CNS Oncology最新文献

{"title":"Synchronous diagnosis of multicentric glioma with distinct isocitrate dehydrogenase molecular profiles: a case report.","authors":"Aditya A Mohan, Kristen Batich, Shih-Hsiu J Wang, Giselle Y López, Michael E Salacz, Katherine B Peters","doi":"10.1080/20450907.2026.2626299","DOIUrl":"10.1080/20450907.2026.2626299","url":null,"abstract":"<p><p>This case report characterizes the molecular pathology of two synchronous IDH mutant gliomas in a 28-year-old female patient. The patient exhibited symptoms of dizziness, retro-orbital pain, headache, and numbness with paresthesia in her right arm. MRI imaging revealed two distinct non-enhancing T2/FLAIR hyperintense lesions in the left frontal and parietal lobes. Histopathologic and molecular analyses, including whole exome sequencing, were performed on the resected specimens from each location. The left parietal tumor was diagnosed as a grade 4 astrocytoma with an IDH1 R132H mutation, while the left frontal tumor was classified as a grade 2 oligodendroglioma with an IDH1 R132S mutation. Given the distinct molecular profiles of both synchronous tumors, treatment consideration was given to each individual primary tumor.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"15 1","pages":"2626299"},"PeriodicalIF":0.0,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12915774/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146164401","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Arginine deprivation induces prolonged autophagy activation and ROS-dependent cell death in glioblastoma multiforme.","authors":"Maya Abdelkhalek, Yasmin El-Jawhari, Maha Shaheen, Rawan Saad, Majd Sarieddine, Nour El-Husseini, Mirvat El-Sibai, Ralph J Abi-Habib","doi":"10.1080/20450907.2026.2666026","DOIUrl":"https://doi.org/10.1080/20450907.2026.2666026","url":null,"abstract":"<p><strong>Background: </strong>Arginine deprivation was shown to be selectively cytotoxic to GBM cells with cell death being caspase-independent, non-apoptotic. In this study, we assess the impact of prolonged arginine deprivation on GBM cells, the potential activation of autophagy, the mechanism of its activation, and its impact on cell cytotoxicity.</p><p><strong>Methods: </strong>GBM cell lines A172 and U251 were used in this study and arginine deprivation was induced by a cobalt-substituted, PEGylated human arginase I [HuArgI (Co)-PEG5000].</p><p><strong>Results: </strong>Arginine deprivation induced prolonged cytotoxicity up to 120 hours post-treatment. Cytotoxicity persisted despite cells overexpressing ASS-1. Arginine deprivation also induced a marked and sustained activation of autophagy, starting at 24 hours and lasting up to 120 hours post-treatment. Autophagy was shown to induce cell death since its inhibition by chloroquine significantly decreased cytotoxicity, indicating that the extensive activation of autophagy is resulting in cell death of GBM cells. Moreover, ROS was shown to accumulate in treated cells and neutralizing ROS using NAC did not affect the autophagic response but led to a complete reversal of cytotoxicity, demonstrating that death by autophagy is dependent on ROS generation.</p><p><strong>Conclusion: </strong>Arginine deprivation leads to sustained activation of autophagy and subsequent ROS-dependent cell death in GBM cells.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"15 1","pages":"2666026"},"PeriodicalIF":0.0,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833780","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diffuse pediatric-type high grade glioma, RTK1 subtype, subclass C with <i>SYN2</i>::<i>PPARG</i> fusion in an older adult.","authors":"Mikaela N Brentlinger, Jemini Patel, Ahmed Khan, Eduardo Castro-Echeverry, Zied Abdullaev, Kenneth Aldape, Norman L Lehman","doi":"10.1080/20450907.2026.2641996","DOIUrl":"10.1080/20450907.2026.2641996","url":null,"abstract":"<p><strong>Abstract: </strong>Diffuse pediatric-type high-grade glioma (pHGG), RTK1 subtype, is an uncommon aggressive tumor affecting both children and adults. We describe the case of a 66-year-old woman who presented with a left frontal lobe mass. Following surgical resection, the patient developed herpes simplex virus 2 meningoencephalitis, resulting in death. Histological examination revealed a high-grade glioma demonstrating nuclear pleomorphism, high mitotic activity, vascular proliferation and necrosis. The tumor also exhibited oligodendroglial-like features, nuclear clusters and small true rosette-like structures. Genetic analysis identified partial arm 1p loss and 19q loss, <i>PDGFRA</i>, <i>MYCN</i> and <i>MDM4</i> amplification, an <i>ATRX</i> mutation and a novel <i>SYN2::PPARG</i> fusion. Homozygous <i>CDKN2A/B</i> deletion was also present. Genomic DNA methylation profiling matched diffuse pediatric-type high-grade glioma, RTK1 subtype, subclass C. This case underscores the importance of utilizing advanced molecular and genomic techniques for accurately diagnosing glial tumors. Further study of the <i>SYN2</i>::<i>PPARG</i> fusion in gliomas could potentially offer insights into its role in glioma biology and possibly help elucidate therapeutic strategies for tumors with <i>PPARG</i> fusions.</p><p><strong>Article highlights: </strong>We report a case of diffuse pediatric-type high-grade glioma (pHGG), RTK1 subtype, subclass C  in an adult confirmed by genomic DNA methylation analysis. The tumor demonstrated <i>PDGFRA</i>, <i>MYCN</i> and <i>MDM4</i> amplification, and <i>ATRX</i> and <i>KIT</i> pathogenic mutations. The tumor also harbored a <i>SYN2</i>::<i>PPARG</i> gene fusion not previously reported in glioma. The case illustrates the importance of integrating advanced molecular techniques into the diagnostic process for older patients with atypical presentations of high-grade glioma. The potential biological significance and possible future therapeutic implications of the <i>SYN2</i>::<i>PPARG</i> gene fusion are discussed.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"15 1","pages":"2641996"},"PeriodicalIF":0.0,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13020859/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147509971","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Atypical behavior of recurrent glioblastoma tumor cells with a highly adherent radial glial phenotype.","authors":"Manjusha Vaidya, Jonhoi Smith, Julia Pessaia, Melvin Field, Kiminobu Sugaya","doi":"10.1080/20450907.2025.2559576","DOIUrl":"10.1080/20450907.2025.2559576","url":null,"abstract":"<p><p>Glioblastoma (GBM) is the most common malignant primary brain tumor, characterized by a high recurrence rate despite aggressive therapy. We present a case of a 32-year-old male with a recurrent WHO Grade IV IDH1-mutant astrocytoma after undergoing standard Stupp protocol chemoradiation and tumor-treating field therapy. Repeat surgery was performed where in vitro analysis of recurrent GBM cells revealed atypical behavior, rapid adhesion within minutes of plating, and the formation of radial glial-like cells (RGCs) with 3D aggregated cells, phenotypes absent in the primary tumor. These brain lipid-binding protein positive RGCs exhibited elongated processes that facilitated cancer cell migration, potentially contributing to tumor invasiveness. Extensive treatment between the primary and recurrent tumors may have induced this phenotypic shift, highlighting therapy-induced plasticity as a key factor in recurrence. The emergence of RGCs in recurrent GBM underscores the need for targeted therapies addressing tumor adaptability to improve treatment outcomes.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"14 1","pages":"2559576"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12452472/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145085166","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of radiation fractionation on pseudoprogression in older patients with glioblastoma: a retrospective cohort study.","authors":"Derek L Chien, Sara J Hardy, Jennifer N Serventi, Jacqueline M Behr, Nimish A Mohile, Lauryn E Hemminger","doi":"10.1080/20450907.2025.2584958","DOIUrl":"10.1080/20450907.2025.2584958","url":null,"abstract":"<p><strong>Aims: </strong>We aimed to investigate a potential association between hypofractionated radiotherapy (HFRT) vs. conventional radiotherapy (CRT) and development of pseudoprogression in patients over the age of 65 treated for glioblastoma (GBM).</p><p><strong>Materials & methods: </strong>Seventy-nine patients with glioblastoma (29 who received HFRT and 50 who received CRT) were included in this retrospective cohort study from a single institution. Demographic, clinical, and radiation information, including development of pseudoprogression and standard prognostic factors like Karnofsky Performance Status (KPS) and extent of surgical resection, were collected.</p><p><strong>Results: </strong>Radiation regimen alone was not associated with development of pseudoprogression. Patients who had lower KPS at the time of diagnosis and received HFRT had lower rates of pseudoprogression. There was no association between radiation regimen, pseudoprogression, and any other clinical factors.</p><p><strong>Conclusion: </strong>Older patients with glioblastoma who receive HFRT are not more likely to develop pseudoprogression than those who receive CRT. Patients with lower functional status receiving HFRT may be less likely to mount an inflammatory response leading to pseudoprogression. Prospective investigation is warranted to validate these results and evaluate other factors leading to treatment complications in older patients with glioblastoma in order to optimize outcomes and minimize toxicity.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"14 1","pages":"2584958"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12599363/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145457900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical decision making in the era of supramarginal glioma resections: a current perspective and narrative review.","authors":"Max O Krucoff","doi":"10.1080/20450907.2025.2571341","DOIUrl":"10.1080/20450907.2025.2571341","url":null,"abstract":"<p><p>Whether to surgically resect a margin of grossly normal appearing brain around anatomically amenable diffuse gliomas (i.e., perform a supratotal, supramarginal, or supramaximal resection) has been controversial. Over the past 5-10 years, however, evidence published by multiple independent groups has established a substantial survival benefit to this approach, moving the field towards a consensus that supramarginal resections should be offered when possible. However, many practitioners remain hesitant to offer supratotal resections due to concerns for variable neuropsychological outcomes and a mindset of \"first, do no harm.\" Unfortunately, and perhaps counterintuitively, available data also suggest that opting for more conservative surgical approaches when more aggressive resections are possible may result in both suboptimal long-term functional and survival outcomes. To explore this complex and actively evolving issue, here I review evidence surrounding the multidimensional clinical impacts of supramarginal resections across all diffuse glioma subtypes. I then evaluate what is known about anatomical-functional relationships subserving cognition, behavior, and mood regulation, and I examine ethical considerations that arise when counseling patients at the difficult time of diagnosis. I then conclude with a set of case examples that demonstrate how the principles explored in this review can be applied in real-world situations to optimize, individualize, and humanize oncological and functional outcomes.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"14 1","pages":"2571341"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12574574/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145376264","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Multiple brain lesions in a patient with relapsed hairy cell leukemia: a case report and review of the literature.","authors":"Katell Le Dû, Jacques Delaunay, Maud Voldoire, Thomas Cuvier, Pierre-Yves Renard, Benoît Quilichini, Eric Wafflart, Julien Dubreuil, Sophie Sadot-Lebouvier","doi":"10.1080/20450907.2025.2563981","DOIUrl":"10.1080/20450907.2025.2563981","url":null,"abstract":"<p><p>Cerebral lesions are rare in hairy cell leukemia (HCL), and its incidence remains to be determined. Identifying the cause can be challenging. In this report, we present a case of brain lesions occurring several years after diagnosis. A 76-year-old male patient presented to the Emergency Department with confusion. He had been diagnosed with HCL in 1999 and had received five lines of treatment. Cerebral imaging revealed multiple nodular lesions, with edema and a hemorrhagic appearance. Cerebrospinal fluid tests were negative. The tumor origin was retained due to concomitant relapse (blood, lymph nodes). Despite the partial efficacy of rituximab-cladribine treatment, the patient died of <i>Candida</i> pneumonia. A review of the literature (PubMed, CrossRef, Google Scholar) identified seventeen cases between 1966 and 2024, with a median age of 59 years (33-80). Cladribine, with or without rituximab, was the most widely prescribed treatment regimen with a complete response rate of 57%. Four (23.5%) patients died (two from infection, one from gastrointestinal bleeding and one from an unknown cause). These atypical presentations suggest that brain imaging and advanced biological investigations should be performed to guide management.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"14 1","pages":"2563981"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12477879/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145147976","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sustained response to larotrectinib in a pediatric patient with recurrent STRN3::NTRK2 fusion-positive pilocytic astrocytoma.","authors":"Kishore Balasubramanian, Kar-Ming Fung, Rene Y McNall-Knapp, Karl Balsara","doi":"10.1080/20450907.2025.2558455","DOIUrl":"10.1080/20450907.2025.2558455","url":null,"abstract":"<p><p>A 7-year-old female with recurrent midline pilocytic astrocytoma harboring a rare <i>STRN3::NTRK2</i> fusion achieved sustained near-complete radiographic and clinical response to larotrectinib, a selective TRK inhibitor. Initial subtotal resection of the midbrain/thalamic tumor was followed by progression, prompting molecular profiling that identified the <i>STRN3::NTRK2</i> fusion. Larotrectinib therapy initiated at recurrence resulted in a rapid reduction by 3 months, resolution of pontine extension by 6 months, and near-complete resolution by 15 months. This case highlights the potential of molecular diagnostics in pediatric neuro-oncology, particularly for BRAF-negative midline gliomas where NTRK fusions are rare but actionable. The durable response supports prioritizing larotrectinib over conventional chemotherapy in unresectable/progressive NTRK-driven gliomas. Routine fusion screening in BRAF-negative cases should be considered to identify candidates for targeted therapy. This report expands the known spectrum of NTRK2 partners in pilocytic astrocytoma and reinforces the use of TRK inhibitors as a treatment for molecularly defined subsets of pediatric glioma.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"14 1","pages":"2558455"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12498535/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145225053","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autologous stem cell transplantation in adults with atypical teratoid rhabdoid tumor: a case report and review.","authors":"Jackson Griffith-Linsley, William Robert Bell, Aaron Cohen-Gadol, Diane Donegan, Angela Richardson, Michael Robertson, Kevin Shiue, Kathryn Nevel","doi":"10.1080/20450907.2024.2375960","DOIUrl":"10.1080/20450907.2024.2375960","url":null,"abstract":"<p><p><b>Aim:</b> Atypical teratoid rhabdoid tumor (ATRT) is a rare and highly aggressive primary CNS neoplasm, predominantly observed in children. The use of autologous stem cell transplantation (ASCT) in pediatric ATRT has shown promise; however, its utility in adult ATRT remains unclear. <b>Patients & methods:</b> This study presents the case of an adult patient with ATRT who is in remission after ASCT and reviews the literature on ASCT in adults with ATRT. Four cases of ATRT in adults who underwent ASCT were identified, with pertinent data summarized. <b>Results:</b> All five patients survived longer than the historical average survival rate, four of whom had no clinical or radiographic evidence of disease at the final follow-up. <b>Conclusion:</b> Based on limited data, there may be a role for ASCT in the treatment of adults with ATRT.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"13 1","pages":"2375960"},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11285279/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141747640","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development of brain metastases in non-small-cell lung cancer: high-risk features.","authors":"Nolan Winslow, Jacqueline Boyle, William Miller, Yanzhi Wang, Francois Geoffroy, Andrew J Tsung","doi":"10.1080/20450907.2024.2395804","DOIUrl":"10.1080/20450907.2024.2395804","url":null,"abstract":"<p><p><b>Aim:</b> Brain metastases (BM) are a common site of disease progression and treatment failure in non-small-cell lung cancer (NSCLC) and can be identified in up to 30-50% of patients. Although they are common, there is no standardized screening protocol for development of BM in NSCLC. Multiple clinical variables predict increased BM occurrence, and, when present, should be used to initiate screening MRI.<b>Materials & methods:</b> We performed a single center retrospective review of NSCLC patients, examining BM development and overall survival. Available clinical, radiographic and molecular data were reviewed for association with BM and overall survival. A predictive model for BM development was created for multivariate analysis.<b>Results:</b> Risk factors for new BM development in NSCLC included younger age, larger primary lung tumor, Karnofsky performance score (KPS) <70, pre-existing liver or bone metastases, large cell histology and family history of cancer. Factors associated with decreased OS were larger primary lung tumor, extracranial metastases at time of diagnosis, large cell histology and poorly-differentiated carcinoma histology.<b>Conclusion:</b> There are multiple high risk features for developing BM in NSCLC. Each of these factors should routinely be investigated, and presence should prompt brain MRI to allow earlier diagnosis and treatment of BM.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"13 1","pages":"2395804"},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11404603/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142281455","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}