The last decade’s experience of management of central neurocytomas: Treatment strategies and new options

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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Craniopharyngioma: a comparison of tumor control with various treatment strategies

Neurosurgical FOCUS ◽

10.3171/2010.1.focus09307 ◽

2010 ◽

Vol 28 (4) ◽

pp. E5 ◽

Cited By ~ 108

Author(s):

Isaac Yang ◽

Michael E. Sughrue ◽

Martin J. Rutkowski ◽

Rajwant Kaur ◽

Michael E. Ivan ◽

...

Keyword(s):

Tumor Resection ◽

Treatment Strategies ◽

Group Versus ◽

Outcome Data ◽

Treatment Modalities ◽

Rank Test ◽

Tumor Control ◽

Subtotal Resection ◽

Log Rank Test ◽

Significant Difference

Object Craniopharyngiomas have a propensity to recur after resection, potentially causing death through their aggressive local behavior in their critical site of origin. Recent data suggest that subtotal resection (STR) followed by adjuvant radiotherapy (XRT) may be an appealing substitute for gross-total resection (GTR), providing similar rates of tumor control without the morbidity associated with aggressive resection. Here, the authors summarize the published literature regarding rates of tumor control with various treatment modalities for craniopharyngiomas. Methods The authors performed a comprehensive search of the English language literature to identify studies publishing outcome data on patients undergoing surgery for craniopharyngioma. Rates of progression-free survival (PFS) and overall survival (OS) were determined through Kaplan-Meier analysis. Results There were 442 patients who underwent tumor resection. Among these patients, GTR was achieved in 256 cases (58%), STR in 101 cases (23%), and STR+XRT in 85 cases (19%). The 2- and 5-year PFS rates for the GTR group versus the STR+XRT group were 88 versus 91%, and 67 versus 69%, respectively. The 5- and 10-year OS rates for the GTR group versus the STR+XRT group were 98 versus 99%, and 98 versus 95%, respectively. There was no significant difference in PFS (log-rank test) or OS with GTR (log-rank test). Conclusions Given the relative rarity of craniopharyngioma, this study provides estimates of outcome for a variety of treatment combinations, as not all treatments are an option for all patients with these tumors.

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Allotransplantation for chronic lymphocytic leukemia

Hematology ◽

10.1182/asheducation-2009.1.602 ◽

2009 ◽

Vol 2009 (1) ◽

pp. 602-609 ◽

Cited By ~ 24

Author(s):

Peter Dreger

Keyword(s):

Chronic Lymphocytic Leukemia ◽

Immune Modulation ◽

Residual Disease ◽

Treatment Strategies ◽

Treatment Resistance ◽

Lymphocytic Leukemia ◽

Successful Outcome ◽

Mortality And Morbidity ◽

Poor Risk ◽

Leukemic Clone

AbstractEfforts to develop curative treatment strategies for chronic lymphocytic leukemia (CLL) in recent years have focused on allogeneic stem cell transplantation (alloSCT). The crucial anti-leukemic principle of alloSCT in CLL appears to be the immune-mediated anti-host activities conferred with the graft (graft-versus-leukemia effects, GVL). Evidence for GVL in CLL is provided by studies analyzing the kinetics of minimal residual disease on response to immune modulation after transplantation, suggesting that GVL can result in complete and durable suppression of the leukemic clone. AlloSCT from matched related or unrelated donors can overcome the treatment resistance of poor-risk CLL, ie, purine analogue refractory disease and CLL with del 17p-. Even with reduced-intensity conditioning, alloSCT in CLL is associated with significant mortality and morbidity due to graft-versus-host disease, which has to be weighed against the risk of the disease when defining the indication for transplantation. Therefore, it can be regarded as a reasonable treatment option only for eligible patients who fulfill accepted criteria for poor-risk disease. If alloSCT is considered, it should be performed before CLL has advanced to a status of complete refractoriness to assure an optimum chance for a successful outcome. Prospective trials are underway to prove whether allo-SCT can indeed change the natural history of poor-risk CLL.

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Clival chordoma molecular subtypes and clinical behavior

Journal of Clinical Oncology ◽

10.1200/jco.2009.27.15_suppl.10538 ◽

2009 ◽

Vol 27 (15_suppl) ◽

pp. 10538-10538

Author(s):

D. M. Park ◽

D. M. Prevedello ◽

P. Gardner ◽

D. Lee ◽

S. Henry ◽

...

Keyword(s):

Residual Disease ◽

Response To Therapy ◽

Treatment Modalities ◽

Proliferation Index ◽

Fish Analysis ◽

Prognostic Information ◽

Ki 67 ◽

Clinical Behavior ◽

Clival Chordoma

10538 Background: Chordoma is a primary bone cancer of the axial skeleton with predilection for local invasion. Primary treatment modalities consist of surgery and radiation therapy with limited role for chemotherapy. Because patients with chordoma exhibit heterogeneous clinical history and response to therapy, we investigated potential markers predictive of clinical behavior. Methods: This study analyzed 35 clival chordoma specimens with documented immunohistochemistry (IHC) staining for Ki-67 proliferation index from 29 patients. Detailed additional pathologic studies were performed prospectively on 12 consecutive patients. This consisted of the following: IHC for brachyury, cytokeratin, p53, and epidermal growth factor receptor (EGFR); fluorescent in situ hybridization (FISH) analysis for EGFR amplification and presence of 1p36, 9p21, and 19q13 loci; polymerase chain reaction based loss of heterozygosity analysis (LOH) of 1p, 9p, 10q, 17p, and 19p loci. Results: All confirmed chordoma specimens were positive for brachyury and negative for amplification of EGFR. Increase in Ki-67 index was associated with recurrence in 5 of 6 cases. Out of the 12 patients with detailed molecular pathologic studies, 5 had loss of 1p, 2 had deletion of 9p21 (p16), and 2 had loss of 10q (PTEN). The loss of 1p and 9p were detected via both FISH and LOH analyses. 3 of the 12 patients’ tumor specimens showed a Ki-67 index of greater than 10%; the remaining specimens were less than 5%. All 3 patients with high Ki-67 index had loss of either 9p (p16) or 10q (PTEN). The tumor specimen from these 3 patients also exhibited enhanced in vitro propagation. Conclusions: Subtyping of clival chordomas based on molecular phenotype may provide prognostic information. The prospective nature of this study in concert with assessment of residual disease after surgery may allow for identification of distinct subpopulations of patients at greater risk of disease recurrence for consideration of adjuvant therapy. The correlative in vitro study will provide an opportunity to evaluate targeted therapies customized to individual molecular subgroups. No significant financial relationships to disclose.

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A meta-analysis of tumor control rates and treatment-related morbidity for patients with glomus jugulare tumors

Journal of Neurosurgery ◽

10.3171/2010.9.jns10699 ◽

2011 ◽

Vol 114 (5) ◽

pp. 1299-1305 ◽

Cited By ~ 73

Author(s):

Michael E. Ivan ◽

Michael E. Sughrue ◽

Aaron J. Clark ◽

Ari J. Kane ◽

Derick Aranda ◽

...

Keyword(s):

English Language ◽

Meta Analysis ◽

Treatment Modalities ◽

Cranial Neuropathy ◽

Tumor Control ◽

Subtotal Resection ◽

Diverse Range ◽

Tumor Control Rate ◽

Glomus Jugulare

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.

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Management strategies for pediatric patients with tectal gliomas: a systematic review

Neurosurgical Review ◽

10.1007/s10143-021-01653-8 ◽

2021 ◽

Author(s):

Megan M. J. Bauman ◽

Archis R. Bhandarkar ◽

Clark R. Zheng ◽

Cecile Riviere-cazaux ◽

Cynthia J. Beeler ◽

...

Keyword(s):

Systematic Review ◽

Pediatric Population ◽

Obstructive Hydrocephalus ◽

Management Strategies ◽

Treatment Strategies ◽

Treatment Modalities ◽

Management Options ◽

Csf Diversion ◽

Aggressive Tumors ◽

Level I

AbstractPediatric tectal gliomas generally have a benign clinical course with the majority of these observed radiologically. However, patients often need treatment for obstructive hydrocephalus and occasionally require cytotoxic therapy. Given the lack of level I data, there is a need to further characterize management strategies for these rare tumors. We have therefore performed the first systematic review comparing various management strategies. The literature was systematically searched from January 1, 2000, to July 30, 2020, to identify studies reporting treatment strategies for pediatric tectal gliomas. The systematic review included 355 patients from 14 studies. Abnormal ocular findings—including gaze palsies, papilledema, diplopia, and visual field changes—were a common presentation with between 13.6 and 88.9% of patients experiencing such findings. CSF diversion was the most performed procedure, occurring in 317 patients (89.3%). In individual studies, use of CSF diversion ranged from 73.1 to 100.0%. For management options, 232 patients were radiologically monitored (65.4%), 69 received resection (19.4%), 30 received radiotherapy (8.4%), and 19 received chemotherapy (5.4%). When examining frequencies within individual studies, chemotherapy ranged from 2.5 to 29.6% and radiotherapy ranged from 2.5 to 28.6%. Resection was the most variable treatment option between individual studies, ranging from 2.3 to 100.0%. Most tectal gliomas in the pediatric population can be observed through radiographic surveillance and CSF diversion. Other forms of management (i.e., chemotherapy and radiotherapy) are warranted for more aggressive tumors demonstrating radiological progression. Surgical resection should be reserved for large tumors and/or those that are refractory to other treatment modalities.

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Long-term follow-up of lateral ventricular central neurocytoma treated with subtotal resection followed by concurrent chemoradiotherapy and add on chemotherapy – Case report from a Tertiary Kenyan Cancer Hospital

Surgical Neurology International ◽

10.25259/sni_389_2020 ◽

2020 ◽

Vol 11 ◽

pp. 272

Author(s):

Vijayakumar Narayanan ◽

Kiboi Julius ◽

James Mbogo

Keyword(s):

Concurrent Chemoradiotherapy ◽

Residual Disease ◽

Central Neurocytoma ◽

Treatment Modalities ◽

Subtotal Resection ◽

Transcallosal Approach ◽

Ventriculoperitoneal Shunting ◽

Microscopic Evaluation ◽

Long Term Follow Up

Background: Central neurocytomas are rare, mostly benign neuroectodermal tumors of the central nervous system typically located within the lateral and third ventricles of cerebrum. No consensus guidelines for the management of central neurocytoma available due to the rarity of the disease. Case Description: We report a case of right ventricular central neurocytoma of a 28-year-old lady who had a subtotal resection and ventriculoperitoneal shunting. Postoperatively, she was treated with concomitant chemotherapy with oral temozolomide and radiotherapy, followed by add-on chemotherapy with same drug. Imaging, microscopic evaluation, treatment modalities, and outcome of treatment are presented. Conclusion: Subtotal resection of tumor through transcallosal approach and ventriculoperitoneal shunt was performed. Imaging done 2 weeks postsurgery confirmed residual disease. Concurrent chemoradiotherapy (54 Gy in 30 fractions +Oral Temozolomide 75 mg/m2 daily), followed by six cycles of 5-day chemotherapy with temozolomide (150 mg/m2 in Cycle 1, and 175 mg/m2 in subsequent cycles) at 28-day intervals, was given. No major toxicities encountered. Follow-up scan after 36 months showed complete remission.

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Cervical Chordomas: Multicenter Case Series and Meta-analysis

10.21203/rs.3.rs-229633/v1 ◽

2021 ◽

Author(s):

Oluwaseun Akinduro ◽

Diogo P. Garcia ◽

Ricardo A Domingo ◽

Tito Vivas-Buitrago ◽

Bernardo Sousa-Pinto ◽

...

Keyword(s):

Meta Analysis ◽

Tertiary Care ◽

Case Series ◽

Tumor Control ◽

Subtotal Resection ◽

En Bloc ◽

Intention To Treat ◽

Risk Of Death ◽

Local Progression ◽

Current Series

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.

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New Trends and Developments in Patients with Severe Aortic Stenosis – Towards Optimal Treatment?

European Cardiology Review ◽

10.15420/ecr.2009.5.2.81 ◽

2009 ◽

Vol 5 (2) ◽

pp. 81 ◽

Cited By ~ 2

Author(s):

Martijn WA van Geldorp ◽

Johanna JM Takkenberg ◽

Ad JJC Bogers ◽

A Pieter Kappetein ◽

◽

...

Keyword(s):

Aortic Stenosis ◽

Severe Aortic Stenosis ◽

Surgical Techniques ◽

Treatment Strategies ◽

Tissue Doppler ◽

Doppler Imaging ◽

Treatment Modalities ◽

Diagnostic Tools ◽

Number Of Patients ◽

Transcatheter Valve

Over the next few decades the number of patients diagnosed with aortic stenosis is expected to rise as the population ages and the use of several diagnostic tools expands. This will result in a growing need for both medical and surgical treatment and stimulate the development of new diagnostic and surgical techniques. This article briefly describes the prevalence, pathogenesis and clinical presentation of patients with aortic stenosis and focuses on developments in diagnostic tools, treatment strategies and treatment modalities: the use of echocardiography, tissue Doppler imaging, stress testing and biomarkers is discussed, as well as timing of surgery and the role microsimulation can play in prosthesis selection. Furthermore, newly developed transcatheter valve implantation techniques and their possible role in treating ‘inoperable’ or ‘elderly’ patients are discussed.

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Pediatric ganglioglioma of the brainstem and cervicomedullary junction: a retrospective cohort study

Journal of Neurosurgery Pediatrics ◽

10.3171/2019.7.peds1961 ◽

2020 ◽

Vol 25 (1) ◽

pp. 30-36

Author(s):

Soliman Oushy ◽

Avital Perry ◽

Christopher S. Graffeo ◽

Aditya Raghunathan ◽

Lucas P. Carlstrom ◽

...

Keyword(s):

Tertiary Care ◽

Significant Risk ◽

Foot Drop ◽

Cranial Neuropathy ◽

Primary Study ◽

Tumor Control ◽

Subtotal Resection ◽

Low Grade ◽

Surgical Morbidity ◽

Cervicomedullary Junction

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.

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