Diffuse midline glioma with an H3 K27M mutation: A comparison integrating the clinical, radiological, molecular features between adult and pediatric patients.

Abstract:

BACKGROUND:Diffuse midline glioma (DMG), H3 K27M-mutant occurs in both adult and pediatric populations. The characteristics of the two DMG groups were systematically explored in this study. METHODS: One-hundred-sixteen patients diagnosed with H3 K27M-mutant DMG in Beijing Tiantan Hospital from May 2016 to December 2018 were included. Patients were classified into adult group (n=57, 49.1%) and pediatric group (n=59, 50.9%). The clinical, radiological and molecular features were compared between the groups. Univariate and multivariate analyses were performed to identify prognostic factors. RESULTS:Compared with the adult group, pediatric patients had a younger age (8.9±4.1 vs. 35.1±11.8 years, P<0.001), a lower preoperative Karnofsky performance scale score (62.9±15.5 vs. 72.1±16.5, P=0.004), a lower rate of total resection (5.7% vs. 26.8%, P=0.009), a larger tumor size (4.4±0.9 vs. 3.9±1.5 cm, P=0.045), a higher Ki-67 index (63.0% vs. 37.8%, P=0.047), and higher rates of postoperative cranial nerve palsy (61.0% vs. 36.8%, P=0.009) and ataxia (45.8% vs. 26.3%, P=0.029). Adult DMG was predominantly located in the thalamus, while the predilection site for pediatric DMG was brainstem (P<0.001). Kaplan-Meier plot showed that the median survival of adult and pediatric DMG was 16.0 (9.7-22.3) months and 10.0 (8.3-11.7) months, respectively, which imparted a significant difference (P=0.008). Age at diagnosis, radiotherapy, motor deficit and hydrocephalus were confirmed as independent prognostic factors according to the multivariate analysis (P<0.05). CONCLUSION:Compared with adult patients, children with H3 K27M-mutant DMG confer distinct clinical, radiological and molecular characteristics, and have a dismal prognosis. Radiotherapy is an independent factor associated with prolonged survival.

journal_name

Neuro Oncol

journal_title

Neuro-oncology

authors

Jiang H,Yang K,Ren X,Cui Y,Li M,Lei Y,Lin S

doi

10.1093/neuonc/noz152

subject

Has Abstract

pub_date

2019-08-24 00:00:00

eissn

1522-8517

issn

1523-5866

pii

5554271

pub_type

杂志文章,收录出版
  • Brain metastases in melanoma: roles of neurotrophins.

    abstract::Brain metastasis, which occurs in 20% to 40% of all cancer patients, is an important cause of neoplastic morbidity and mortality. Successful invasion into the brain by tumor cells must include attachment to microvessel endothelial cells, penetration through the blood-brain barrier, and, of relevance, a response to bra...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,评审

    doi:10.1215/s115285170300067x

    authors: Denkins Y,Reiland J,Roy M,Sinnappah-Kang ND,Galjour J,Murry BP,Blust J,Aucoin R,Marchetti D

    更新日期:2004-04-01 00:00:00

  • REV3L confers chemoresistance to cisplatin in human gliomas: the potential of its RNAi for synergistic therapy.

    abstract::The REV3L gene, encoding the catalytic subunit of human polymerase zeta, plays a significant role in the cytotoxicity, mutagenicity, and chemoresistance of certain tumors. However, the role of REV3L in regulating the sensitivity of glioma cells to chemotherapy remains unknown. In this study, we investigated the expres...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1215/15228517-2009-015

    authors: Wang H,Zhang SY,Wang S,Lu J,Wu W,Weng L,Chen D,Zhang Y,Lu Z,Yang J,Chen Y,Zhang X,Chen X,Xi C,Lu D,Zhao S

    更新日期:2009-12-01 00:00:00

  • Dogs are man's best friend: in sickness and in health.

    abstract::With the median survival of 14.6 months following best available standard of care, malignant gliomas (MGs) remain one of the biggest therapeutic challenges of the modern time. Although the last several decades have witnessed tremendous advancement in our understanding of MG and evolution of many successful preclinical...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,评审

    doi:10.1093/neuonc/now109

    authors: Bentley RT,Ahmed AU,Yanke AB,Cohen-Gadol AA,Dey M

    更新日期:2017-03-01 00:00:00

  • Altered brain morphology after focal radiation reveals impact of off-target effects: implications for white matter development and neurogenesis.

    abstract:Background:Children with brain tumors treated with cranial radiation therapy (RT) often exhibit cognitive late effects, commonly associated with reduced white matter (WM) volume and decreased neurogenesis. The impact of radiation damage in particular regions or tissues on brain development as a whole has not been eluci...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/nox211

    authors: Beera KG,Li YQ,Dazai J,Stewart J,Egan S,Ahmed M,Wong CS,Jaffray DA,Nieman BJ

    更新日期:2018-05-18 00:00:00

  • An epidemiology report for primary central nervous system tumors in adolescents and young adults: a nationwide population-based study in France, 2008-2013.

    abstract:BACKGROUND:Primary central nervous system tumors (PCNST) among adolescents and young adults (AYA, 15-39 y) have rarely been reported. We present a nationwide report of PCNST histologically confirmed in the French AYA population between 2008 and 2013. METHODS:Patients were identified through the French Brain Tumor Data...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/noz227

    authors: Ng S,Zouaoui S,Bessaoud F,Rigau V,Roux A,Darlix A,Bauchet F,Mathieu-Daudé H,Trétarre B,Figarella-Branger D,Pallud J,Frappaz D,Roujeau T,Bauchet L

    更新日期:2020-06-09 00:00:00

  • Survivorship care planning and implementation in neuro-oncology.

    abstract::Survivorship has become a significant topic within oncologic care. The tools and means by which the provision of survivorship care can be implemented and delivered are in development and are the focus of significant research oncology-wide. These tools and methods include innovations of survivorship care delivery, surv...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/noy110

    authors: Leeper H,Milbury K

    更新日期:2018-11-09 00:00:00

  • High-dose methotrexate is beneficial in parenchymal brain masses of uncertain origin suspicious for primary CNS lymphoma.

    abstract::In patients with parenchymal brain masses of uncertain origin responsive to corticosteroids, primary CNS lymphoma (PCNSL) should be considered. PCNSL is a rare but aggressive brain tumor that is highly sensitive to high-dose methotrexate (HDMTX)-based chemotherapy. We report a series of six patients with brain masses ...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1215/15228517-2006-037

    authors: Kiewe P,Loddenkemper C,Anagnostopoulos I,Reinwald M,Thiel E,Korfel A

    更新日期:2007-04-01 00:00:00

  • Rhenium-186 liposomes as convection-enhanced nanoparticle brachytherapy for treatment of glioblastoma.

    abstract::Although external beam radiation is an essential component to the current standard treatment of primary brain tumors, its application is limited by toxicity at doses more than 80 Gy. Recent studies have suggested that brachytherapy with liposomally encapsulated radionuclides may be of benefit, and we have reported met...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/nos060

    authors: Phillips WT,Goins B,Bao A,Vargas D,Guttierez JE,Trevino A,Miller JR,Henry J,Zuniga R,Vecil G,Brenner AJ

    更新日期:2012-04-01 00:00:00

  • An NF-κB signature predicts low-grade glioma prognosis: a precision medicine approach based on patient-derived stem cells.

    abstract:Background:While recent genome-wide association studies have suggested novel low-grade glioma (LGG) stratification models based on a molecular classification, we explored the potential clinical utility of patient-derived cells. Specifically, we assayed glioma-associated stem cells (GASC) that are patient-derived and re...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/nox234

    authors: Ius T,Ciani Y,Ruaro ME,Isola M,Sorrentino M,Bulfoni M,Candotti V,Correcig C,Bourkoula E,Manini I,Pegolo E,Mangoni D,Marzinotto S,Radovic S,Toffoletto B,Caponnetto F,Zanello A,Mariuzzi L,Di Loreto C,Beltrami AP,Pia

    更新日期:2018-05-18 00:00:00

  • Long-term outcomes of surgical resection with or without adjuvant radiation therapy for treatment of spinal ependymoma: a retrospective multicenter study by the Korea Spinal Oncology Research Group.

    abstract:BACKGROUND:We sought to determine the surgical treatment and functional outcome and identify the predictors of survival in a retrospective cohort of patients with spinal cord ependymoma using data collected from the Korea Spinal Oncology Research Group database. METHODS:The data regarding 88 patients who had been surg...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,多中心研究

    doi:10.1093/neuonc/not038

    authors: Lee SH,Chung CK,Kim CH,Yoon SH,Hyun SJ,Kim KJ,Kim ES,Eoh W,Kim HJ

    更新日期:2013-07-01 00:00:00

  • A randomized, placebo-controlled pilot trial of armodafinil for fatigue in patients with gliomas undergoing radiotherapy.

    abstract:BACKGROUND:Fatigue is common among glioma patients undergoing radiotherapy (RT) and impacts quality of life (QOL). We evaluated whether armodafinil, a wakefulness-promoting medication, improves fatigue in glioma patients undergoing RT. METHODS:Eligibility criteria included age ≥18 years, Karnofsky performance status ≥...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,随机对照试验

    doi:10.1093/neuonc/now007

    authors: Lee EQ,Muzikansky A,Drappatz J,Kesari S,Wong ET,Fadul CE,Reardon DA,Norden AD,Nayak L,Rinne ML,Alexander BM,Arvold ND,Doherty L,Stefanik J,LaFrankie D,Ruland SF,Pulverenti J,Smith KH,Gaffey SC,Hammond S,Wen PY

    更新日期:2016-06-01 00:00:00

  • Racial differences in primary central nervous system lymphoma incidence and survival rates.

    abstract::To determine racial and ethnic differences in incidence and survival in patients with primary central nervous system lymphoma (PCNSL), NCI Surveillance, Epidemiology, and End Results (SEER) program data from 1992 to 2002 were queried. Data were substratified by age (20-49 years vs. 50 or above) and race (White, Black,...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1215/15228517-2008-103

    authors: Pulido JS,Vierkant RA,Olson JE,Abrey L,Schiff D,O'Neill BP

    更新日期:2009-06-01 00:00:00

  • Paraneoplastic chorea: case study with autopsy confirmation.

    abstract::A 67-year-old man presented with a 7-month history of insidiously progressive chorea, ataxia, and vertigo. Neurologic examination revealed deficits referable to the basal nuclei, cerebellar vermis, and vestibular nuclei. Small-cell lung cancer was diagnosed by fine-needle biopsy of a parahilar mass. After chemotherapy...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,评审

    doi:10.1093/neuonc/4.3.192

    authors: Tremont-Lukats IW,Fuller GN,Ribalta T,Giglio P,Groves MD

    更新日期:2002-07-01 00:00:00

  • Serum-free culture success of glial tumors is related to specific molecular profiles and expression of extracellular matrix-associated gene modules.

    abstract:BACKGROUND:Recent molecular characterization studies have identified clinically relevant molecular subtypes to coexist within the same histological entities of glioma. Comparative studies between serum-supplemented and serum-free (SF) culture conditions have demonstrated that SF conditions select for glioma stem-like c...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/not116

    authors: Balvers RK,Kleijn A,Kloezeman JJ,French PJ,Kremer A,van den Bent MJ,Dirven CM,Leenstra S,Lamfers ML

    更新日期:2013-12-01 00:00:00

  • A single intravenous injection of oncolytic picornavirus SVV-001 eliminates medulloblastomas in primary tumor-based orthotopic xenograft mouse models.

    abstract::Difficulties of drug delivery across the blood-brain barrier (BBB) and failure to eliminate cancer stem cells (CSCs) are believed to be the major causes of tumor recurrences in children with medulloblastoma (MB). Seneca Valley virus-001 (SVV-001) is a naturally occurring oncolytic picornavirus that can be systemically...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/noq148

    authors: Yu L,Baxter PA,Zhao X,Liu Z,Wadhwa L,Zhang Y,Su JM,Tan X,Yang J,Adesina A,Perlaky L,Hurwitz M,Idamakanti N,Police SR,Hallenbeck PL,Blaney SM,Chintagumpala M,Hurwitz RL,Li XN

    更新日期:2011-01-01 00:00:00

  • Recent advances in intradural spinal tumors.

    abstract::Intradural spinal tumors are rare tumors of the central nervous system. Due to the eloquence of the spinal cord and its tracts, the compact architecture of the cord and nerves, and the infiltrative nature of some of these tumors, surgical resection is difficult to achieve without causing neurological deficits. Likewis...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,评审

    doi:10.1093/neuonc/nox230

    authors: Abd-El-Barr MM,Huang KT,Moses ZB,Iorgulescu JB,Chi JH

    更新日期:2018-05-18 00:00:00

  • Gadd45a sensitizes medulloblastoma cells to irradiation and suppresses MMP-9-mediated EMT.

    abstract::Medulloblastomas are the most common malignant tumors of the central nervous system during childhood. Radiation-induced medulloblastoma tumor recurrences are aggressive and metastatic in nature. In the present study, we demonstrate that Gadd45a expression can sensitize medulloblastoma cells to radiotherapy. We have el...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/nor109

    authors: Asuthkar S,Nalla AK,Gondi CS,Dinh DH,Gujrati M,Mohanam S,Rao JS

    更新日期:2011-10-01 00:00:00

  • Endogenous inhibitors of angiogenesis in malignant gliomas: nature's antiangiogenic therapy.

    abstract::Angiogenesis is necessary for tumor growth beyond a volume of approximately 2 mm(3). This observation, along with the accessibility of tumor vessels to therapeutic targeting, has resulted in a research focus on inhibitors of angiogenesis. A number of endogenous inhibitors of angiogenesis are found in the body. Some of...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,评审

    doi:10.1215/S115285170400119X

    authors: Rege TA,Fears CY,Gladson CL

    更新日期:2005-04-01 00:00:00

  • Arterial spin labeling MR imaging for differentiation between high- and low-grade glioma-a meta-analysis.

    abstract:Background:Arterial spin labeling is an MR imaging technique that measures cerebral blood flow (CBF) non-invasively. The aim of the study is to assess the diagnostic performance of arterial spin labeling (ASL) MR imaging for differentiation between high-grade glioma and low-grade glioma. Methods:Cochrane Library, Emba...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,meta分析

    doi:10.1093/neuonc/noy095

    authors: Falk Delgado A,De Luca F,van Westen D,Falk Delgado A

    更新日期:2018-10-09 00:00:00

  • The L84F polymorphic variant of human O6-methylguanine-DNA methyltransferase alters stability in U87MG glioma cells but not temozolomide sensitivity.

    abstract::First-line therapy for patients with glioblastoma multiforme includes treatment with radiation and temozolomide (TMZ), an oral DNA alkylating chemotherapy. Sensitivity of glioma cells to TMZ is dependent on the level of cellular O(6)-methylguanine-DNA methyltransferase (MGMT) repair activity. Several common coding-reg...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1215/15228517-2008-080

    authors: Remington M,Chtchetinin J,Ancheta K,Nghiemphu PL,Cloughesy T,Lai A

    更新日期:2009-02-01 00:00:00

  • PDGFRA, PDGFRB, EGFR, and downstream signaling activation in malignant peripheral nerve sheath tumor.

    abstract::We investigated the activation of platelet-derived growth factor (PDGF) receptor A (PDGFRA), PDGF receptor B (PDGFRB), epidermal growth factor receptor (EGFR), and their downstream pathways in malignant peripheral nerve sheath tumors (MPNSTs). PDGFRA, PDGFRB, and EGFR were immunohistochemically, biochemically, cytogen...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1215/15228517-2009-003

    authors: Perrone F,Da Riva L,Orsenigo M,Losa M,Jocollè G,Millefanti C,Pastore E,Gronchi A,Pierotti MA,Pilotti S

    更新日期:2009-12-01 00:00:00

  • Phase I and pharmacokinetic study of karenitecin in patients with recurrent malignant gliomas.

    abstract::Karenitecin is a highly lipophilic camptothecin analogue with a lactone ring that is relatively resistant to inactivating hydrolysis under physiologic conditions. This phase I clinical trial was conducted to determine the maximum tolerated dose (MTD) of karenitecin in adults with recurrent malignant glioma (MG), to de...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1215/15228517-2008-030

    authors: Grossman SA,Carson KA,Phuphanich S,Batchelor T,Peereboom D,Nabors LB,Lesser G,Hausheer F,Supko JG,New Approaches to Brain Tumor Therapy CNS Consortium.

    更新日期:2008-08-01 00:00:00

  • A novel tumor-promoting role for nuclear factor IA in glioblastomas is mediated through negative regulation of p53, p21, and PAI1.

    abstract::Background Nuclear factor IA (NFIA), a transcription factor and essential regulator in embryonic glial development, is highly expressed in human glioblastoma (GBM) compared with normal brain, but its contribution to GBM and cancer pathogenesis is unknown. Here we demonstrate a novel role for NFIA in promoting growth a...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/not167

    authors: Lee JS,Xiao J,Patel P,Schade J,Wang J,Deneen B,Erdreich-Epstein A,Song HR

    更新日期:2014-01-01 00:00:00

  • Telomere maintenance and the etiology of adult glioma.

    abstract::A growing body of epidemiologic and tumor genomic research has identified an important role for telomere maintenance in glioma susceptibility, initiation, and prognosis. Telomere length has long been investigated in relation to cancer, but whether longer or shorter telomere length might be associated with glioma risk ...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,评审

    doi:10.1093/neuonc/nov082

    authors: Walsh KM,Wiencke JK,Lachance DH,Wiemels JL,Molinaro AM,Eckel-Passow JE,Jenkins RB,Wrensch MR

    更新日期:2015-11-01 00:00:00

  • A phase II trial of primary temozolomide in patients with grade III oligodendroglial brain tumors.

    abstract::Glial tumors with oligodendroglial components are considered chemo-responsive. Forty newly diagnosed patients (11 anaplastic oligodendrogliomas [OD] and 29 anaplastic oligoastrocytomas [OA]) were enrolled into this multicenter, open-label, single-arm Phase II trial of first-line temozolomide (200 mg/m(2) on days 1-5 e...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,多中心研究

    doi:10.1093/neuonc/nop065

    authors: Gan HK,Rosenthal MA,Dowling A,Kalnins R,Algar E,Wong N,Benson A,Woods AM,Cher L

    更新日期:2010-05-01 00:00:00

  • Establishing percent resection and residual volume thresholds affecting survival and recurrence for patients with newly diagnosed intracranial glioblastoma.

    abstract:INTRODUCTION:Surgery is first-line therapy for glioblastoma, and there is evidence that gross total resection is associated with improved survival. Gross total resection, however, is not always possible, and relationships among extent (percent) of resection (EOR), residual volume (RV), and survival are unknown. The goa...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/not137

    authors: Chaichana KL,Jusue-Torres I,Navarro-Ramirez R,Raza SM,Pascual-Gallego M,Ibrahim A,Hernandez-Hermann M,Gomez L,Ye X,Weingart JD,Olivi A,Blakeley J,Gallia GL,Lim M,Brem H,Quinones-Hinojosa A

    更新日期:2014-01-01 00:00:00

  • Response of rat intracranial 9L gliosarcoma to microbeam radiation therapy.

    abstract::Radiotherapeutic doses for malignant gliomas are generally palliative because greater, supposedly curative doses would impart clinically unacceptable damage to nearby vital CNS tissues. To improve radiation treatment for human gliomas, we evaluated microbeam radiation therapy, which utilizes an array of parallel, micr...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/4.1.26

    authors: Dilmanian FA,Button TM,Le Duc G,Zhong N,Peña LA,Smith JA,Martinez SR,Bacarian T,Tammam J,Ren B,Farmer PM,Kalef-Ezra J,Micca PL,Nawrocky MM,Niederer JA,Recksiek FP,Fuchs A,Rosen EM

    更新日期:2002-01-01 00:00:00

  • Homeobox C9 suppresses Beclin1-mediated autophagy in glioblastoma by directly inhibiting the transcription of death-associated protein kinase 1.

    abstract:BACKGROUND:The transcription factor homeobox C9 (HOXC9) plays a crucial role in developmental regulatory systems, where it determines the specific positional identities of cells along the anteroposterior axis. The expression of HOXC9 has been found to be dysregulated in some cancers such as lung cancer, breast cancer, ...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/nov281

    authors: Xuan F,Huang M,Liu W,Ding H,Yang L,Cui H

    更新日期:2016-06-01 00:00:00

  • Enhanced expression of coproporphyrinogen oxidase in malignant brain tumors: CPOX expression and 5-ALA-induced fluorescence.

    abstract::In photodynamic diagnosis, 5-aminolevulinic acid (5-ALA) is widely used for the fluorescence-guided resection of malignant brain tumors, where 5-ALA is converted to protoporphyrin IX, which exhibits strong fluorescence. Little is known, however, about the detailed molecular mechanisms underlying 5-ALA-induced fluoresc...

    journal_title:Neuro-oncology

    pub_type: 杂志文章,收录出版

    doi:10.1093/neuonc/nor116

    authors: Takahashi K,Ikeda N,Nonoguchi N,Kajimoto Y,Miyatake S,Hagiya Y,Ogura S,Nakagawa H,Ishikawa T,Kuroiwa T

    更新日期:2011-11-01 00:00:00

  • Fractional anisotropy of the optic radiations is associated with visual acuity loss in optic pathway gliomas of neurofibromatosis type 1.

    abstract:BACKGROUND:No more than half of patients with neurofibromatosis type 1 (NF1)-associated optic pathway gliomas (OPGs) develop vision loss. Prospectively identifying those who will require therapy remains challenging, because no reliable factors have yet been identified that predict future vision loss. To determine wheth...

    journal_title:Neuro-oncology

    pub_type: 杂志文章

    doi:10.1093/neuonc/not068

    authors: de Blank PM,Berman JI,Liu GT,Roberts TP,Fisher MJ

    更新日期:2013-08-01 00:00:00