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Objective: Olfactory neuroblastoma is a rare sinonasal malignancy with comparatively positive prognosis and survival, but with a range of biological behaviors that can be difficult to prognosticate with current means of risk stratification. Neutrophil‐to‐lymphocyte ratio (NLR) has been found across a diverse range of malignancies to be associated with poorer outcomes. This paper aims to elucidate the relationship of NLR with olfactory neuroblastoma to assess its prognostic value in this setting. Study Design: Retrospective chart review. Setting: A single tertiary care academic hospital. Methods: The study cohort included all patients treated for initial presentation of olfactory neuroblastoma from 2004 to 2020. NLR was calculated from preoperative labs, and each patient was evaluated for Kadish staging, Hyams grade, intraoperative positive margin, use of adjuvant therapy, posttreatment recurrence, and death. All statistical analysis was conducted using R and relationship between NLR and variables was assessed via binomial logistic regression. Results: Forty‐four patients were included, 24 were male. Average age 52.8, average length of follow‐up was 9.6 years. Patients were grouped by low (Kadish A/B) and advanced (Kadish C/D) stage, n = 23 and n = 21, respectively, and low (Hyams I/II) and high (Hyams III/IV) risk, n = 15 and n = 11, respectively. Advanced Kadish stage was associated with elevated NLR, odds ratio 5.69 [2.30, 20.7], P =.001. No other variables were associated with elevated NLR including Hyams grade, margin status, recurrence, and mortality. Conclusion: Higher Kadish grade is associated with elevated NLR which may provide novel prognostic value to current risk‐stratifying systems. [ABSTRACT FROM AUTHOR]

Key Points: Resumption of continuous positive airway pressure (CPAP) in the immediate postoperative period after endoscopic endonasal approaches (EEA) for pituitary adenomas can be safe. [ABSTRACT FROM AUTHOR]

This article discusses the use of endoscopic endonasal approaches (EEA) in the management of foramen magnum meningiomas (FMM). FMM is a rare type of intracranial meningioma, and traditional treatment involves transcranial corridors. The study presents the experience of the authors in using EEA for FMM resection and highlights its role and limitations. The results show that EEA can be an alternative corridor for carefully selected patients with ventral FMM, especially when involvement or encasement of the anterior spinal arteries is suspected. However, CSF leak remains a significant limitation. [Extracted from the article]

Background: Ebstein's anomaly (EA) is a rare congenital heart disease of the tricuspid valve and right ventricle. Patients with EA often manifest with left ventricular noncompaction (LVNC), a cardiomyopathy. Despite implication of cardiac sarcomere genes in some cases, very little is understood regarding the genetic etiology of EA/LVNC. Our study describes a multigenerational family with at least 10 of 17 members affected by EA/LVNC. Methods: We performed echocardiography on all family members and conducted exome sequencing of six individuals. After identifying candidate variants using two different bioinformatic strategies, we confirmed segregation with phenotype using Sanger sequencing. We investigated structural implications of candidate variants using protein prediction models. Results: Exome sequencing analysis of four affected and two unaffected members identified a novel, rare, and damaging coding variant in the Kelch‐like family member 26 (KLHL26) gene located on chromosome 19 at position 237 of the protein (GRCh37). This variant region was confirmed by Sanger sequencing in the remaining family members. KLHL26 (c.709C > T p.R237C) segregates only with EA/LVNC‐affected individuals (FBAT p <.05). Investigating structural implications of the candidate variant using protein prediction models suggested that the KLHL26 variant disrupts electrostatic interactions when binding to part of the ubiquitin proteasome, specifically Cullin3 (CUL3), a component of E3 ubiquitin ligase. Conclusion: In this familial case of EA/LVNC, we have identified a candidate gene variant, KLHL26 (p.R237C), which may have an important role in ubiquitin‐mediated protein degradation during cardiac development. [ABSTRACT FROM AUTHOR]

The inside cover image is based on the Original Article I Novel KLHL26 variant associated with a familial case of Ebsteinss anomaly and left ventricular noncompaction i by Sai Suma K. Samudrala et al., https://doi.org/10.1002/mgg3.1152. GLO:FU4V/01apr20:mgg31271-toc-0001.jpg PHOTO (COLOR): . gl. [Extracted from the article]