scholarly journals Cervical Chordomas: Multicenter Case Series and Meta-analysis

Author(s):  
Oluwaseun Akinduro ◽  
Diogo P. Garcia ◽  
Ricardo A Domingo ◽  
Tito Vivas-Buitrago ◽  
Bernardo Sousa-Pinto ◽  
...  

Abstract Background En bloc spondylectomy is the gold standard for surgical resection of sacral chordomas (CHO), but the effect of extent of resection on recurrence and survival in patients with CHO of the cervical spine remains elusive. Methods MEDLINE, Embase, Scopus, and Cochrane were systematically reviewed. Patients with cervical CHO treated at three tertiary-care academic institutions were reviewed for inclusion. We performed an individual participant data meta-analysis to assess the overall survival (OS) and progression free survival (PFS) after en bloc-gross total resection (GTR) and intralesional-GTR compared to subtotal resection (STR). We then performed an intention-to-treat analysis including all patients with attempted en bloc resection in the en bloc group, regardless of the surgical margins. Results There was a total of 13 series including 161 patients with cervical CHO, including our current series of 22 patients. GTR (en bloc-GTR + intralesional-GTR) was associated with a significant decrease in the risk of local progression (pooled hazard ratio (PHR) = 0.22; 95% CI = 0.08–0.59; p = 0.003) and risk of death (PHR = 0.31; 95%; CI:0.12–0.83; p = 0.020). A meta-regression analyses determined that intralesional-GTR improved PFS (PHR = 0.35; 95%CI = 0.16–0.76; p = 0.009) as well as OS (PHR = 0.25; 95%CI = 0.08–0.79; p = 0.019) when compared to STR. En bloc-GTR was associated with a significant reduction in the risk of local progression (PHR = 0.06; 95%CI = 0.01–0.77; p = 0.030), but not a decreased OS (PHR = 0.50; 95% CI = 0.19–1.27; p = 0.145). Our intention-to-treat analyses revealed a near significant improvement in OS for the en bloc group (PHR: 0.15; 95%CI:0.02–1.22; p = 0.054), and nearly identical improvement in PFS. Radiation data was not available for the studies included in the meta-analysis. Conclusion This is the first and only meta-analysis of patients with cervical CHO. We found that both en bloc-GTR and intralesional-GTR resulted in improved local tumor control when compared to STR.

2020 ◽  
Vol 25 (1) ◽  
pp. 30-36
Author(s):  
Soliman Oushy ◽  
Avital Perry ◽  
Christopher S. Graffeo ◽  
Aditya Raghunathan ◽  
Lucas P. Carlstrom ◽  
...  

OBJECTIVEGanglioglioma is a low-grade central nervous system neoplasm with a pediatric predominance, accounting for 10% of all brain tumors in children. Gangliogliomas of the cervicomedullary junction (GGCMJs) and brainstem (GGBSs) present a host of management challenges, including a significant risk of surgical morbidity. At present, understanding of the prognostic factors—including BRAF V600E status—is incomplete. Here, the authors report a single-institution GGCMJ and GGBS experience and review the pertinent literature.METHODSA prospectively maintained neurosurgical database at a large tertiary care academic referral center was retrospectively queried for cases of GGCMJ pathologically confirmed in the period from 1995 to 2015; appropriate cases were defined by diagnosis codes and keywords. Secondary supplemental chart review was conducted to confirm or capture relevant data. The primary study outcome was treatment failure as defined by evidence of radiographic recurrence or progression and/or clinical or functional decline. A review of the literature was conducted as well.RESULTSFive neurosurgically managed GGBS patients were identified, and the neoplasms in 4 were classified as GGCMJ. All 5 patients were younger than 18 years old (median 15 years, range 4–16 years) and 3 (60%) were female. One patient underwent gross-total resection, 2 underwent aggressive subtotal resection (STR), and 2 underwent stereotactic biopsy only. All patients who had undergone STR or biopsy required repeat resection for tumor control or progression. Progressive disease was treated with radiotherapy in 2 patients, chemotherapy in 2, and chemoradiotherapy alone in 1. Immunostaining for BRAF V600E was positive in 3 patients (60%). All 5 patients experienced at least one major complication, including wound infection, foot drop, hemiparesis, quadriparesis, cranial neuropathy, C2–3 subluxation, syringomyelia, hydrocephalus, aspiration, and coma. Overall mortality was 20%, with 1 death observed over 11 years of follow-up.CONCLUSIONSGGBS and GGCMJ are rare, benign posterior fossa tumors that carry significant perioperative morbidity. Contemporary management strategies are heterogeneous and include combinations of resection, radiotherapy, and chemotherapy. The BRAF V600E mutation is frequently observed in GGBS and GGCMJ and appears to have both prognostic and therapeutic significance with targeted biological agents.


Author(s):  
C Dandurand ◽  
AA Sepehry ◽  
MH Asadi Lari ◽  
R Akagami ◽  
PA Gooderham

Background: The optimal therapeutic approach for adult craniopharyngioma remains controversial. Some advocate for gross total resection (GTR), while others support subtotal resection followed by adjuvant radiotherapy (STR + XRT). Methods: MEDLINE (1946 to July 1st 2016) and EMBASE (1980 to June 30th 2016) were systematically reviewed. Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline was followed. At our institution, from 1975 to 2013, 33 patients were treated with initial surgical resection for adult onset craniopharyngioma. 22 patients were included in the present case series. Results: Eligible studies (n=21) were identified from the literature in addition to a case series of our institutional experience. Three groups were available for analysis: GTR, STR + XRT, and STR. The rates of recurrence were 17 %, 27 % and 45%, respectively. This differs from childhood population. The difference in risk of recurrence after GTR vs. STR + XRT did not reach significance (OR: 0.63, 95% confidence interval: 0.33-1.24, P=0.18). This maybe because of low pooled sample size (n=99) who underwent STR+XRT. Conclusions: This is the first and largest meta-analysis examining rate of recurrence in adult craniopharyngioma. Thus, when safe and feasible, a goal of gross total resection should be favored. Each patient should be considered on a case-by-case basis.


2010 ◽  
Vol 113 (5) ◽  
pp. 1087-1092 ◽  
Author(s):  
Michael E. Sughrue ◽  
Martin J. Rutkowski ◽  
Derick Aranda ◽  
Igor J. Barani ◽  
Michael W. McDermott ◽  
...  

Object Although there is a considerable volume of literature available on the treatment of patients with cavernous sinus meningiomas (CSMs), most of the data regarding tumor control and survival come from case studies or single-institution series. The authors performed a meta-analysis of reported tumor control and survival rates of patients described in the published literature, with an emphasis on specific prognostic factors. Methods The authors systematically analyzed the published literature and found more than 3000 patients treated for CSMs. Separate meta-analyses were performed to calculate pooled rates of recurrence and cranial neuropathy after 1) gross-total resection, 2) subtotal resection without adjuvant postoperative radiotherapy or radiosurgery, and 3) stereotactic radiosurgery (SRS) alone. Results were expressed as pooled proportions, and random-effects models were used to incorporate any heterogeneity present to generate a pooled proportion. Individual studies were weighted using the inverse variance method, and 95% CIs for each group were calculated from the pooled proportions. Results A total of 2065 nonduplicated patients treated for CSM met inclusion criteria for the analysis. Comparisons of the 95% CIs for recurrence of these 3 cohorts revealed that SRS-treated patients experienced improved rates of recurrence (3.2% [95% CI 1.9–4.5%]) compared with either gross-total resection (11.8% [95% CI 7.4–16.1%]) or subtotal resection alone (11.1% [95% CI 6.6–15.7%]) (p < 0.01). The authors found that the pooled mixed-effects rate of cranial neuropathy was markedly higher in patients undergoing resection (59.6% [95% CI 50.3–67.5%]) than for those undergoing SRS alone (25.7% [95% CI 11.5–38.9%]) (p < 0.05). Conclusions Radiosurgery provided improved rates of tumor control compared with surgery alone, regardless of the subjective extent of resection.


2021 ◽  
Vol 12 ◽  
pp. 485
Author(s):  
Antonio Crea ◽  
Gianluca Grimod ◽  
Gianluca Scalia ◽  
Mariarosaria Verlotta ◽  
Lucio Mazzeo ◽  
...  

Background: Primary intradiploic meningiomas, extra-axial tumors arising primarily in the skull, are rare. The authors reported a complex case of intradiploic intraosseous metaplastic meningioma of the left medial wall and orbital roof with the left frontal sinus invasion and left ethmoidal body bone substitution. The authors also conducted a systematic review concerning diagnosis and management of patients affected by purely calvarial intradiploic meningiomas along with a focus on fronto-orbito-ethmoidal ones. Methods: A literature search was conducted using PubMed and Scopus databases according to preferred reporting items for systematic reviews and meta-analysis statement and with the following Mesh terms: Intradiploic, intraosseous, calvarial, and meningioma. Eligibility criteria were limited by the nature of existing literature on intradiploic meningiomas, consisting of only case series, and case reports. Results: A total of 128 published studies were identified through our search. 41 studies were included in this systematic review, 59 patients with a female/male ratio of 1.2/1. The mean age of the patients is of 47.69 years (range 3–84 years). Only seven out of 59 patients (11.9%) presented a complex intradiploic meningioma located in fronto-orbito-ethmoidal region like our case. In almost all patients, a gross-total resection was performed (96.6%) and only in two patients (3.4%) a subtotal resection was achieved. Conclusion: The authors shared this successfully treated case to add to the overall clinical experience in the management of this rare subtype tumor, with the hope that more studies are conducted to further address the mechanism of intradiploic meningiomas development.


Author(s):  
Om Parkash ◽  
Roger Christopher Gill ◽  
Iman Qaiser ◽  
Muhammad Sharoz Rabbani ◽  
Tabish chawala ◽  
...  

Abstract Gastric volvulus is a rare surgical emergency which mostly presents in children and occasionally in adults. It results due to pathological malrotaion of the stomach along its longitudinal or short axis. The condition, if not treated promptly, can result in increased morbidity and caries a high risk of death. Very few cases have been reported in literature regards this important clinical condition. Early decompression and repair of anatomical defects are the corner stone of its management. There is a dire need to develop guidelines and algorithms for management and treatment of this rare condition to improve patient outcome, prevent recurrence and facilitate early diagnosis by practicing physicians and surgeons. We report three such cases which presented to our setup in emergency along with a brief description of how they were successfully managed. Keywords: Gastric Volvulus, Malrotation, Vomiting, Continuous...


2011 ◽  
Vol 114 (5) ◽  
pp. 1299-1305 ◽  
Author(s):  
Michael E. Ivan ◽  
Michael E. Sughrue ◽  
Aaron J. Clark ◽  
Ari J. Kane ◽  
Derick Aranda ◽  
...  

Object Because of the rarity of glomus jugulare tumors, a variety of treatment paradigms are currently used. There is no consensus regarding the optimal management to control tumor burden while minimizing treatment-related morbidity. In this study, the authors assessed data collected from 869 patients with glomus jugulare tumors from the published literature to identify treatment variables that impacted clinical outcomes and tumor control rates. Methods A comprehensive search of the English-language literature identified 109 studies that collectively described outcomes for patients with glomus jugulare tumors. Univariate comparisons of demographic information between treatment cohorts were performed to detect differences in the sex distribution, age, and Fisch class of tumors among various treatment modalities. Meta-analyses were performed on calculated rates of recurrence and cranial neuropathy after subtotal resection (STR), gross-total resection (GTR), STR with adjuvant postoperative radiosurgery (STR+SRS), and stereotactic radiosurgery alone (SRS). Results The authors identified 869 patients who met their inclusion criteria. In these studies, the length of follow-up ranged from 6 to 256 months. Patients treated with STR were observed for 72 ± 7.9 months and had a tumor control rate of 69% (95% CI 57%–82%). Those who underwent GTR had a follow-up of 88 ± 5.0 months and a tumor control rate of 86% (95% CI 81%–91%). Those treated with STR+SRS were observed for 96 ± 4.4 months and had a tumor control rate of 71% (95% CI 53%–83%). Patients undergoing SRS alone had a follow-up of 71 ± 4.9 months and a tumor control rate of 95% (95% CI 92%–99%). The authors' analysis found that patients undergoing SRS had the lowest rates of recurrence of these 4 cohorts, and therefore, these patients experienced the most favorable rates of tumor control (p < 0.01). Patients who underwent GTR sustained worse rates of cranial nerve (CN) deficits with regard to CNs IX–XI than those who underwent SRS alone; however, the rates of CN XII deficits were comparable. Conclusions The authors' analysis is limited by the quality and accuracy of these studies and may reflect source study biases, as it is impossible to control for the quality of the data reported in the literature. Finally, due to the diverse range of data presentation, the authors found that they were limited in their ability to study and control for certain variables. Some of these limitations should be minimized with their use of meta-analysis methods, which statistically evaluate and adjust for between-study heterogeneity. These results provide the impetus to initiate a prospective study, appropriately controlling for variables that can confound the retrospective analyses that largely comprise the existing literature.


Neurosurgery ◽  
2017 ◽  
Vol 83 (4) ◽  
pp. 631-641 ◽  
Author(s):  
Charlotte Dandurand ◽  
Amir Ali Sepehry ◽  
Mohammad Hossein Asadi Lari ◽  
Ryojo Akagami ◽  
Peter Gooderham

Abstract BACKGROUND The optimal therapeutic approach for adult craniopharyngioma remains controversial. Some advocate for gross total resection (GTR), while others advocate for subtotal resection followed by adjuvant radiotherapy (STR + XRT). OBJECTIVE To conduct a systematic review and meta-analysis assessing the rate of recurrence in the follow-up of 3 yr in adult craniopharyngioma stratified by extent of resection and presence of adjuvant radiotherapy. METHODS MEDLINE (1946-July 1, 2016) and EMBASE (1980-June 30, 2016) were systematically reviewed. From1975 to 2013, 33 patients were treated with initial surgical resection for adult onset craniopharyngioma at our center and were reviewed for inclusion in this study. RESULTS Data from 22 patients were available for inclusion as a case series in the systematic review. Eligible studies (n = 21) were identified from the literature in addition to a case series of our institutional experience. Three groups were available for analysis: GTR, STR + XRT, and STR. The rates of recurrence were 17%, 27%, and 45%, respectively. The risk of developing recurrence was significant for GTR vs STR (odds ratio [OR]: 0.24, 95% confidence interval [CI]: 0.15-0.38) and STR + XRT vs STR (OR: 0.20, 95% CI: 0.10-0.41). Risk of recurrence after GTR vs STR + XRT did not reach significance (OR: 0.63, 95% CI: 0.33-1.24, P = .18). CONCLUSION This is the first and largest systematic review focusing on the rate of recurrence in adult craniopharyngioma. Although the rates of recurrence are favoring GTR, difference in risk of recurrence did not reach significance. This study provides guidance to clinicians and directions for future research with the need to stratify outcomes per treatment modalities.


Stroke ◽  
2015 ◽  
Vol 46 (suppl_1) ◽  
Author(s):  
Georgios Tsivgoulis ◽  
Ramin Zand ◽  
Aristeidis H Katsanos ◽  
Nitin Goyal ◽  
Ken Uchino ◽  
...  

Background&Purpose: Shortening door-to-needle time may lead to intravenous thrombolysis (IVT) administration in stroke mimics (SM). We sought to determine safety of IVT in SM using prospective, single-center data and by conducting a comprehensive meta-analysis of reported case series. Subjects&Methods: We analyzed consecutive IVT-treated patients during a 5-year period (2009-2013) at a tertiary care stroke center. Non-contrast head CT, NIHSS pre-treatment and modified Rankin scores (mRS) at discharge were obtained as standard of care. SM was defined as no acute ischemic lesions on MRI and the final non-stroke diagnosis established by radiologists and treating physicians unaware of the purposes of this study. Symptomatic intracerebral hemorrhage (sICH) was defined as imaging evidence of ICH with NIHSS increase of 4 or more points. Functional independence (FI) was defined as mRS 0-1. We also conducted a comprehensive meta-analysis of all available case-series reporting safety of IVT in SM using PRISMA guidelines. Results: Of 516 consecutive IVT patients at our tertiary care center (50% men; mean age 60±14years; median NIHSS 11, range 3-22), SM comprised 75 cases (14.5%, 95%CI by adjusted wald method: 11.7%-17.9%). sICH occurred in one patient (1.3%; 95%CI:0%-7.9%), while we documented no cases of orolingual edema (OE) or major extracranial hemorrhagic complications (0%; 95%CI:0%-4.2%). Two patients experienced minor extra-cranial hemorrhagic complications (1 case of gingival & 1 case of nose bleeding). Meta-analysis of 9 studies including 8,942 patients treated with IVT and reporting safety outcomes in 392 SM showed pooled rates of sICH and OE 0.5% (95%CI: 0%-2%) and 0.3% (95%CI: 0%-2%) respectively. We documented no heterogeneity between estimates of sICH (I squared statistic=0%; p=0.941 Cohran Q test), and OE (I squared statistic=0%; p=0.913 Cohran Q test). Conclusions: Our prospectively collected data coupled with the findings of the comprehensive meta-analysis underscore the safety of IVT in SM given the low prevalence of hemorrhagic and systemic complications and the lack of heterogeneity across reported case-series.


2012 ◽  
Vol 116 (2) ◽  
pp. 349-354 ◽  
Author(s):  
Ari J. Kane ◽  
Michael E. Sughrue ◽  
Martin J. Rutkowski ◽  
Derick Aranda ◽  
Stephen A. Mills ◽  
...  

Object The literature, at present, provides limited information about extraventricular neurocytomas (EVNs) and is almost exclusively composed of case reports or small case series. Treatment for EVNs has largely been guided by results from central neurocytoma outcome studies. The authors present an analysis of all reported intracranial EVN cases to establish if tumor histopathological features can substratify EVN into groups with differing prognosis and help guide treatment decisions. Methods The authors identified studies reporting histology, treatment modality, and outcomes for patients with intracranial EVN. The rates of recurrence and survival for patients were compared using Kaplan-Meier analysis. Atypical tumors, defined by MIB-1 labeling index exceeding 3% or atypical histological features, were compared with typical tumors, and patients 50 years of age or older were compared with those younger than 50 years of age. Results Eighty-five patients met the inclusion criteria, and 27% of them had an atypical histology. Typical EVNs had a better prognosis than atypical EVNs after primary treatment, with a 5-year recurrence rate of 36% compared with 68% (p < 0.001), and a 5-year mortality rate of 4% compared with 44%, respectively (p < 0.001). Age younger 50 years was associated with a better prognosis than age equal to or greater than 50 years, with a 5-year recurrence rate of 33% and 74%, respectively (p < 0.001), and a 5-year mortality rate of 4% and 52%, respectively (p < 0.001). Multivariate analysis demonstrated that atypical EVNs carried significantly increased risk for recurrence (hazard ratio [HR] 4.91, p < 0.001) and death (HR 22.91, p < 0.01). Gross-total resection was superior to subtotal resection (STR) alone in tumor control rates for typical EVNs (95% and 68%, p < 0.05), and there was a trend for adjuvant external-beam radiotherapy to benefit STR. There was suggestion of similar trends in patients with atypical EVNs. Conclusions There are at least 2 distinct histological subtypes of EVN, with different prognostic significances. Atypia or MIB-1 labeling index greater than 3% is a significant predictor of poor prognosis for EVNs. Complete resection or more aggressive attempts at providing adjuvant therapy following STR appear to improve the prognosis for patients with EVNs. Although the authors' results are informative, there are limitations to their analysis. Given the relatively modest total number of cases reported, as well as the nature of the disaggregated analysis, the authors were not able to use formal meta-analytical methods to limit the impact of between center heterogeneity. Additionally, they were not able to control for individual differences in data analysis and presentation across the different studies included in their analysis.


2021 ◽  
Vol 12 ◽  
pp. 336
Author(s):  
Alexander Konovalov ◽  
Sergey Maryashev ◽  
David Pitskhelauri ◽  
Vitaly Siomin ◽  
Andrey Golanov ◽  
...  

Background: The purpose of the presented work is to evaluate the last decade’s experience in surgical management of central neurocytoma (CN) and elucidate on the treatment strategies and new options. Methods: The current series consists of the remaining 125 patients (70 females and 55 males) operated on during the past decade from 2008 to 2018. Most tumors were resected through transcortical (n = 76, 61%), or transcallosal (n = 40, 32%) approaches. In 5 (4%) patients with predominantly posterior location of the tumor, non-dominant superior parietal lobule approach was utilized. Both approaches (transcortical + transcallosal) were used in 4 (3%) of cases. Seven consecutive patients with large CN underwent prophylactic intraventricular stenting to prevent hydrocephalus. Results: Gross total resection was achieved in 45 patients (36%), subtotal resection (STR) in 40 (32%) cases. After surgery, 63 (50%) patients had neurocognitive problems, including disorientation, attention deficit, global amnesia, short-term memory deficits, and perceptual motor and social cognition problems. A total of 26 patients (21%) had postoperative hemorrhage in the resection bed. Obstructive hydrocephalus was noted in 25 (20%) patients. The entrapment of the occipital and/or temporal horns was observed in seven cases. None of the seven patients with prophylactic intraventricular stents required shunting. Conclusion: Although high rates of gross total or STR can be expected, the mortality and morbidity remain significant even in the modern neurosurgical era. Prophylactic intraventricular stenting in patients with large posteriorly located tumors with hydrocephalus may prevent ventricular entrapment and shunting. The main risk factors for recurrence are presence of residual disease and Ki-67 index over 5%. Recurrent symptomatic tumors should be treated surgically, whereas asymptomatic progression can be managed with stereotactic radiosurgery. Both treatment modalities are associated with low risk of complications and high tumor control rates.


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