Nerve sheath tumors involving the sacrum

Journal ArticleNerve sheath tumors that involve the sacrum are rare. Delayed presentation is common because of their slow-growing nature, the permissive surrounding anatomical environment, and nonspecific symptoms. Consequently, these tumors are usually of considerable size at the time of diagnosis. The authors discuss a case of a sacral nerve sheath tumor. They also propose a classification scheme for these tumors based on their location with respect to the sacrum into three types (Types I-III). Type I tumors are confined to the sacrum; Type II originate within the sacrum but then locally metastasize through the anterior and posterior sacral walls into the presacral and subcutaneous spaces, respectively; and Type III are located primarily in the presacral/retroperitoneal area. The overwhelming majority of sacral nerve sheath tumors are schwannomas. Neurofibromas and malignant nerve sheath tumors are exceedingly rare. Regardless of their histological features, the goal of treatment is complete excision. Adjuvant radiotherapy may be used in patients in whom resection was subtotal. Approaches to the sacrum can generally be classified as anterior or posterior. Type I tumors may be resected via a posterior approach alone, Type III may require an anterior approach, and Type II tumors usually require combined anterior-posterior surgery

Treatment of metastatic spinal epidural disease: a review of the literature

Journal ArticleObject. Spinal cord compression is one of the most dreaded complications of metastatic cancer. It can lead to a number of sequelae, including pain, spinal instability, neurological deficits, and a reduction in the patient's quality of life. Except in selected circumstances, treatment is palliative. Treatment options include surgery, radiation, and chemotherapy. The goal of this study was to summarize the existing data on the outcomes of various treatment methods for metastatic spinal epidural disease and to make appropriate recommendations for their use. Methods. The authors used a search strategy that included an electronic database search, a manual search of journals, analysis of bibliographies in relevant review papers, and consultation with the senior author. There is good evidence, including Class I data, that steroid drugs constitute a beneficial adjunctive therapy in patients with myelopathy from epidural compression. Historically, conventional radiation therapy has been viewed as the first-line treatment because it has been shown to be as effective as a decompressive laminectomy, with a lower incidence of complications (Class II data). Nevertheless, in the last 20 years there has been remarkable progress in surgical techniques and technology. Currently, the goals of surgery are to achieve a circumferential decompression of the spinal cord, and to reconstruct and immediately stabilize the spinal column. Results in a large body of literature support the belief that surgery is better at retaining or regaining neurological function than radiation and that surgery is highly effective in relieving pain. Most of the data on the treatment of metastatic spinal disease are Class II or III, but the preliminary results of a well-designed, randomized controlled trial in which surgery is compared with standard radiation therapy represents the first Class I data. Conclusions. As the number of treatment options for metastatic spinal disease has grown, it has become clear that effective implementation of these treatments can only be achieved by a multidisciplinary approach

OR noise: the potential for hearing loss

Journal ArticleWith the use of the electric or air-powered drill in cranial or spinal procedures comes the potential for hearing loss among operating team members. Exposure to loud noise can result in sensorineural hearing loss, and this loss is a function of sound pressure levels and duration of exposure. Much research has been done regarding the impact of noise-creating devices on patient and user safety in the field of neuro-otology (1, 6). Michaelides and Kartush measured peak and impulse sound pressure levels one centimeter away from their application in the temporal bone in a variety of otologic instruments, including lasers (potassium-titanyl-phosphate, erbium, and carbon dioxide) and drills (microdrill and pneumatic) (4). Only the erbium laser and air-powered cutting drills had impulse and peak sound pressure levels of greater than 100 A-weighted decibels, dBA. Kylen and colleagues found that the noise levels were influenced primarily by the size of the burr; diamond burrs generated less noise than cutting ones and variations in rotation speed had only a slight influence on the noise levels produced (2). Prasad and Reddy recently tested a variety of bone drills and microdebriders used in otolaryngological surgery and found them all to be safe, posing no occupational hazard to the user (7)

Stress and burnout: insidious influences

Journal ArticleA career in neurosurgery may take a substantial toll on an individual?s body and mind. From the ?baptism by fire? experienced by a brand-new junior resident, to keeping one?s head above water as the residency proceeds, to the realization shortly after graduation of being responsible for the health and welfare of one?s patients, and finally to the difficulties of lifelong challenges in patient care and administration, neurosurgeons are under continual stress that varies considerably among individuals and lasts for the duration of one?s career

Potentially useful outcome measures for clinical research in pediatric neurosurgery

Journal ArticleThe choice of outcome (or outcomes) and their measurement are critical for a sound clinical trial. Surgeons have traditionally measured simple outcomes such as death, duration of survival, or tumor recurrence but have recently developed more sophisticated measures of the effect of an intervention. Many outcome measures require a lengthy maturation process, which includes a determination of the instrument's validity, reliability, and sensitivity; thus, using established instruments rather than creating new ones is recommended. The authors illustrate several guidelines for the determination of appropriate outcome measures by using examples from their experience and describe several outcome measures that can be used in pediatric neurosurgery. These include general outcome measures such as the Pediatric Evaluation of Disability Inventory and the Functional Independence Measure for Children, which measure physical function and independence in chronically ill and disabled children as well as disease-specific measures for hydrocephalus (Hydrocephalus Outcome Questionnaire), cerebral palsy (gross motor function and performance measures), head injury (Pediatric Cerebral Performance Category and Children's Coma Scale), and oncology (Pediatric Cancer Quality-of-Life Inventory)

Marked reduction of cerebral vasospasm with lumbar drainage of cerebrospinal fluid after subarachnoid hemorrhage

Journal ArticleObject. Cerebral vasospasm after subarachnoid hemorrhage (SAH) continues to be a major source of morbidity in patients despite significant clinical and basic science research. Efforts to prevent vasospasm by removing spasmogens from the subarachnoid space have produced mixed results. The authors hypothesize that lumbar cisternal drainage can remove blood from the basal subarachnoid spaces more effectively than an external ventricular drain (EVD). This nonrandomized, controlled-cohort study was undertaken to evaluate the effectiveness of a lumbar drain in patients with SAH compared with those in whom an EVD or no form of cerebrospinal fluid (CSF) drainage was used to prevent the development of clinical vasospasm and its sequelae. Methods. The authors collected data on 266 patients with nontraumatic SAH who were admitted to the University of Utah Health Sciences Center between January 1994 and January 2003. Of these, 167 met the study entry criteria. The treatment group consisted of 81 patients in whom a lumbar drain had been placed for CSF shunting, whereas the control group was composed of 86 patients who received no form of CSF drainage or who were treated solely with an EVD. Primary outcome measures were as follows: 1) clinically evident vasospasm; 2) the need for endovascular intervention; 3) vasospasm-induced infarction; 4) disposition at time of discharge; and 5) Glasgow Outcome Scale (GOS) score at 1 to 3 months postdischarge. Secondary outcomes included length of stay and the need for CSF shunting. The presence of a lumbar drain conferred a statistically significant protective and beneficial effect across all outcome measures, reducing the incidence of clinical vasospasm from 51 to 17%, the need for angioplasty from 45 to 17%, and the occurrence of vasospastic infarction from 27 to 7% (all p < 0.001-0.008). Patients in the treatment group were more likely to be discharged home (54% compared with 25%, p = 0.002) and to have a GOS score of 5 at follow up (71% compared with 35%, p < 0.001). The mean number of days spent in the intensive care unit and in the hospital overall was also fewer in the treatment group. A similar degree of benefit was found in patients with different Fisher grades and regardless of whether an EVD was needed on presentation, both by subgroup analysis and multivariate logistic regression modeling. There was no statistical difference between the groups in terms of patients requiring a shunt. Complications with lumbar drains were rare and yielded no permanent sequelae. Conclusions. Shunting of CSF through a lumbar drain after an SAH markedly reduces the risk of clinically evident vasospasm and its sequelae, shortens hospital stay, and improves outcome. Its beneficial effects are probably mediated through the removal of spasmogens that exist in the CSF. The results of this study warrant a randomized clinical trial, which is currently under way

Assessing the validity of the endoscopic shunt insertion trial: did surgical experience affect the results?

Journal ArticleObject. Most surgical procedures are associated with a learning curve in which the success rate is lower early in the experience before mistakes have been identified and modifications made to the procedure. Negative results obtained early in a trial's learning curve may be a matter of timing rather than a reflection of the procedure's effectiveness. The recently published results of the Endoscopic Shunt Insertion Trial (ESIT) represent the notion that endoscopically placed shunts were no more likely to survive than conventionally placed shunts. This negative result may be due to inexperience in performing endoscopic surgeries. Methods. Surgical experience was assessed in two ways. Shunt survival rates were compared between cases treated endoscopically in the 1st and last years of the ESIT. The effect of center volume was evaluated using a Cox proportional hazard model in which the following variables were analyzed: age at registration, the diagnosis of myelomeningocele, head size, method of shunt placement (endoscopic compared with conventional), and center volume. There was no difference in survival (endurance) of the shunt between patients enrolled in the 1st and last years (log rank = 0.08, p = 0.77). Likewise, no variable in the Cox multivariate model, including center volume, was a significant factor in predicting shunt survival. Conclusions. The primary result of the ESIT was found to be internally valid. The fact that endoscopic shunt placement did not benefit patients evaluated in the study was not due to early timing of the trial. Any learning curve among the participating surgeons did not adversely affect the results

Clinical outcomes and patient-matched molecular composition of relapsed medulloblastoma

© 2021 by American Society of Clinical Oncology. Creative Commons Attribution Non-Commercial No Derivatives 4.0 License: https://creativecommons.org/licenses/by-nc-nd/4.0/Purpose: We sought to investigate clinical outcomes of relapsed medulloblastoma and to compare molecular features between patient-matched diagnostic and relapsed tumors. Methods: Children and infants enrolled on either SJMB03 (NCT00085202) or SJYC07 (NCT00602667) trials who experienced medulloblastoma relapse were analyzed for clinical outcomes, including anatomic and temporal patterns of relapse and postrelapse survival. A largely independent, paired molecular cohort was analyzed by DNA methylation array and next-generation sequencing. Results: A total of 72 of 329 (22%) SJMB03 and 52 of 79 (66%) SJYC07 patients experienced relapse with significant representation of Group 3 and wingless tumors. Although most patients exhibited some distal disease (79%), 38% of patients with sonic hedgehog tumors experienced isolated local relapse. Time to relapse and postrelapse survival varied by molecular subgroup with longer latencies for patients with Group 4 tumors. Postrelapse radiation therapy among previously nonirradiated SJYC07 patients was associated with long-term survival. Reirradiation was only temporizing for SJMB03 patients. Among 127 patients with patient-matched tumor pairs, 9 (7%) experienced subsequent nonmedulloblastoma CNS malignancies. Subgroup (96%) and subtype (80%) stabilities were largely maintained among the remainder. Rare subgroup divergence was observed from Group 4 to Group 3 tumors, which is coincident with genetic alterations involving MYC, MYCN, and FBXW7. Subgroup-specific patterns of alteration were identified for driver genes and chromosome arms. Conclusion: Clinical behavior of relapsed medulloblastoma must be contextualized in terms of up-front therapies and molecular classifications. Group 4 tumors exhibit slower biological progression. Utility of radiation at relapse is dependent on patient age and prior treatments. Degree and patterns of molecular conservation at relapse vary by subgroup. Relapse tissue enables verification of molecular targets and identification of occult secondary malignancies.info:eu-repo/semantics/publishedVersio

Molecular heterogeneity and CXorf67 alterations in posterior fossa group A (PFA) ependymomas

Of nine ependymoma molecular groups detected by DNA methylation profiling, the posterior fossa type A (PFA) is most prevalent. We used DNA methylation profiling to look for further molecular heterogeneity among 675 PFA ependymomas. Two major subgroups, PFA-1 and PFA-2, and nine minor subtypes were discovered. Transcriptome profiling suggested a distinct histogenesis for PFA-1 and PFA-2, but their clinical parameters were similar. In contrast, PFA subtypes differed with respect to age at diagnosis, gender ratio, outcome, and frequencies of genetic alterations. One subtype, PFA-1c, was enriched for 1q gain and had a relatively poor outcome, while patients with PFA-2c ependymomas showed an overall survival at 5 years of > 90%. Unlike other ependymomas, PFA-2c tumors express high levels of OTX2, a potential biomarker for this ependymoma subtype with a good prognosis. We also discovered recurrent mutations among PFA ependymomas. H3 K27M mutations were present in 4.2%, occurring only in PFA-1 tumors, and missense mutations in an uncharacterized gene, CXorf67, were found in 9.4% of PFA ependymomas, but not in other groups. We detected high levels of wildtype or mutant CXorf67 expression in all PFA subtypes except PFA-1f, which is enriched for H3 K27M mutations. PFA ependymomas are characterized by lack of H3 K27 trimethylation (H3 K27-me3), and we tested the hypothesis that CXorf67 binds to PRC2 and can modulate levels of H3 K27-me3. Immunoprecipitation/mass spectrometry detected EZH2, SUZ12, and EED, core components of the PRC2 complex, bound to CXorf67 in the Daoy cell line, which shows high levels of CXorf67 and no expression of H3 K27-me3. Enforced reduction of CXorf67 in Daoy cells restored H3 K27-me3 levels, while enforced expression of CXorf67 in HEK293T and neural stem cells reduced H3 K27-me3 levels. Our data suggest that heterogeneity among PFA ependymomas could have clinicopathologic utility and that CXorf67 may have a functional role in these tumors