Potential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor.

Intracranial mesenchymal tumor (IMT), FET::CREB fusion-positive is a provisional tumor type in the 2021 WHO classification of central nervous system tumors with limited information available. Herein, we describe five new IMT cases from four females and one male with three harboring an EWSR1::CREM fusion and two featuring an EWSR1::ATF1 fusion. Uniform manifold approximation and projection of DNA methylation array data placed two cases to the methylation class "IMT, subclass B", one to "meningioma-benign" and one to "meningioma-intermediate". A literature review identified 74 cases of IMTs (current five cases included) with a median age of 23 years (range 4-79 years) and a slight female predominance (female/male ratio = 1.55). Among the confirmed fusions, 25 (33.8%) featured an EWSR1::ATF1 fusion, 24 (32.4%) EWSR1::CREB1, 23 (31.1%) EWSR1::CREM, one (1.4%) FUS::CREM, and one (1.4%) EWSR1::CREB3L3. Among 66 patients with follow-up information available (median: 17 months; range: 1-158 months), 26 (39.4%) experienced progression/recurrences (median 10.5 months; range 0-120 months). Ultimately, three patients died of disease, all of whom underwent a subtotal resection for an EWSR1::ATF1 fusion-positive tumor. Outcome analysis revealed subtotal resection as an independent factor associated with a significantly shorter progression free survival (PFS; median: 12 months) compared with gross total resection (median: 60 months; p < 0.001). A younger age (< 14 years) was associated with a shorter PFS (median: 9 months) compared with an older age (median: 49 months; p < 0.05). Infratentorial location was associated with a shorter overall survival compared with supratentorial (p < 0.05). In addition, the EWSR1::ATF1 fusion appeared to be associated with a shorter overall survival compared with the other fusions (p < 0.05). In conclusion, IMT is a locally aggressive tumor with a high recurrence rate. Potential risk factors include subtotal resection, younger age, infratentorial location, and possibly EWSR1::ATF1 fusion. Larger case series are needed to better define prognostic determinants in these tumors.

Frailty in middle cranial fossa approach for encephalocele or cerebrospinal fluid leak repair.

Objective: The modified 5-item frailty index (mFI-5) is a concise, comorbidity-based risk stratification tool that can predict adverse outcomes after surgery. The goal of this study was to understand the frailty of patients undergoing surgery for temporal encephalocele or cerebrospinal fluid (CSF) leak and the utility of mFI-5 for predicting increased post-operative outcomes. Methods: A retrospective review of adults with temporal encephalocele or CSF leak who underwent middle cranial fossa (MCF) approach craniotomies with or without mastoidectomy from January 2015 through August 2021 at a tertiary care academic medical center was performed. Patients who underwent additional surgeries or extended surgical approaches were excluded. The mFI-5 was calculated for all patients. Demographic and clinical data were obtained from the medical record. Results: Thirty-six patients underwent 40 MCF approach craniotomies for temporal encephalocele or CSF leak, including three revision cases and one patient with sequential bilateral operations. Mean age was 54.1 ± 10.8 years, and 66.7% were female. In the univariable regression analysis, mFI-5 score, age, and procedure time use were significantly associated with increased hospital length of stay (LOS) but not increased intensive care unit (ICU) LOS. Anesthesia time and lumbar drain were significantly associated with increased hospital LOS and ICU LOS, and they remained significantly associated with increased hospital LOS in the multivariable model. Conclusion: Frailty is associated with increased hospital LOS stay among patients undergoing MCF approach for CSF leak or encephalocele. Reducing anesthesia time and avoiding lumbar drain use are potentially modifiable risk factors that can reduce the LOS and associated costs. Level of Evidence: 4.

Low-Pressure Hydrocephalus in Spontaneous Angiogram-Negative Subarachnoid Hemorrhage Following COVID-19 Infection.

A preliminary report warned that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) could have neuro-invasive potential as it was observed that some patients showed neurologic symptoms such as headache, nausea, and vomiting. Following early speculation there have been reports of neurologic manifestations involving both the central nervous system and peripheral nervous system including reports that coronavirus disease 2019 (COVID-19) may increase the risk of acute ischemic stroke. Here we present a patient with recent COVID-19 infection who experienced low-pressure hydrocephalus requiring high-output cerebrospinal fluid (CSF) diversion following spontaneous angiogram-negative subarachnoid hemorrhage. We hypothesize that patients who are either currently or who have recently been infected with SARS-CoV-2 may have altered ventricular compliance and/or altered CSF hydrodynamics from mechanisms that are not yet understood but potentially related to previously described pathophysiologic mechanisms of the virus and associated inflammatory reaction.

Downstaging of cervical cancer in Tanzania over a 16-year period.

Globally, the highest cervical cancer mortality rates are found in East Africa. Visual inspection with acetic acid (VIA)-based screening in resource-poor settings has been shown to decrease the proportion of women presenting with late-stage cervical cancer, a process known as clinical downstaging. The only cancer treatment center in Tanzania, Ocean Road Cancer Institute (ORCI) in Dar es Salaam, opened a VIA-based cervical cancer screening program in 2002. We reviewed 6,676 medical records of cervical cancer patients at the ORCI from 2002-2011 to 2014-2018 for stage at diagnosis and screening status, among other variables. We investigated whether clinical downstaging occurred in this period among women screened at the ORCI, when compared to unscreened women. Our results indicated that the proportion of women presenting with late-stage cervical cancer among women screened at the ORCI decreased by 27.7% over the 16-year period (χ2 = 16.99; p = 0.0002). Among unscreened women, a non-significant 13.2% decrease in late-stage disease was observed (χ2 = 1.74; p = 0.4179). Our results suggest clinical downstaging occurred among women screened at the ORCI over the 16-year period, and this difference may be attributed to the screening program as the same decrease in stage was not observed among unscreened women during the same time period. At present, less than one percent of Tanzanian women receive yearly cervical cancer screenings. Access to screening through expansion of the ORCI screening clinic and the creation of more clinics should be prioritized.

The history of neurosurgery at the University of Nebraska Medical Center.

The Department of Neurosurgery at the University of Nebraska Medical Center has grown considerably from one neurosurgeon in 1923 into a first-class department with diverse subspecialty care and innovative faculty. Founding neurosurgeon Dr. J. Jay Keegan, a student of Harvey Cushing, instituted a legacy of clinical and research excellence that he passed on to his successors. The department created a lecture series to honor Keegan's pioneering techniques and impact in the field, featuring prominent neurosurgeons from across the country. Keegan's successors, such as Dr. Lyal Leibrock, grew the department through a unique partnership with private practice. The current faculty has continued the tradition of exceptional resident training and innovative patient care.

Clinical and Short-Term Radiographic Outcomes of Minimally Invasive Transforaminal Lumbar Interbody Fusion With Expandable Lordotic Devices.

BACKGROUND: Minimally invasive transforaminal lumbar interbody fusion (MIS-TLIF) is a well-accepted procedure for the treatment of degenerative lumbar disease. However, its ability to restore lumbar lordosis has been limited. Development of expandable lordotic interbody devices has challenged this limitation, furthering the scope of minimally invasive surgery. OBJECTIVE: To evaluate the radiographic and clinical effects of expandable lordotic interbody devices placed through an MIS-TLIF approach. METHODS: We conducted a retrospective review of 32 1-level and 18 2-level MIS-TLIFs performed using lordotic expandable interbody devices. Lumbar radiographic measurements, Oswestry Disability Index scores (ODI), and Visual Analogue Scale scores (VAS) were obtained at preoperative, 6 wk follow up, and last follow up time points. Last follow up occurred at a mean of 11.5 ± 7.6 mo (mean ± SD). RESULTS: At 6-wk follow-up, segmental lordosis, disc height, and foraminal height increased by an average of 3.4°, 6.4 mm, and 4.4 mm, respectively. Only the 2-level group showed a significant increase in lumbar lordosis of 5.8°. No significant changes occurred in sacral slope, pelvic tilt, or pelvic incidence. Average ODI and VAS decreased by -12.0 and -4.5, respectively. Postoperative lumbar lordosis inversely correlated with preoperative lordosis in patients with an initial Pelvic Incidence to Lumbar Lordosis mismatch (PI-LL) of >10°, (r = -0.5, P = .009). CONCLUSION: When applied across 2-levels, MIS-TLIF using expandable lordotic interbody devices produced a significant increase in lumbar lordosis. Preoperative lumbar lordosis was found to be a predictor of postoperative lumbar lordotic change in patients with sagittal imbalance.

Hemorrhage Rate After External Ventricular Drain Placement in Subarachnoid Hemorrhage: Time to Heparin Administration.

OBJECTIVE: The use of antiplatelet or anticoagulants has previously been shown to increase hemorrhagic complications of ventricular catheterization. Although heparin use 24 h after ventriculostomy appears safe, the safety of heparin immediately (within 4 h) after ventriculostomy is unknown. The objective of this study was to assess the safety of heparin immediately (within 4 h) after ventriculostomy in subarachnoid hemorrhage (SAH) patients undergoing endovascular treatment. PATIENTS AND METHODS: This is a retrospective cohort study of 46 patients with aneurysmal SAH secondary to aneurysm rupture who required ventriculostomy. Post-ventriculostomy imaging was carefully reviewed for tract hemorrhaging. Timing of heparinization was noted. Early heparinization was within 4 h after ventriculostomy, and intermediate heparinization was between 4 and 24 h after ventriculostomy. RESULTS: Overall, the tract hemorrhage rate was 26.1% for the study cohort-mostly grade I tract hemorrhages-consistent with the existing literature. The tract hemorrhage rate in the early (<4 h) heparin group was a remarkable 58.8%. The hemorrhages were also notably larger in the early (<4 h) heparin group. CONCLUSION: Although heparin appears to be safe after 4 h, immediate heparinization (within 4 h) after ventriculostomy significantly increases the odds of tract hemorrhage. Additional time should be afforded between ventriculostomy and heparinization to avoid potentially devastating external ventricular drain tract hemorrhage. It is advisable to wait a sufficient time (at least 4 h) after ventriculostomy before embarking on endovascular treatment of ruptured aneurysms.

Multinodular and Vacuolating Neuronal Tumor: A Rare Seizure-associated Entity.

Multinodular and vacuolating neuronal tumor is a recently described seizure-associated entity with overlapping features of a malformative and neoplastic process. We report a case of multinodular and vacuolating neuronal tumor in a 29-year-old man with a history of recent headaches and complex partial seizures. Neuroimaging revealed a nonenhancing, T2 and T2 fluid-attenuated inversion recovery hyperintense multinodular lesion in the right temporal lobe. Lesional tissue demonstrated well-demarcated nodules of ganglioid cells with vacuolation of both the perikarya and the fibrillary neuropil-like background. The ganglioid cells showed weak cytoplasmic reactivity for synaptophysin and were nonreactive for neurofilament and chromogranin. CD34-positive stellate cells were present within the nodules. A 50-gene next-generation sequencing panel did not identify any somatic mutations in genomic DNA extracted from the tumor.

Perineal pain secondary to tethered cord syndrome: retrospective review of single institution experience.

OBJECT: Tethered cord syndrome (TCS) encompasses a spectrum of neurological dysfunction related to excessive tension on the distal spinal cord resulting in anatomic deformation and metabolic disturbance. Symptoms typically manifest as back/leg pain, neurogenic bladder dysfunction, constipation, sphincter abnormalities, and scoliosis. To date, among the least well-described symptoms of TCS is pain or hypersensitivity in the perineal region. The authors reviewed their experience with spinal cord detethering to identify and further characterize those who present with perineal pain or hypersensitivity. METHODS: Cases of spinal cord detethering at a single institution were retrospectively reviewed. Patients were initially identified by procedural codes. Cases were reviewed for presenting symptoms, specifically perineal pain or hypersensitivity. Magnetic resonance image (MRI) findings, clinical outcome, and length of follow-up were also noted. RESULTS: Of the 491 patients identified, seven patients (1.4%) were identified as having preoperative perineal pain or hypersensitivity. All of these patients had complete resolution of perineal pain/hypersensitivity at the time of last follow-up. Furthermore, five (71%) of these patients experienced resolution of all initial symptoms. CONCLUSION: Perineal pain or hypersensitivity can be an important symptom of spinal cord tethering. Spinal cord detethering may result in a good outcome and relief of perineal pain or hypersensitivity.

Most women diagnosed with cervical cancer by a visual screening program in Tanzania completed treatment: evidence from a retrospective cohort study.

BACKGROUND: Visual inspection with acetic acid (VIA) to identify and treat pre-cancerous lesions is effective for cervical cancer prevention. Screening programs also facilitate screening and diagnosis of invasive cancers that must be referred for radiation therapy or chemotherapy. This study compared characteristics of women diagnosed with invasive cervical cancer by a VIA screening program who did and did not follow up for treatment and who did and did not complete treatment at the Ocean Road Cancer Institute (ORCI), Dar es Salaam, Tanzania. METHODS: We conducted a retrospective cohort study of ORCI screening referrals from the period November 2002 to June 2011. Women referred for treatment of invasive disease (n = 980) were identified from an existing database of all women attending the screening clinic during this period (n = 20,131) and matched to a dataset of all cervical cancer patients attending ORCI in this period (n = 8,240). Treatment information was abstracted from patient records of women who followed up. Records of a random sample (n = 333) of unscreened patients were reviewed for disease stage. RESULTS: Of the 980 women referred women, 829 (84.6%) sought treatment. Most of those women (82.8%) completed their prescribed radiation. Lower disease stage, having a skilled occupation, residence in Dar es Salaam, and younger age were independently associated with loss to follow-up. Higher disease stage, residence in Dar es Salaam, older age, and later year of first treatment appointment were independently associated with incomplete treatment among those who followed up. Significantly more screened women had stage 1 disease (14.0%) than unscreened women (7.8%). CONCLUSIONS: Most women referred from the screening clinic completed treatment for their cancer at ORCI. Some of those lost to follow-up may have sought treatment elsewhere. In most cases, the screening clinic appears to facilitate diagnosis and treatment, rather than screening, for women with invasive cervical cancer.

Intracranial malignant triton tumor in a patient with neurofibromatosis type 1: case report and review of the literature.

We report the fourth case of an intracranial malignant triton tumor not associated with a cranial nerve in a 26-year-old male with a clinical history of neurofibromatosis type 1. The patient was found unresponsive and displayed confusion, lethargy, hyperreflexia, and dysconjugate eye movements upon arrival at the emergency room. MRI revealed a large bifrontal mass. Biopsy demonstrated a high-grade spindle cell tumor with focal areas of rhabdomyoblasts that stained positive for desmin, myogenin, and muscle-specific actin. Electron microscopy showed skeletal muscle differentiation. Based on the clinical history of NF1 and the pathologic results, a diagnosis of malignant triton tumor was made. The differential diagnosis, immunohistochemistry, molecular genetics, and treatment of malignant triton tumor are reviewed.

Remote cerebellar hemorrhage following supratentorial cerebrovascular surgery.

Three patients with remote cerebellar hemorrhage following supratentorial cerebrovascular surgery are presented. Remote cerebellar hemorrhage is a rare surgical complication that is most often associated with aneurysm clipping or temporal lobectomies. Bleeding occurs on the superior cerebellar cortex and is believed to be venous in origin. The precise pathogenesis of remote cerebellar hemorrhage has yet to be fully elucidated but is generally considered to be a consequence of intraoperative cerebrospinal fluid loss causing caudal displacement of the cerebellum with resultant stretching of the supracerebellar veins. This case series will hopefully shed further light on the incidence, presentation, workup, and treatment of this particular complication of supratentorial surgery.

Intraventricular tissue plasminogen activator for intraventricular hemorrhage caused by an arteriovenous malformation.

The use of thrombolytics delivered through an external ventricular drain has improved outcomes in intraventricular hemorrhage, a disease with a poor prognosis; however, presence of an arteriovenous malformation is generally considered a contraindication to thrombolytic use. Due do the high mortality with the current standard of care, thrombolytics should be considered as an acceptable treatment option despite the presence of an arteriovenous malformation in certain clinical situations. We review the available literature and present an additional patient to make the case for the use of thrombolytics for intraventricular hemorrhage from an arteriovenous malformation.

Analysis of adjacent segment degeneration with laminectomy above a fused lumbar segment.

Although recent data suggests that lumbar fusion with decompression contributes to some marginal acceleration of adjacent segment degeneration (ASD), few studies have evaluated whether it is safe to perform a laminectomy above a fused segment. This study investigates the hypothesis that laminectomy above a fused lumbar segment does not increase the incidence of ASD, and assesses the benefits and risks of performing a laminectomy above a lumbar fusion. A retrospective review of 171 patients who underwent decompression and instrumented fusion of the lumbar spine was performed to analyze the association between ASD and laminectomy above the fused lumbar segment. Patients were divided into two groups - one group with instrumented fusion alone and the other group with instrumented fusion plus laminectomy above the fused segment. Of the 171 patients, 34 underwent additional decompressive laminectomy above the fused segment. There was a significant increase in ASD incidence as well as progression of ASD grade in both groups. There was no significant increase in ASD in patients with decompressive laminectomy above the fused lumbar segment compared to patients with laminectomy limited to the fused segment. This retrospective review of 171 patients who underwent decompression and instrumented fusion with follow-up radiographs demonstrates that laminectomy decompression above a fused segment does not significantly increase radiographic ASD. There is, however, a significant increase in ASD over time, which was observed throughout the entire cohort likely representing a natural progression of lumbar spondylosis above the fusion segment.