Immune-Mediated Necrotizing Myopathies: Current Landscape.

PURPOSE OF REVIEW: Immune-mediated necrotizing myopathy (IMNM), characterized by acute or subacute onset, severe weakness, and elevated creatine kinase levels, poses diagnostic and therapeutic challenges. This article provides a succinct overview of IMNM, including clinical features, diagnostic strategies, and treatment approaches. RECENT FINDINGS: Recent insights highlight the different clinical presentations and therapeutic options of IMNM stratified by autoantibody positivity and type. Additionally, recent findings call into question the reported link between statin use and IMNM. This review synthesizes current knowledge on IMNM, emphasizing its distinct clinical features and challenging management. The evolving understanding of IMNM underscores the need for a comprehensive diagnostic approach that utilizes a growing range of modalities. Early and aggressive immunomodulatory therapy remains pivotal. Ongoing research aims to refine diagnostic tools and therapeutic interventions for this challenging muscle disorder, underscoring the importance of advancing our understanding to enhance patient outcomes.

Histopathological and molecular predictors of growth patterns and recurrence in craniopharyngiomas: a systematic review.

Craniopharyngiomas (CPs) are rare, benign tumors derived from Rathke's pouch, known for their high recurrence rates and associated morbidity and mortality. Despite significant investigation on risk factors for recurrence, a lack of consensus persists. Recent research suggests that specific histopathological and molecular characteristics are prognostic for disease progression. In this systematic review, we analyzed and consolidated key features of CPs that contribute to increased recurrence rates. This systematic review was performed in accordance with PRISMA guidelines. A search string was created with the keywords "craniopharyngioma," "histology," "histopathology," "molecular," and "recurrence." Literature was collected from 2006 to 2016 on the PubMed/Medline and Web of Science databases. The initial search resulted in 242 papers, examined with inclusion and exclusion criteria. The final review included a total of 37 studies, 36 primary studies covering a total of 1461 patients and 1 previous meta-analysis. Cystic lesions and whorl-like arrays were found to be associated with increased recurrence, while previously considered reactive gliosis and finger-shaped protrusions were not. The genetic elements found to be associated with increased risk of recurrence were Ki-67, Ep-CAM, PTTG-1, survivin, and certain RAR isotypes, as well as the glycoproteins osteonectin and chemokines CXCL12/CXCR4. The effects of VEGF, HIF-1α, and p53, despite extensive study, yielded conflicting results. Certain histopathological and molecular characteristics of CPs provide insight into their pathogenesis, likelihood of recurrence, and potential novel targets for therapy.

Outcomes following endoscopic endonasal resection of sellar and supresellar lesions in pediatric patients.

PURPOSE: The endoscopic endonasal approach (EEA) is a credible surgical alternative for the resection of sellar and suprasellar lesions such as pituitary adenomas, craniopharyngiomas, and Rathke cleft cysts. However, its application to pediatric patients poses several unique challenges that have not yet been well evaluated. The authors evaluate the safety, efficacy, and outcomes associated with the use of the EEA for treatment of these pathologic entities in pediatric patients. METHODS: Retrospective review of 30 patients between the ages of two and 24 who underwent endoscopic endonasal resection of sellar or suprasellar lesions between January 2010 and December 2015. Endocrinological and ophthalmological outcomes, as well as extent of resection and complications were all evaluated. RESULTS: Gross total resection was achieved in eight of the nine pituitary adenomas, nine of the 12 craniopharyngiomas, and six of the nine Rathke cleft cysts. Of the 30 patients, 22 remained disease free at last follow-up. A total of six patients developed hypopituitarism and five developed diabetes insipidus. Eleven patients experienced improved vision, sixteen experienced no change, and one patient experienced visual worsening. Postoperative cerebrospinal fluid leak was seen in a single case and later resolved, vasospasm/stroke was experienced by 10% of patients, and new obesity was recorded in 10% of patients. There were no perioperative deaths. CONCLUSIONS: Endoscopic endonasal resection is a safe and effective surgical alternative for the management of sellar and suprasellar pathologies in pediatric populations with excellent outcomes, minimal complications, and a low risk of morbidity.