral junction (CVJ) schwannomas are rare, with surgery and stereotactic radiosurgery (SRS) being effective yet challenging options. We systematically reviewed the literature on CVJ schwannomas.
METHODS
PubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA statement to include studies reporting CVJ schwannomas. Clinical features, management, and outcomes were analyzed.
RESULTS
We collected 353 patients from 101 included articles. Presenting symptoms were mostly neck pain (30.3%) and headache (26.3%), with most cranial neuropathies involving the XII (31.2%) and X (24.4%) nerves. Most tumors originated from C2 (30.9%) and XII (29.4%) nerves, being extracranial (45.1%) and intradural-extradural (44.2%). Erosion of C1-C2 vertebrae (37.1%), the hypoglossal canal (28.3%), and/or jugular foramen (20.1%) were noted. All tumors were operated, preferably with the retrosigmoid approach (36.5%), with the far-lateral approach (29.7%) or with the posterior approach and cervical laminectomy (26.9%), far-lateral approaches (14.2%), or suboccipital craniotomy with concurrent cervical laminectomy (14.2%). Complete tumor resection was obtained most frequently (61.5%). Adjuvant post-surgery stereotactic radiosurgery was delivered in 5.9% patients. Median follow-up was 27 months (range, 12-252). Symptom improvement was noted in 88.1% of cases, and cranial neuropathies showed improvement in 10.2%. Post-surgical complications occurred in 83 patients (23.5%), mostly dysphagia (7.4%), new cranial neuropathies (6.2%), and cerebrospinal fluid leak (5.9%). A total of 16 patients (4.5%) had tumor recurrence and 7 died (2%), with median overall survival of 2.7 months (range, 0.1-252).
CONCLUSIONS
Microsurgical resection is safe and effective for CVJ schwannomas. Data on SRS efficacy and indications are still lacking, and its role deserves further evaluation.
Topics: Cranial Nerve Diseases; Humans; Neoplasm Recurrence, Local; Neurilemmoma; Radiosurgery
PubMed: 35877244
DOI: 10.3390/curroncol29070384 -
World Neurosurgery Sep 2021Hemangiopericytomas (HPCs) and solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin. Here, the authors present a rare case of anaplastic HPC in the...
BACKGROUND
Hemangiopericytomas (HPCs) and solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin. Here, the authors present a rare case of anaplastic HPC in the jugular foramen (JF). The authors also conduct a systematic review of the literature to examine the current fund of knowledge on JF HPC/SFTs.
METHODS
A systematic MEDLINE search was conducted using key words "hemangiopericytoma" OR "solitary fibrous tumor" AND "jugular foramen" OR "extracranial" OR "skull base." Clinicopathologic characteristics and outcomes of the present case were reviewed and compared with those in the literature.
RESULTS
A 41-year-old male, who had undergone stereotactic radiation therapy 6 years ago for a presumed glomus jugulare tumor, presented to our institution with worsening dysphagia, hoarseness, persistent tongue weakness, and radiographic evidence of tumor progression. The patient underwent uncomplicated gross total resection with sacrifice of the infiltrated hypoglossal nerve. Histopathologic evaluation revealed anaplastic HPC/SFT (World Health Organization grade III). Review of the literature yielded 9 additional cases of JF HPC/SFT in 5 males (56%) and 4 females (44%), with a mean age of 49.6 years old. Patients commonly presented with pain (37.5%) and lower cranial nerve deficits (100%). Preoperative diagnoses included glomus jugulare (n = 2) or JF schwannomas (n = 3). All patients underwent microsurgical resection of the lesion, except for 1 who refused all treatment after diagnostic biopsy.
CONCLUSION
The authors present the only reported case of anaplastic HPC of the JF. The illustrative case and those found on systematic review of the literature highlight the importance of tissue diagnosis and appropriate management.
Topics: Adult; Diagnostic Errors; Glomus Jugulare; Hemangiopericytoma; Humans; Jugular Foramina; Magnetic Resonance Imaging; Male; Radiosurgery
PubMed: 34182175
DOI: 10.1016/j.wneu.2021.06.094 -
The Annals of Otology, Rhinology, and... Dec 2021To report a recalcitrant spontaneous cerebrospinal fluid (CSF) fistula arising from multiple, anatomically-linked lateral skull base defects, and to review the available...
OBJECTIVES
To report a recalcitrant spontaneous cerebrospinal fluid (CSF) fistula arising from multiple, anatomically-linked lateral skull base defects, and to review the available literature to determine optimal techniques for operative repair of congenital CSF fistulae.
METHODS
A patient with recurrent episodes of otologic meningitis was found to have a patent tympanomeningeal fissure, also known as a Hyrtl's fissure, and internal auditory canal (IAC) diverticulum that communicated with the jugular bulb. A systematic review of the literature characterized all reports of spontaneous congenital perilabyrinthine CSF leaks, and all cases of Hyrtl's fissures.
RESULTS
An 11-year-old female was referred for recurrent meningitis. Imaging demonstrated a fistulous connection between the middle ear and IAC diverticulum via the jugular foramen. Specifically, a Hyrtl's fissure was identified, as well as demineralized bone around the jugular bulb. Obliteration of the fissure was initially performed, and a fistula reformed 4 months later. Multifocal CSF egress in the hypotympanum was identified on re-exploration, and middle ear obliteration with external auditory canal (EAC) overclosure was performed. A systematic review of the literature demonstrated 19 cases of spontaneous congenital perilabyrinthine CSF leaks. In total, 6 cases had multiple sources of CSF leak and 2 had history suggestive of intracranial hypertension. All of these noted cases demonstrated leak recurrence. Middle ear obliteration with EAC overclosure was successful in 4 recalcitrant cases.
CONCLUSIONS
Repair of spontaneous congenital perilabyrinthine CSF leaks in cases demonstrating multiple sources of egress or signs of intracranial hypertension should be approached with caution. Middle ear obliteration with EAC overclosure may provide the most definitive management option for these patients, particularly if initial attempt at primary repair is unsuccessful.
Topics: Cerebrospinal Fluid Otorrhea; Child; Ear, Inner; Female; Fistula; Humans; Mastoidectomy; Skull Base; Tomography, X-Ray Computed; Tympanic Membrane
PubMed: 33834882
DOI: 10.1177/00034894211007242 -
Acta Neurochirurgica Feb 2022Non-vestibular schwannomas are relatively rare, with trigeminal and jugular foramen schwannomas being the most common. This is a heterogenous group which requires...
Management of non-vestibular schwannomas in adult patients: a systematic review and consensus statement on behalf of the EANS skull base section Part III: Lower cranial nerve schwannomas, jugular foramen (CN IX, X, XI) and hypoglossal schwannoma (XII).
BACKGROUND
Non-vestibular schwannomas are relatively rare, with trigeminal and jugular foramen schwannomas being the most common. This is a heterogenous group which requires detailed investigation and careful consideration to management strategy. The optimal management for these tumours remains unclear, and there are several controversies. The aim of this paper is to provide insight into the main principles defining management and surgical strategy, in order to formulate a series of recommendations.
METHODS
A task force was created by the EANS skull base section committee along with its members and other renowned experts in the field to generate recommendations for the surgical management of these tumours on a European perspective. To achieve this, the task force performed an extensive systematic review in this field and had discussions within the group. This article is the third of a three-part series describing non-vestibular schwannomas (IX, X, XI, XII).
RESULTS
A summary of literature evidence was proposed after discussion within the EANS skull base section. The constituted task force dealt with the practice patterns that exist with respect to preoperative radiological investigations, ophthalmological assessments, optimal surgical and radiotherapy strategies and follow-up management.
CONCLUSION
This article represents the consensually derived opinion of the task force with respect to the treatment of non-vestibular schwannomas. For each of these tumours, the management paradigm is shifting towards the compromise between function preservation and progression free survival.
Topics: Adult; Cranial Nerve Neoplasms; Cranial Nerves; Humans; Jugular Foramina; Neurilemmoma; Skull Base
PubMed: 34854994
DOI: 10.1007/s00701-021-05072-y -
Brain Sciences Sep 2022Staged surgery strategy was preferred for patients with intra-extracranial communicating jugular foramen paraganglioma (IECJFP). A female patient who presented mild...
Staged surgery strategy was preferred for patients with intra-extracranial communicating jugular foramen paraganglioma (IECJFP). A female patient who presented mild tinnitus, headache, and dizziness, together with preoperative related imaging, was diagnosed with a left intra-extracranial communicating jugular foramen lesion in November 2015 and accepted an initial operation for the intracranial tumor by retrosigmoid approach. The pathologic report was paraganglioma. In November 2021, a subtotal resection of the extracranial tumor was conducted for prominent lower cranial nerves (LCNs) deficit and middle ear involvement by infratemporal approach. In patients with IECJFP accompanied by LCNs deficit and middle ear involvement, an initial surgery for extracranial lesion and a second procedure for intracranial tumor were appropriate. However, the first operation for the intracranial lesion was preferred in IECJFP cases without LCNs deficit and middle ear involvement, as it could remove compression to the neurovascular structure and brain stem, clarify a pathological diagnosis, avoid a CSF leak, and prevent a severe neurological disorder from extracranial lesion excision. Subtotal resection of the extracranial tumor would be performed when lesion became larger combined with obvious LCNs disorder and tympanic cavity involvement. Consideration of specific staged surgical strategy for IECJFP in accordance with preoperative LCNs deficit and tympanic cavity involvement could prevent critical postoperative neurological deficit and improve quality of life in the long term.
PubMed: 36138993
DOI: 10.3390/brainsci12091257 -
Neurosurgery Apr 2024Schwannomas originating from the lower cranial nerves (LCNS) are rare and pose a significant surgical challenge. Resection is the mainstay treatment; however, risk of...
BACKGROUND AND OBJECTIVES
Schwannomas originating from the lower cranial nerves (LCNS) are rare and pose a significant surgical challenge. Resection is the mainstay treatment; however, risk of treatment morbidity is considerable, and the available literature regarding differential treatment outcomes in this vulnerable population is sparse.
METHODS
A single-institution cohort study and systematic literature review of LCNS were performed.
RESULTS
Fifty-eight patients were included: 34 underwent surgical resection and 24 underwent stereotactic radiosurgery (SRS). The median age at diagnosis was 48 years (range 17-74). Presenting symptoms were dysphagia (63%), dysarthria/hypophonia (47%), imbalance (33%), and hearing loss/tinnitus (30%). Tumor size was associated with surgical resection, as compared with initial SRS (4.1 cm vs 1.5 cm, P = .0001). Gross total resection was obtained in 52%, with tumor remnants predominantly localized to the jugular foramen (62%). Post-treatment worsening of symptoms occurred in 68% of surgical and 29% of SRS patients ( P = .003). Postoperative symptoms were mostly commonly hypophonia/hoarseness (63%) and dysphagia (59%). Seven patients (29%) had new neurological issues after SRS treatment, but symptoms were overall milder. The median follow-up was 60 months (range 12-252); 98% demonstrated meaningful clinical improvement. Eighteen surgical patients (53%) underwent adjuvant radiation at a median of 5 months after resection (range 2-32). At follow-up, tumor control was 97% in the surgical cohort and 96% among SRS patients.
CONCLUSION
Although LCNS resection is potentially morbid, most postoperative deficits are transient, and patients achieve excellent tumor control-particularly when paired with adjuvant SRS. For minimally symptomatic patients undergoing surgical intervention, we advise maximally safe resection with intracapsular dissection to preserve nerve integrity where possible. For residual or as a primary treatment modality, SRS is associated with low morbidity and high rates of long-term tumor control.
Topics: Humans; Adolescent; Young Adult; Adult; Middle Aged; Aged; Cohort Studies; Deglutition Disorders; Neurilemmoma; Treatment Outcome; Cranial Nerves; Radiosurgery; Retrospective Studies; Follow-Up Studies
PubMed: 37874134
DOI: 10.1227/neu.0000000000002735 -
Acta Neurochirurgica Feb 2022Non-vestibular schwannomas are relatively rare, with trigeminal and jugular foramen schwannomas being the most common. This is a heterogeneous group which requires...
Management of non-vestibular schwannomas in adult patients: a systematic review and consensus statement on behalf of the EANS skull base section. Part I: oculomotor and other rare non-vestibular schwannomas (I, II, III, IV, VI).
BACKGROUND
Non-vestibular schwannomas are relatively rare, with trigeminal and jugular foramen schwannomas being the most common. This is a heterogeneous group which requires detailed investigation and careful consideration to management strategy. The optimal management for these tumours remains unclear, and there are several controversies. The aim of this paper is to provide insight into the main principles defining management and surgical strategy, in order to formulate a series of recommendations.
METHODS
A task force was created by the EANS skull base section along with its members and other renowned experts in the field to generate recommendations for the surgical management of these tumours on a European perspective. To achieve this, the task force performed an extensive systematic review in this field and had discussions within the group. This article is the first of a three-part series describing non-vestibular schwannomas (I, II, III, IV, VI).
RESULTS
A summary of literature evidence was proposed after discussion within the EANS skull base section. The constituted task force dealt with the practice patterns that exist with respect to pre-operative radiological investigations, ophthalmological assessments, optimal surgical and radiotherapy strategies and follow-up management.
CONCLUSION
This article represents the consensually derived opinion of the task force with respect to the treatment of non-vestibular schwannomas. For each of these tumours, the management of these patients is complex, and for those which are symptomatic tumours, the paradigm is shifting towards the compromise between function preservation and progression-free survival.
Topics: Adult; Consensus; Humans; Neurilemmoma; Radiosurgery; Skull Base
PubMed: 34755208
DOI: 10.1007/s00701-021-05048-y -
Acta Neurochirurgica Feb 2022Non-vestibular schwannomas are relatively rare, with trigeminal and jugular foramen schwannomas being the most common. This is a heterogenous group which requires...
Management of non-vestibular schwannomas in adult patients: a systematic review and consensus statement on behalf of the EANS skull base section Part II: Trigeminal and facial nerve schwannomas (CN V, VII).
BACKGROUND
Non-vestibular schwannomas are relatively rare, with trigeminal and jugular foramen schwannomas being the most common. This is a heterogenous group which requires detailed investigation and careful consideration to management strategy. The optimal management for these tumours remains unclear and there are several controversies. The aim of this paper is to provide insight into the main principles defining management and surgical strategy, in order to formulate a series of recommendations.
METHODS
A task force was created by the EANS skull base section committee along with its members and other renowned experts in the field to generate recommendations for the surgical management of these tumours on a European perspective. To achieve this, the task force performed an extensive systematic review in this field and had discussions within the group. This article is the second of a three-part series describing non-vestibular schwannomas (V, VII).
RESULTS
A summary of literature evidence was proposed after discussion within the EANS skull base section. The constituted task force dealt with the practice patterns that exist with respect to pre-operative radiological investigations, ophthalmological assessments, optimal surgical and radiotherapy strategies, and follow-up management.
CONCLUSION
This article represents the consensually derived opinion of the task force with respect to the treatment of trigeminal and facial schwannoma. The aim of treatment is maximal safe resection with preservation of function. Careful thought is required to select the appropriate surgical approach. Most middle fossa trigeminal schwannoma tumours can be safely accessed by a subtemporal extradural middle fossa approach. The treatment of facial nerve schwannoma remains controversial.
Topics: Adult; Consensus; Cranial Nerve Neoplasms; Facial Nerve; Humans; Neurilemmoma; Retrospective Studies; Skull Base; Treatment Outcome
PubMed: 35079891
DOI: 10.1007/s00701-021-05092-8