{"title":"A pragmatic diagnostic approach to primary intracranial germ cell tumors and their treatment outcomes.","authors":"Jeyaanth Venkatasai, Rajesh Balakrishnan, Balakrishnan Rajkrishna, Patricia Sebastain, Rikki Rorima John, Harshad Arvind Vanjare, Krishna Prabhu, Bijesh Nair, Leni Grace Mathew, Selvamani Backianathan","doi":"10.2217/cns-2021-0012","DOIUrl":"10.2217/cns-2021-0012","url":null,"abstract":"<p><p><b>Background:</b> Primary intracranial germ cell tumors (ICGCT) are often diagnosed with tumor markers and imaging, which may avoid the need for a biopsy. An intracranial germ cell tumor with mild elevation of markers is seldom stratified as a distinct entity. <b>Methods:</b> Fifty-nine patients were stratified into three groups: pure germinoma (PG), secreting germinoma (SG) and non-germinomatous germ cell tumors (NGGCTs). <b>Results:</b> At 5 years, progression-free survival and overall survival of the three groups (PG vs SG vs NGGCT) were 91% versus 81% versus 59%, and 100% versus 82% versus 68%, respectively. There was no statistically significant difference in outcome among histologically and clinically diagnosed germinomas. <b>Conclusion:</b> A criterion for clinical diagnosis when a biopsy is not feasible is elucidated, and comparable outcomes were demonstrated with histologically diagnosed germinomas.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/86/0f/cns-10-79.PMC8610002.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39645455","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}
CNS OncologyPub Date : 2021-09-01Epub Date: 2021-08-11DOI: 10.2217/cns-2021-0007
Srinivas Annavarapu, Anagha Gogate, Trang Pham, Kalatu Davies, Prianka Singh, Nicholas Robert
{"title":"Treatment patterns and outcomes for patients with newly diagnosed glioblastoma multiforme: a retrospective cohort study.","authors":"Srinivas Annavarapu, Anagha Gogate, Trang Pham, Kalatu Davies, Prianka Singh, Nicholas Robert","doi":"10.2217/cns-2021-0007","DOIUrl":"https://doi.org/10.2217/cns-2021-0007","url":null,"abstract":"<p><p><b>Aim:</b> Investigate real-world outcomes and healthcare utilization of patients with glioblastoma multiforme (GBM) related to O<sup>6</sup>-methylguanine DNA methyltransferase (MGMT) promoter testing and methylation. <b>Patients & methods:</b> US Oncology Network data were analyzed for patients receiving first-line (1L) treatment for GBM. <b>Results:</b> Most patients received 1L radiation with temozolomide. Unadjusted median overall survival (OS) was higher in tested versus untested (median:18.1 vs 11.8 months) and in methylated versus unmethylated (median: 25.5 vs 12.4 months). Untested status, unmethylated MGMT and older age were associated with reduced OS and longer 1L treatment with increased OS. Similar findings were observed for progression-free survival. Utilization was similar between cohorts. <b>Conclusion:</b> In community oncology practices, MGMT methylation and testing were predictive of better survival in GBM.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/df/e0/cns-10-76.PMC8461754.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39301194","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}
CNS OncologyPub Date : 2021-09-01Epub Date: 2021-09-06DOI: 10.2217/cns-2021-0002
Ngan Nguyen, Jordan Redfield, Matthew Ballo, Madison Michael, Jeffrey Sorenson, Daniel Dibaba, Jim Wan, Glenda Delgado Ramos, Manjari Pandey
{"title":"Identifying the optimal cutoff point for MGMT promoter methylation status in glioblastoma.","authors":"Ngan Nguyen, Jordan Redfield, Matthew Ballo, Madison Michael, Jeffrey Sorenson, Daniel Dibaba, Jim Wan, Glenda Delgado Ramos, Manjari Pandey","doi":"10.2217/cns-2021-0002","DOIUrl":"https://doi.org/10.2217/cns-2021-0002","url":null,"abstract":"<p><p><b>Aim:</b> To define the optimal cutoff point for determining methylation status of O6-methylguanine-DNA methyltransferase (MGMT) by pyrosequencing in glioblastoma. <b>Patients & methods:</b> A retrospective study of 109 glioblastoma patients was performed to determine the optimal cutoff point for MGMT methylation status. <b>Results:</b> Receiver operating characteristic (ROC) analysis revealed 21% as the optimal cutoff (sensitivity: 68%; specificity: 59%) for MGMT methylation corresponding with the highest likelihood ratio of 1.66 and accuracy of 0.65. Methylation status (hazard ratio: 0.453; 95% CI: 0.279-0.735; p = 0.001) was associated with better overall survival. The crude model indicated linearity between methylation percent and survival rate; an increase of 10% of methylation resulted in a reduction of risk of death by 20% (p = 0.004). <b>Conclusion:</b> ROC analysis determined 21% as the optimal cutoff point for MGMT methylation status by pyrosequencing.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/14/0e/cns-10-74.PMC8461752.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39405027","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}
CNS OncologyPub Date : 2021-09-01DOI: 10.2217/cns-2021-0008
Tanvi Nadkarni, Kimberly Hamilton, Faraze Niazi, Melanie Ward, Uchenna Okakpu, Rudolph J Castellani, Ion Prisneac, Ugur Sener
{"title":"Histone-mutant glioma presenting as diffuse leptomeningeal disease.","authors":"Tanvi Nadkarni, Kimberly Hamilton, Faraze Niazi, Melanie Ward, Uchenna Okakpu, Rudolph J Castellani, Ion Prisneac, Ugur Sener","doi":"10.2217/cns-2021-0008","DOIUrl":"https://doi.org/10.2217/cns-2021-0008","url":null,"abstract":"<p><p>Glioblastoma multiforme is the most common malignant primary brain tumor in adults. Histone H3 mutations have been identified in pediatric and adult gliomas, with H3K27M mutations typically associated with a posterior fossa midline tumor location and poor prognosis. Leptomeningeal disease is a known complication of histone-mutant glioma, but uncommon at the time of initial diagnosis. We describe a case of glioblastoma with H3K27M mutation that initially presented with progressive vision loss due to diffuse leptomeningeal disease in the absence of a mass lesion other than a small cerebellar area of enhancement and with cerebrospinal fluid cytology negative for malignant cells on two occasions, highlighting the importance of including primary CNS malignancies in the differential of diffuse radiographic leptomeningeal enhancement.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c2/52/cns-10-75.PMC8461753.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39390407","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}
CNS OncologyPub Date : 2021-09-01Epub Date: 2021-09-21DOI: 10.2217/cns-2021-0009
Jennifer H Kang, Christa B Swisher, Evan D Buckley, James E Herndon, Eric S Lipp, John P Kirkpatrick, Annick Desjardins, Henry S Friedman, Margaret O Johnson, Dina M Randazzo, David M Ashley, Katherine B Peters
{"title":"Primary brain tumor patients admitted to a US intensive care unit: a descriptive analysis.","authors":"Jennifer H Kang, Christa B Swisher, Evan D Buckley, James E Herndon, Eric S Lipp, John P Kirkpatrick, Annick Desjardins, Henry S Friedman, Margaret O Johnson, Dina M Randazzo, David M Ashley, Katherine B Peters","doi":"10.2217/cns-2021-0009","DOIUrl":"https://doi.org/10.2217/cns-2021-0009","url":null,"abstract":"<p><p><b>Purpose:</b> To describe our population of primary brain tumor (PBT) patients, a subgroup of cancer patients whose intensive care unit (ICU) outcomes are understudied. <b>Methods:</b> Retrospective analysis of PBT patients admitted to an ICU between 2013 to 2018 for an unplanned need. Using descriptive analyses, we characterized our population and their outcomes. <b>Results:</b> Fifty-nine PBT patients were analyzed. ICU mortality was 19% (11/59). The most common indication for admission was seizures (n = 16, 27%). <b>Conclusion:</b> Our ICU mortality of PBT patients was comparable to other solid tumor patients and the general ICU population and better than patients with hematological malignancies. Further study of a larger population would inform guidelines for triaging PBT patients who would most benefit from ICU-level care.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/68/64/cns-10-77.PMC8461751.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39456351","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}
CNS OncologyPub Date : 2021-06-01Epub Date: 2021-05-21DOI: 10.2217/cns-2020-0029
Kelly Chamberlin, Gregory Chamberlin, Katherine Saunders, Simon Khagi
{"title":"Next-generation sequencing reveals novel mutations in a collision tumor of glioblastoma and meningioma.","authors":"Kelly Chamberlin, Gregory Chamberlin, Katherine Saunders, Simon Khagi","doi":"10.2217/cns-2020-0029","DOIUrl":"https://doi.org/10.2217/cns-2020-0029","url":null,"abstract":"<p><p>Primary intracranial collision tumors are rare in patients without predisposing factors. We report such a case in a 42-year-old female who presented with headaches and altered mental status. Imaging revealed a single heterogeneous, rim-enhancing lesion in the left parieto-occipital periventricular region, involving the corpus callosum. Stereotactic biopsy demonstrated glioblastoma. Subsequent tumor resection showed histologic evidence of glioblastoma and meningioma. Next-generation sequencing was performed on both tumor components. The glioblastoma exhibited a <i>CDKN2A</i> homozygous deletion and novel missense mutations in <i>TAF1L</i> and <i>CSMD3</i>, while no definitive genetic alterations were identified in the meningioma. Next-generation sequencing may yield insight into molecular drivers of intracranial collision tumors and aid in identifying future therapeutic targets.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/47/43/cns-10-70.PMC8162195.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39003040","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}
CNS OncologyPub Date : 2021-06-01Epub Date: 2021-05-19DOI: 10.2217/cns-2020-0026
Anda-Alexandra Calinescu, McKenzie C Kauss, Zain Sultan, Wajd N Al-Holou, Sue K O'Shea
{"title":"Stem cells for the treatment of glioblastoma: a 20-year perspective.","authors":"Anda-Alexandra Calinescu, McKenzie C Kauss, Zain Sultan, Wajd N Al-Holou, Sue K O'Shea","doi":"10.2217/cns-2020-0026","DOIUrl":"https://doi.org/10.2217/cns-2020-0026","url":null,"abstract":"<p><p>Glioblastoma, the deadliest form of primary brain tumor, remains a disease without cure. Treatment resistance is in large part attributed to limitations in the delivery and distribution of therapeutic agents. Over the last 20 years, numerous preclinical studies have demonstrated the feasibility and efficacy of stem cells as antiglioma agents, leading to the development of trials to test these therapies in the clinic. In this review we present and analyze these studies, discuss mechanisms underlying their beneficial effect and highlight experimental progress, limitations and the emergence of promising new therapeutic avenues. We hope to increase awareness of the advantages brought by stem cells for the treatment of glioblastoma and inspire further studies that will lead to accelerated implementation of effective therapies.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b4/aa/cns-10-73.PMC8162173.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38995913","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}
CNS OncologyPub Date : 2021-06-01Epub Date: 2021-05-21DOI: 10.2217/cns-2021-0003
Ilaria Maggio, Enrico Franceschi, Alicia Tosoni, Vincenzo Di Nunno, Lidia Gatto, Raffaele Lodi, Alba A Brandes
{"title":"Meningioma: not always a benign tumor. A review of advances in the treatment of meningiomas.","authors":"Ilaria Maggio, Enrico Franceschi, Alicia Tosoni, Vincenzo Di Nunno, Lidia Gatto, Raffaele Lodi, Alba A Brandes","doi":"10.2217/cns-2021-0003","DOIUrl":"10.2217/cns-2021-0003","url":null,"abstract":"<p><p>Meningiomas are the most common primary intracranial tumors. The majority of meningiomas are benign, but they can present different grades of dedifferentiation from grade I to grade III (anaplastic/malignant) that are associated with different outcomes. Radiological surveillance is a valid option for low-grade asymptomatic meningiomas. In other cases, the treatment is usually surgical, aimed at achieving a complete resection. The use of adjuvant radiotherapy is the gold standard for grade III, is debated for grade II and is not generally indicated for radically resected grade I meningiomas. The use of systemic treatments is not standardized. Here we report a review of the literature on the clinical, radiological and molecular characteristics of meningiomas, available treatment strategies and ongoing clinical trials.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3d/53/cns-10-72.PMC8162186.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39001625","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}
CNS OncologyPub Date : 2021-06-01Epub Date: 2021-04-28DOI: 10.2217/cns-2020-0030
Justin Thomas Low, Shih-Hsiu Wang, Katherine B Peters
{"title":"Diffuse midline glioma with H3 K27M-mutation in an 83-year-old woman.","authors":"Justin Thomas Low, Shih-Hsiu Wang, Katherine B Peters","doi":"10.2217/cns-2020-0030","DOIUrl":"https://doi.org/10.2217/cns-2020-0030","url":null,"abstract":"<p><p>Diffuse midline gliomas harboring histone H3 K27M mutations are most commonly found in the brainstem of children. This mutation confers a WHO grade IV designation and is associated with a particularly poor prognosis. Although traditionally considered to be a disease of children and young adults, a number of recent reports have described H3 K27M mutations in older adults with diffuse midline gliomas. Here, we present the unusual case of a diffuse midline glioma in the pons and cerebellum of an 83-year-old woman and review the evolving clinical literature on this entity in adults. This case underscores that it may occur even in older adults, in whom prognostic and treatment paradigms used in pediatrics may not be directly applicable.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/26/d9/cns-10-71.PMC8162147.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38923906","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}
CNS OncologyPub Date : 2021-03-01Epub Date: 2021-01-15DOI: 10.2217/cns-2020-0027
Timothy A Gregory, Lyndon B Chumbley, John W Henson, Brett J Theeler
{"title":"Adult pilocytic astrocytoma in the molecular era: a comprehensive review.","authors":"Timothy A Gregory, Lyndon B Chumbley, John W Henson, Brett J Theeler","doi":"10.2217/cns-2020-0027","DOIUrl":"10.2217/cns-2020-0027","url":null,"abstract":"<p><p>Adult pilocytic astrocytoma (PA) is less prevalent than pediatric PA and is associated with a worse prognosis. In a literature review, we found that 88.3% of the molecular alterations in adult PA are associated with MAPK pathway dysregulation. The most common alterations are fusions of <i>BRAF</i>. Understanding of the mechanisms underlying this pathway has evolved substantially, heralding advancements in specific targeted therapy. Here, we review clinical and molecular features of adult PA, characteristics predicting aggressive behavior and approaches to standard and investigational therapies. We highlight epigenetic profiling and integrated diagnosis as an essential component of classifying PA.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/81/ff/cns-10-68.PMC7962176.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38742225","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}