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1.
World Neurosurg ; 139: 12-19, 2020 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-32251827

RESUMO

BACKGROUND: Ewing-like sarcoma with capicua transcriptional repressor (CIC) rearrangement is a unique class of undifferentiated round cell sarcomas characterized by CIC-double homeobox 4 gene fusion. Despite showing great histologic resemblance to Ewing sarcomas, they have proved to be a distinct pathological entity from the immunohistochemistry and genetic examinations and the response to treatment. We have presented a case of CIC-rearranged Ewing-like sarcoma with cerebral metastasis managed with operative resection and gamma knife radiosurgery. CASE DESCRIPTION: A 56-year-old woman had initially presented with an ulcerating lesion of the right fifth toe. The histological and immunohistochemical analysis revealed features consistent with CIC-rearranged Ewing-like sarcoma, which was confirmed with genetic analysis. Despite aggressive local control and a multidrug chemotherapy regimen, the patient developed multifocal metastases involving the lungs, femur, and cerebrum. The cerebral lesions were managed with surgery and gamma knife radiosurgery, with mixed results. CONCLUSION: CIC-rearranged Ewing-like sarcomas have recently been recognized as a distinct disease entity with a highly aggressive course. Treatment paradigms have yet to be defined to properly manage such an aggressive pathological process.

2.
Otol Neurotol ; 2020 Apr 07.
Artigo em Inglês | MEDLINE | ID: mdl-32271262

RESUMO

OBJECTIVE: To report and discuss the effectiveness of stereotactic radiosurgery (SRS) or fractionated radiotherapy (FRT) for tumor control following surgical resection of endolymphatic sac tumors (ELST). STUDY DESIGN: Retrospective case series. SETTING: Multi-institutional academic referral centers. PATIENTS: Patients undergoing surgical resection for ELST followed by SRS or FRT. INTERVENTION(S): Surgical resection followed by radiotherapy. MAIN OUTCOME MEASURES: Local tumor control. RESULTS: Two of the five patients experienced tumor recurrence after gross total microsurgical at 78 and 11 months, respectively. The former patient received salvage 3D conformal radiotherapy (3D-CRT) and while the latter patient underwent three courses of salvage SRS for recurrence, two of which were in-field and was disease-free at last follow up. Two additional patients underwent subtotal tumor resection (STR) followed by intensity-modulated radiation therapy (IMRT) and are currently without disease. One patient underwent STR followed by proton-beam therapy (PBT) and was free of disease at most recent follow-up. CONCLUSION: SRS/FRT remains a useful adjuvant for treatment of residual or recurrent ELSTs, where the risk of revision microsurgical resection is high.

3.
Neurosurgery ; 2020 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-32267504

RESUMO

BACKGROUND: Stereotactic radiosurgery (SRS) is a safe and effective treatment for acromegaly. OBJECTIVE: To improve understanding of clinical and dosimetric factors predicting biochemical remission. METHODS: A single-institution cohort study of nonsyndromic, radiation-naïve patients with growth hormone-producing pituitary adenomas (GHA) having single-fraction SRS between 1990 and 2017. Exclusions were treatment with pituitary suppressive medications at the time of SRS, or <24 mo of follow-up. The primary outcome was biochemical remission-defined as normalization of insulin-like growth factor-1 index (IGF-1i) off suppression. Biochemical remission was assessed using Cox proportional hazards. Prior studies reporting IGF-1i were assessed via systematic literature review and meta-analysis using random-effect modeling. RESULTS: A total of 102 patients met study criteria. Of these, 46 patients (45%) were female. The median age was 49 yr (interquartile range [IQR] = 37-59), and the median follow-up was 63 mo (IQR = 29-100). The median pre-SRS IGF-1i was 1.66 (IQR = 1.37-3.22). The median margin dose was 25 Gy (IQR = 21-25); the median estimated biologically effective dose (BED) was 169.49 Gy (IQR = 124.95-196.00). Biochemical remission was achieved in 58 patients (57%), whereas 22 patients (22%) had medication-controlled disease. Pre-SRS IGF-1i ≥ 2.25 was the strongest predictor of treatment failure, with an unadjusted hazard ratio (HR) of 0.51 (95% CI = 0.26-0.91, P = .02). Number of isocenters, margin dose, and BED predicted remission on univariate analysis, but after adjusting for sex and baseline IGF-1i, only BED remained significant-and was independently associated with outcome in continuous (HR = 1.01, 95% CI = 1.00-1.01, P = .02) and binary models (HR = 2.27, 95% CI = 1.39-5.22, P = .002). A total of 24 patients (29%) developed new post-SRS hypopituitarism. Pooled HR for biochemical remission given subthreshold IGF-1i was 2.25 (95% CI = 1.33-3.16, P < .0001). CONCLUSION: IGF-1i is a reliable predictor of biochemical remission after SRS. BED appears to predict biochemical outcome more reliably than radiation dose, but confirmatory study is needed.

4.
Neurosurgery ; 2020 Mar 06.
Artigo em Inglês | MEDLINE | ID: mdl-32140720

RESUMO

Systematic reviews and meta-analyses in the neurosurgical literature have surged in popularity over the last decade. It is our concern that, without a renewed effort to critically interpret and appraise these studies as high or low quality, we run the risk of the quality and value of evidence-based medicine in neurosurgery being misinterpreted. Correspondingly, we have outlined 4 major domains to target in interpreting neurosurgical systematic reviews and meta-analyses based on the lessons learned by a collaboration of clinicians and academics summarized as 4 pearls. The domains of (1) heterogeneity, (2) modeling, (3) certainty, and (4) bias in neurosurgical systematic reviews and meta-analyses were identified as aspects in which the authors' approaches have changed over time to improve robustness and transparency. Examples of how and why these pearls were adapted were provided in areas of cranial neuralgia, spine, pediatric, and neuro-oncology to demonstrate how neurosurgical readers and writers may improve their interpretation of these domains. The incorporation of these pearls into practice will empower neurosurgical academics to effectively interpret systematic reviews and meta-analyses, enhancing the quality of our evidence-based medicine literature while maintaining a critical focus on the needs of the individual patients in neurosurgery.

5.
Int J Neurosci ; : 1-4, 2020 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-32019398

RESUMO

Objective: Intracranial dural arteriovenous fistulas represent pathological connections between dural arteries and dural veins, dural sinuses or meningeal veins in the absence of an intervening capillary bed. They are thought to be acquired secondary to trauma, surgery, sinus thrombosis, venous hypertension or arterial dysplasia. Methods: A 66-year-old Asian female presented with subarachnoid hemorrhage secondary to ruptured 2-mm saccular aneurysm of the left middle cerebral artery associated with fusiform dilatation. It was successfully treated with endovascular coiling. A right frontal external ventricular drain was also placed to treat her hydrocephalus. On post-bleed day 10, she became acutely unresponsive with a fixed and dilated right pupil. Head CT was obtained and revealed an acute right subdural hematoma which was emergently evacuated. Results: No obvious bleeders were identified during surgery. Patient improved and repeat catheter angiography a week later showed a new dural arteriovenous fistula fed by the anterior falcine artery and the middle meningeal artery to a cortical vein draining into the superior sagittal sinus. Conclusion: We hope that the present report will raise awareness to treating physicians to be cognizant of this unusual complication in their differential diagnosis when treating patients with an EVD in place.

6.
Otolaryngol Head Neck Surg ; 162(4): 530-537, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31986971

RESUMO

OBJECTIVE: To ascertain the relationship among vestibular schwannoma (VS) tumor volume, growth, and hearing loss. STUDY DESIGN: Retrospective cohort study. SETTING: Single tertiary center. SUBJECTS AND METHODS: Adults with observed VS and serviceable hearing at diagnosis were included. The primary outcome was the development of nonserviceable hearing as estimated using the Kaplan-Meier method. Associations of tumor volume with baseline hearing were assessed using Spearman rank correlation coefficients. Associations of volume and growth with the development of nonserviceable hearing over time were assessed using Cox proportional hazards models and summarized with hazard ratios (HRs). RESULTS: Of 230 patients with VS and serviceable hearing at diagnosis, 213 had serial volumetric tumor data for analysis. Larger tumor volume at diagnosis was associated with increased pure-tone average (PTA) (P < .001) and decreased word recognition score (WRS) (P = .014). Estimated rates of maintaining serviceable hearing at 6 and 10 years following diagnosis were 67% and 49%, respectively. Larger initial tumor volume was associated with development of nonserviceable hearing in a univariable setting (HR for 1-cm3 increase: 1.36, P = .040) but not after adjusting for PTA and WRS. Tumor growth was not significantly associated with time to nonserviceable hearing (HR, 1.57; P = .14), although estimated rates of maintaining serviceable hearing during observation were poorer in the group that experienced growth. CONCLUSION: Larger initial VS tumor volume was associated with poorer hearing at baseline. Larger initial tumor volume was also associated with the development of nonserviceable hearing during observation in a univariable setting; however, this association was not statistically significant after adjusting for baseline hearing status.

7.
Neurosurgery ; 86(2): 250-256, 2020 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-30980077

RESUMO

BACKGROUND: Stereotactic radiosurgery (SRS) is a common treatment modality for vestibular schwannoma (VS), with a role in primary and recurrent/progressive algorithms. At our institution, routine magnetic resonance imaging (MRI) is obtained at 6 and 12 mo following SRS for VS. OBJECTIVE: To analyze the safety and financial impact of eliminating the 6-mo post-SRS MRI in asymptomatic VS patients. METHODS: A prospectively maintained SRS database was retrospectively reviewed for VS patients with 1 yr of post-treatment follow-up, 2005 to 2015. Decisions at 6-mo MRI were binarily categorized as routine follow-up vs clinical action-defined as a clinical visit, additional imaging, or an operation as a direct result of the 6-mo study. RESULTS: A total of 296 patients met screening criteria, of whom 53 were excluded for incomplete follow-up and 8 for NF-2. Nine were reimaged prior to 6 mo due to clinical symptoms. Routine 6-mo post-SRS MRI was completed by 226 patients (76% of screened cohort), following from which zero instances of clinical action occurred. When scaled using national insurance database-derived financials-which estimated the mean per-study charge for MRI of the brain with and without contrast at $1767-the potential annualized national charge reduction was approximated as $1 611 504. CONCLUSION: For clinically stable VS, 6-mo post-SRS MRI does not contribute significantly to management. We recommend omitting routine MRI before 12 mo, in patients without new or progressive neurological symptoms. If extrapolated nationally to the more than 100 active SRS centers, thousands of patients would be spared an inconvenient, nonindicated study, and national savings in health care dollars would be on the order of millions annually.

8.
Oper Neurosurg (Hagerstown) ; 18(2): 136-144, 2020 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-31250901

RESUMO

BACKGROUND: Surgical resection is typically cited as the optimal treatment of patients with Spetzler-Martin Grade I-II arteriovenous malformation (AVM). OBJECTIVE: To report our experience with single-fraction stereotactic radiosurgery (SRS) for Spetzler-Martin Grade I-II AVM. METHODS: A prospectively maintained registry was reviewed for patients with nonsyndromic Spetzler-Martin Grade I-II AVM having SRS from 1990 to 2011. Patients with <24 mo of follow-up or prior radiotherapy/SRS were excluded, resulting in a study population of 173 patients. Actuarial analysis was performed using the Kaplan-Meier method, and Cox proportional hazards modeling was performed with excellent outcomes (obliteration without new deficits) as the dependent variable. RESULTS: Median post-SRS follow-up was 68 mo (range, 24-275). AVM obliteration was achieved in 132 (76%) after initial SRS. Eleven additional patients achieved obliteration after repeat SRS for an overall obliteration rate of 83%. The rate of obliteration was 60% at 4 yr and 78% at 8 yr. Post-SRS hemorrhage occurred in 7 patients (4%), resulting in 3 minor deficits (2%) and 1 death (<1%). Radiation-induced complications occurred in 5 patients (3%), resulting in minor deficits only. One hundred and thirty-seven patients (79%) had excellent outcomes at last follow-up. CONCLUSION: SRS is a safe and effective treatment for patients with Spetzler-Martin Grade I-II AVM. Selection bias is likely a contributing factor to explain the superior outcomes generally noted in reported series of microsurgery for patients with low grade AVM.

9.
Otol Neurotol ; 41(2): 258-264, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31789811

RESUMO

OBJECTIVE: The majority of research influencing our understanding of vestibular schwannoma (VS) comes from large tertiary referral centers, and as a consequence, is inherently prone to referral bias. The objective of the current study was to characterize tertiary referral center bias in VS research. STUDY DESIGN: Single-institution retrospective clinical, audiometric, and radiologic review. SETTING: One tertiary referral center. PATIENTS: One hundred twenty-three patients with sporadic VS treated at our institution, consisting of a local cohort of all 41 VS patients residing in the same US county as our medical center and a referral cohort of 82 patients from outside counties matched 2:1 based on age, sex, and year of diagnosis. INTERVENTION: Surgical resection, stereotactic radiosurgery (SRS), observation. MAIN OUTCOME MEASURES: Clinical manifestations, audiometric and radiologic findings, elected treatment modality. RESULTS: Local patients had significantly longer follow-up after initial presentation compared to referral patients (median 4.0 vs 2.2 yr; p = 0.007). Referral patients were significantly less likely to have an incidental VS diagnosis (1% vs. 29%; p < 0.001) and presented with worse symptomatology such as hearing loss (80% vs. 54%; p = 0.002), dizziness (72% vs. 44%; p = 0.002), headache (29% vs. 15%; p = 0.075), and other cranial nerve dysfunction (11% vs. 0%; p = 0.029). Audiometrically, referral patients had significantly poorer word recognition scores at presentation (median 70% vs. 90%; p = 0.043). Local patients were more likely to have tumors confined to the IAC compared with referral patients, and had significantly smaller tumors when restricted to this region (median 4.0 vs 6.5 mm; p = 0.005). Referral patients were significantly more likely to undergo definitive management with either radiosurgery or microsurgery following primary evaluation compared to local patients (48% vs. 24%; p = 0.013). CONCLUSION: These data suggest that the majority of existing literature surrounding VS likely suffers from referral bias, whereby disease characteristics and management decisions are distinct from that of the general VS patient population.

10.
Artigo em Inglês | MEDLINE | ID: mdl-31828345

RESUMO

Endovascular therapy is the primary treatment for the majority of tentorial dural arteriovenous fistulas (dAVF). Surgical occlusion is an effective alternative when embolization is not possible. This video demonstrates microsugical occlusion of a right-sided tentorial dAVF in a symptomatic 45-yr-old male. The dAVF was fed directly by meningohypophyseal trunk. Venous drainage was retrograde through the sphenoparietal sinus, superficial sylvian vein, vein of Labee, and transverse sinus. The patient underwent a right-sided pterional craniotomy; the sylvian fissure was widely opened. Subarachoid dissection was performed until a large arterialized draining vein was identified exiting dura subtemporally. Intraoperative indocyanine green angiography confirmed the fistulous site and the draining vein was occluded and divided. The patient remained neurologically intact after surgery. Immediate angiography demonstrates complete occlusion of the dAVF. This video demonstrates the surgical access obtained through a transylvian approach for this tentorial dAVF. Occlusion of the draining vein, with or without resection of the fistula, is enough to permanently treat these lesions.

11.
Otol Neurotol ; 40(10): 1363-1372, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31725593

RESUMO

OBJECTIVE: To ascertain long-term hearing outcomes in patients with serviceable hearing following microsurgical resection of sporadic vestibular schwannoma (VS). STUDY DESIGN: Retrospective cohort. SETTING: Tertiary academic referral center. PATIENTS: Forty-three adult subjects with unilateral sporadic VS who had serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] class A or B) on initial postoperative audiogram following microsurgical resection between 2003 and 2016 with a minimum of two postoperative audiograms available for review. INTERVENTION: Surgical treatment with a retrosigmoid or middle cranial fossa approach. MAIN OUTCOME MEASURE: Rate of maintaining serviceable hearing, as estimated using the Kaplan-Meier method, in accordance with the 1995 and 2012 AAO-HNS guidelines on reporting hearing outcomes. RESULTS: The median immediate postoperative pure-tone average (PTA) and word recognition score (WRS) were 31 dB and 95%, respectively. At last follow-up, the median PTA was 38 dB with a median change of 5 dB from initial postoperative audiogram, and the median WRS was 90% with a median change of 0% from initial postoperative audiogram. Eight patients developed non-serviceable hearing at a median of 4.1 years following microsurgical resection (interquartile range, 2.9-7.0). The median duration of hearing follow-up for the 35 patients who maintained serviceable hearing was 3.1 years (interquartile range, 2.2-7.5). Tumor control was achieved in 41 (95%) patients. The rate of maintaining serviceable hearing at 5 years was 81%. CONCLUSION: Microsurgical resection provides excellent tumor control and durable long-term hearing in those with AAO-HNS class A or B hearing postoperatively. The paradigm of proactive microsurgical resection-when the tumor is small and hearing is good-hinges on the surgeon's ability to preserve residual hearing in a very high percentage of cases at or near preoperative hearing levels to maintain an advantage over conservative observation with regard to long-term hearing preservation.

12.
J Neurosurg ; : 1-9, 2019 Oct 04.
Artigo em Inglês | MEDLINE | ID: mdl-31585433

RESUMO

OBJECTIVE: Tumor-associated macrophages (TAMs) have been implicated as pathologic actors in phenotypically aggressive vestibular schwannoma (VS), potentially mediated via programmed death-ligand 1 (PD-L1). The authors hypothesized that PD-L1 is a key regulator of the VS immune microenvironment. METHODS: Forty-six consecutive, radiation-naïve, sporadic VSs that were subtotally resected at primary surgery were assessed via immunohistochemical analysis, including analysis of CD163 and PD-L1 expression. Pathologic data were correlated with clinical endpoints, including tumor control, facial nerve function, and complications. RESULTS: Baseline parameters were equivalent between stable and progressive post-subtotal resection (STR) VS. CD163 percent positivity and M2 index were significantly increased among tumors that remained stable (34% vs 21%, p = 0.02; 1.13 vs 0.99, p = 0.0008), as well as patients with favorable House-Brackmann grade I or II facial nerve function (31% vs 13%, p = 0.04; 1.11 vs 0.97, p = 0.05). PD-L1 percent positivity was significantly associated with tumor progression (1% vs 11%, p = 0.01) and unfavorable House-Brackmann grade III-VI facial nerve function (1% vs 38%, p = 0.02). On multivariate analysis, PD-L1 was independently significant in all models (likelihood ratio 4.4, p = 0.04), while CD163 was dependent in all iterations. CONCLUSIONS: In contrast to prior reports, in this study, the authors observed significantly increased levels of M1, CD163+ TAMs in association with VS that progressed after STR. Progressive tumors are characterized by increased PD-L1, potentially highlighting a mechanism of immune evasion that results in TAM deactivation, tumor growth, and further infiltration of anti-tumor immune cells. Targeting PD-1/PD-L1 may offer therapeutic promise, particularly in the setting of disease control after STR.

14.
Otol Neurotol ; 40(10): e1012-e1017, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31634279

RESUMO

OBJECTIVE: To date, prediction models for estimating risk of acquiring non-serviceable hearing in subjects with observed vestibular schwannoma (VS) have evaluated outcomes primarily based on features at initial diagnosis. Herein, we evaluate the association of rate of hearing decline during the initial period of observation with time to non-serviceable hearing. If significant, rate of hearing decline may inform decision making after an introductory period of observation. SETTING: Two tertiary care centers. PATIENTS: VS patients with serviceable hearing who underwent at least three audiograms and two magnetic resonance imaging (MRI) studies before intervention or being lost to follow-up. The rate of change in pure-tone average (PTA) and word recognition score (WRS) was calculated as the score from the second audiogram minus the score from the first audiogram, divided by the duration in months between the two. MAIN OUTCOME MEASURE(S): Serviceable hearing, defined as PTA ≤50 dB HL and WRS ≥50%. RESULTS: Among 266 patients meeting inclusion criteria, 52 developed non-serviceable hearing at last follow-up. Kaplan-Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years were 97% (95-100; 206), 78% (72-85; 98), 68% (60-77; 39), 60% (50-73; 17), and 44% (29-67; 2), respectively. In a univariable setting, each 1 dB increase per month in the rate of initial PTA change was associated with a 96% increased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 1.96; 95% CI 1.44-2.68; p < 0.001). Each 1% increase per month in the rate of initial WRS change was associated with a decreased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 0.79; 95% confidence interval [CI] 0.66-0.94; p = 0.009). After multivariable adjustment, both rate of PTA change (HR 2.42; 95% CI 1.72-3.41; p < 0.001) and rate of WRS change (HR 0.81; 95% CI 0.67-0.99; p = 0.043) remained statistically significantly associated with time to non-serviceable hearing. CONCLUSION: Rate of early PTA and WRS decline during the initial period of observation are significantly associated with time to development of non-serviceable hearing. This information may facilitate accurate patient counseling and inform decision-making regarding prospective disease management.

15.
J Neurosurg ; : 1-8, 2019 Oct 25.
Artigo em Inglês | MEDLINE | ID: mdl-31653808

RESUMO

OBJECTIVE: Stereotactic radiosurgery (SRS) is an accepted treatment option for patients with benign parasellar tumors. Here, the authors' objective was to determine the risk of developing new or progressive internal carotid artery (ICA) stenosis or occlusion after single-fraction SRS for cavernous sinus meningioma (CSM) or growth hormone-secreting pituitary adenoma (GHPA). METHODS: The authors queried their prospectively maintained registry for patients treated with single-fraction SRS for CSM or GHPA in the period from 1990 to 2015. Study criteria included no prior irradiation and ≥ 12 months of post-SRS radiological follow-up. Pre-SRS grading of ICA involvement was applied according to the 1993 classification schemes of Hirsch for CSM or Knosp for GHPA. RESULTS: The authors conducted a retrospective review of 283 patients, 155 with CSMs and 128 with GHPAs. Ninety-three (60%) CSMs were Hirsch category 2 and 3 tumors; 97 (76%) GHPAs were Knosp grade 2-4 tumors. Median follow-up after SRS was 6.6 years (IQR 1-24.9 years). No GHPA or category 1 CSM developed ICA stenosis or occlusion. Three (5.2%) patients with category 2 CSMs had asymptomatic ICA stenosis (n = 2) or occlusion (n = 1); 1 (1.1%) category 2 CSM patient had transient ischemic symptoms. Five (14.3%) category 3 CSMs progressed to ICA occlusion (4 asymptomatic, 1 symptomatic). The median time to stenosis/occlusion was 4.8 years (IQR 1.8-7.6). Five- and 10-year risks of ICA stenosis/occlusion in category 2 and 3 CSM patients were 7.5% and 12.4%, respectively. Five- and 10-year risks of ischemic stroke from ICA stenosis/occlusion in category 2 and 3 CSM patients were both 1.2%. Multivariate analysis showed patient age (HR 0.92, 95% CI 0.86-0.98, p = 0.01), meningioma pathology (HR and 95% CI not defined, p = 0.03), and pre-SRS carotid category (HR 4.51, 95% CI 1.77-14.61, p = 0.004) to be associated with ICA stenosis/occlusion. Internal carotid artery stenosis/occlusion was not related to post-SRS tumor growth (HR and 95% CI not defined, p = 0.41). CONCLUSIONS: New or progressive ICA stenosis/occlusion was common after SRS for CSM but was not observed after SRS for GHPA, suggesting a tumor-specific mechanism unrelated to radiation dose. Pre-SRS ICA encasement or constriction increases the risk of ICA stenosis/occlusion; however, the risk of ischemic complications is very low.

16.
Otol Neurotol ; 40(9): 1224-1229, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31469794

RESUMO

OBJECTIVE: Previous research has shown that tumor growth during observation of small-to-medium sized sporadic vestibular schwannomas (VSs) occurs almost exclusively within 3 to 5 years following diagnosis. This has led some to consider ending surveillance after this interval. This study seeks to characterize a cohort of patients with tumors that exhibited late growth. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Adults with sporadic VSs who initially elected observation with serial magnetic resonance imaging (MRI) surveillance. INTERVENTION(S): None. MAIN OUTCOME MEASURE(S): Linear tumor growth was measured in accordance with AAO-HNS reporting guidelines. Delayed growth was defined as growth ≥2 mm in linear diameter that was first detected 5 years or more from the initial MRI. RESULTS: From a total of 361 patients, 172 experienced tumor growth during the interval of observation. Fourteen of these 172 patients (8.1%) experienced late growth occurring at 5 years or beyond. Among patients with delayed growth, the fastest growth rate after extended quiescence was 1.33 mm/yr, and the longest delay before tumor growth detection was 11.1 years. Additional treatment was recommended for six (42.9%) of the patients with delayed growth. Of 68 tumors that remained in the IAC, 11 (16.2%) demonstrated delayed growth. Of 66 tumors that presented in the CPA, 2 (3.0%) demonstrated delayed growth. Initial size was larger for tumors demonstrating early growth compared with those with delayed growth. For tumors within the IAC, those with early growth had a significantly higher median growth rate than those with delayed growth (1.40 vs. 0.45 mm/yr, p < 0.001). CONCLUSIONS: Delayed growth encompassed 8.1% of growing VSs and 3.9% of all observed tumors. Patients with delayed growth exhibited slower growth rates compared with those who were diagnosed with growth early in their observation course. These findings support the need for lifelong surveillance of untreated VSs given the possibility of clinically significant delayed growth. Increasing the time interval between MRI studies after 5 years is a reasonable concession to balance practicalities of cost and convenience with risk of delayed of tumor growth.

17.
Otol Neurotol ; 40(9): 1230-1236, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31469795

RESUMO

OBJECTIVE: The etiology of sensorineural hearing loss (SNHL) in patients with jugular paraganglioma (JP) whose tumors lack inner ear fistulae or vestibulocochlear nerve involvement is unknown. Recent literature has proposed that occlusion of the inferior cochlear vein may be causative. Herein, we assess the association between radiologic involvement of the cochlear aqueduct (CA) and the development of SNHL. STUDY DESIGN: Blinded, retrospective review of imaging and audiometry. SETTING: Tertiary center. PATIENTS: Adults with JP. INTERVENTION(S): None. MAIN OUTCOME MEASURES: Asymmetric SNHL was assessed continuously as the difference in bone conduction pure-tone average (BCPTA) between ears and as a categorical variable (≥15 dB difference at two consecutive frequencies, or a difference in speech discrimination score of ≥15%). Involvement of the CA was considered present if there was evidence of medial T2 fluid signal loss, contrast enhancement, or bony erosion/expansion. RESULTS: Of 30 patients meeting inclusion criteria, 15 (50%) had asymmetric SNHL. CA involvement was observed in 87% of patients with asymmetric SNHL compared with 13% in those with symmetric hearing (p = 0.0001). Univariate analysis demonstrated that age, sex, and tumor volume were not associated with asymmetric SNHL. The median difference in BCPTA between ears in patients with CA involvement was 21.3 dB HL compared to 1.2 dB HL in those without CA involvement (p < 0.0001). Regression analysis demonstrates that enhancement within the CA is associated with a BCPTA difference of 19.4 dB HL (p = 0.0006). CONCLUSIONS: Cochlear aqueduct involvement by JP is associated with SNHL in the absence of inner ear fistula, vestibulocochlear nerve involvement, or brainstem compression. Correlation with operative findings or histopathologic evidence of tumor involvement may validate this intriguing imaging finding.

18.
J Neurosurg ; : 1-8, 2019 Aug 23.
Artigo em Inglês | MEDLINE | ID: mdl-31443076

RESUMO

OBJECTIVE: Sphenoorbital meningioma (SOM) is a unique skull base tumor, characterized by infiltrative involvement and hyperostosis primarily of the lesser wing of sphenoid bone, with frequent involvement of the orbital compartment. SOM often manifests with proptosis and visual impairment. Surgical technique and outcome are highly variable among studies reported in the literature. The authors present a single-surgeon experience with SOM. METHODS: A retrospective review of a prospectively maintained institutional database was performed. A blinded imaging review by 2 study team members was completed to confirm SOM, after which chart review was carried out to capture demographics and outcomes. All statistical testing was completed using JMP Pro version 14.1.0, with significance defined as p < 0.05. RESULTS: Forty-seven patients who underwent surgery between 2000 and 2017 were included. The median age at surgery was 47 years (range 36-70 years), 81% of patients were female, and the median follow-up was 43 months (range 0-175 months). All operations were performed via a frontotemporal craniotomy, orbitooptic osteotomy, and anterior clinoidectomy, with extensive resection of all involved bone and soft tissue. Preoperatively, proptosis was noted in 44 patients, 98% of whom improved. Twenty-eight patients (60%) had visual deficits before surgery, 21 (75%) of whom improved during follow-up. Visual field defect other than a central scotoma was the only prognostic factor for improvement in vision on multivariate analysis (p = 0.0062). Nine patients (19%) had recurrence or progression during follow-up. CONCLUSIONS: SOM is a unique skull base tumor that needs careful planning to optimize outcome. Aggressive removal of involved bone and periorbita is crucial, and proptosis and visual field defect other than a central scotoma can improve after surgery.

19.
J Neurol Surg B Skull Base ; 80(4): 338-351, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31316880

RESUMO

Introduction Although numerous anatomical and operative atlases have been published, those that have focused on the skull base either have provided views that are quite difficult to achieve in the operating room to better depict surgical anatomy or are written at the level of an audience with considerable knowledge and experience. Methods Five sides of three formalin-fixed latex-injected specimens were dissected under microscopic magnification. A posterior petrosectomy approach was performed by three neurosurgical residents at different training levels with limited previous experience in anatomical dissection mentored by the senior authors (C. L. W. D. and M. J. L.) and a clinical skull base fellow with additional anatomical dissection experience (M. P. C.). Anatomical dissections were performed until the expected level of dissection quality was achieved to demonstrate each important step of the surgical approach that would be understandable to all trainees of all levels. Following dissection education, representative case applications were reviewed. Results The posterior petrosectomy (also known as presigmoid retrolabyrinthine approach) affords excellent access to cranial nerves III to XI and a diverse array of pathologies. Key steps include positioning and skin incision, scalp and muscle flaps, burr holes, craniotomy flap elevation, superficial mastoidectomy, otic capsule exposure and presigmoid dura decompression, primary presigmoid durotomy, inferior temporal durotomy, superior petrosal sinus ligation, tentorium sectioning, and final exposure. Conclusion The posterior petrosectomy is a challenging approach; thorough operative-style laboratory dissection is essential to provide trainees with a suitable guide. We describe a comprehensive approach to learning this technique, intended to be understandable and usable by a resident audience.

20.
J Neurol Surg B Skull Base ; 80(4): 392-398, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31316885

RESUMO

Background There is little data regarding postoperative outcomes of patients with obstructive sleep apnea (OSA) undergoing skull base surgery. The purpose of this study is to determine an association between risk factors and proximity of cerebrospinal fluid (CSF) leak to surgery in patients with OSA undergoing endoscopic skull base surgery. Methods A retrospective review of neurosurgical inpatients, with and without OSA, at a tertiary care institution from 2002 to 2015 that experienced a postoperative CSF leak after undergoing endoscopic skull base surgery. Results Forty patients met inclusion criteria, 12 (30%) with OSA. OSA patients had significantly higher body mass index (BMI; median 39.4 vs. 31.7, p < 0.01) and were more likely to have diabetes (41.7 vs. 10.7%, p = 0.04) than non-OSA patients; otherwise there were no significant differences in clinical comorbidities. No patients restarted positive pressure ventilation (PPV) in the inpatient setting. The type of repair was not a significant predictor of the time from surgery to leak. Patients with OSA experienced postoperative CSF leak 49% sooner than non-OSA patients (Hazard Ratio 1.49, median 2 vs. 6 days, log-rank p = 0.20). Conclusion Patients with OSA trended toward leaking earlier than those without OSA, and no OSA patients repaired with a nasoseptal flap (NSF) had a leak after postoperative day 5. Due to a small sample size this trend did not reach significance. Future studies will help to determine the appropriate timing for restarting PPV in this high risk population. This is important given PPV's significant benefit to the patient's overall health and its ability to lower intracranial pressure.

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